Professional Documents
Culture Documents
The Red Book, 2022-23
The Red Book, 2022-23
Department of Medicine
Massachusetts General Hospital
Harvard Medical School
July 2022 – June 2023
Editors
Krishan Sharma, MD
Christopher Marnell, MD
LETTER FROM THE EDITORS
It is our honor to present the 2022-2023 edition of the Housestaff Manual for Cardiology. Former MGH house
officer, Andrew Sauer, spearheaded the first edition in 2010, with the intention of offering residents a practical
introduction to the major concepts essential to everyday patient care in the Coronary Care Unit (CICU) and the
cardiac Step Down Unit (SDU).
Continuing the efforts of last year’s edition, we hope to present a Red Book that provides an up-to-date,
comprehensive review of core Cardiology topics, yet in a format accessible enough for the busy resident to
pick up and extract critical teaching points to inform their patient care – the core mission the Red Book has
always sought to fulfill.
In 1994, based on the understanding that exceptional patient care is grounded in a rich foundation of evidence,
Albert Shaw and Ravi Thadhani set out to create a reference guide for residents that compiled up-to-date
guidelines for clinical management. This resource, entitled “The MGH Department of Medicine Housestaff
Manual,” has since been updated annually and represents the rigor, autonomy, and pride with which our
residents approach their work. We intend for “The Red Book” to encapsulate these same principles within the
field of cardiology and to pay tribute to the original DOM housestaff manual, now in its 26th year, and known
more affectionately as “The White Book.”
This commitment to well-informed practice is grounded in a genuine desire to understand, connect with, and
improve the lives of our patients, but a more difficult reality emerges when we feel that our efforts have fallen
short. As agents of medical practice, we continuously engage with elusive concepts—illness, intimacy, loss,
grief—and wrestle to ascribe meaning to the intangible. In the year 1980, Dr. Ted Stern, an MGH psychiatrist
and the psychiatric consultant to the medical intensive care unit (MICU), attempted to confront and respond to
these challenges when he purchased a journal, the cover of which was red, and placed it in the MICU. From
then, until 2003, the red book served as a collective forum for resident self-reflection. It supplemented his
weekly “autognosis” (self-knowledge) rounds. After it was filled, seven more volumes followed. Excerpts from
the journals were subsequently complied and incorporated into a book, “On the Edge of Life: Diary of a Medical
Intensive Care Unit” edited by Drs. Mikkael Sekeres (a former MGH house officer) and Ted Stern. While Dr.
Stern’s “Red Book” is no longer in existence, its spirit is still very much alive at MGH, particularly on our
cardiology rotations and exemplified by Dr. Hasan Bazari’s CICU reflection rounds. We hope that this guide will
serve as another reminder to carve out space for introspection and to allow for the projection of kindness and
empathy both inward and outward.
In addition to the individuals mentioned above, we would like to thank the many current and former MGH
housestaff and faculty who have updated and provided original contributions to this guide. We are deeply
appreciative of their dedication, leadership, and commitment, without which “The Red Book” would not be
possible.
The following attending reviewers have provided expert opinion and consultation for past and present editions
of the Red Book:
We would also like to acknowledge the outstanding leadership of our section editors (bold) and content writers:
Finally, we would like to provide a special thank-you to Dr. Dave Dudzinski, Dr. Jay Vyas, the DOM Chief
Residents, and the DOM Staff for supporting our endeavor.
We hope that this guide will continue to assist with the care of cardiac patients at MGH. As such, it is meant to
serve as a reference to be used in conjunction with other resources and never in place of sound clinical
judgment. Thank you for continuing to provide the highest level of care and compassion to our patients.
Krishan Sharma, MD
Christopher Marnell, MD
The Red Book Editors
2021-2022
Table of Contents
INTRODUCTION TO INPATIENT CARDIOLOGY 6
CCU & SDU Rotation Overview 6
Overview of Cardiac Procedures Relevant to CCU/SDU Patients: 9
ACUTE CORONARY SYNDROMES AND ISCHEMIC HEART DISEASE 11
Classification 11
Diagnosis 12
Management 17
ACS – pharmacologic management 18
Coronary Angiography and Percutaneous Coronary Intervention (PCI) 23
Post-Cath Care 28
Cardiopulmonary Bypass Grafting (CABG) 29
Post-MI Complications 30
Special Populations 34
HEART FAILURE 38
DEFINITIONS 38
HISTORY & INITIAL ASSESSMENT 39
CLASSIFICATION 40
ETIOLOGY 40
HFpEF 45
ACUTE RIGHT VENTRICULAR FAILURE (RV FAILURE) 50
ADVANCED THERAPIES FOR CARDIOGENIC SHOCK: MECHANICAL CIRCULATORY SUPPORT (MCS) 51
INTRA-AORTIC BALLOON PUMP (IABP) 52
ADVANCED THERAPY: LONG-TERM DURABLE MECHANICAL CIRCULATORY SUPPORT (LT-MCS) 54
ORTHOTOPIC HEART TRANSPLANT 56
4
Mitral Stenosis (MS) 140
Tricuspid Regurgitation (TR) 144
VASCULAR MEDICINE 146
Venous Thromboembolism 146
Deep Vein Thrombosis 146
Pulmonary Embolism 148
Catheter-associated thrombosis 150
Peripheral Artery Disease 151
Renal Artery Stenosis 154
Aortic Aneurysm 155
Aortic Dissection 158
Quick Reference Guide: Vascular Medicine 163
Congenital Heart Disease 167
PATENT FORAMEN OVALE (PFO) 168
ATRIAL SEPTAL DEFECT 170
VENTRICULAR SEPTAL DEFECT 171
Basic Physiology of Pregnancy 181
ACLS in Pregnancy 182
Cardiac Medications during pregnancy 184
Peripartum Cardiomyopathy (PPCM) 184
Arrhythmias in Pregnancy 187
Pregnancy and Acute Coronary Syndrome 187
5
Introduction to Inpatient Cardiology
TOC
6
Chris Marnell, Krishan Sharma
Introduction to Inpatient Cardiology
TOC
Overnight Support: There are many layers of overnight support in the CCU, each useful in different circumstances
● Cardiology Night Teach: Beginning in AY 2021-2022 there will be a dedicated NT for the CCU and SDU. Can be
contacted for procedural supervision, general cardiology, or critical care questions, or if needing support for
admissions (NT can either help stabilize patients or coordinate with chiefs to hit in an additional admitting resident).
The Cards NT is also responsible for running codes in the CCU overnight and will be paged by the OA
● Overnight Cardiology Fellow: There is now an in-house 1st or 2nd year cardiology fellow who covers the CCU and
SDU in addition to seeing consults on the floors/ED and performing STAT TTEs. The fellow should be first call for
issues with Blue team patients and can help mobilize other cardiology resources as needed (i.e., cath lab, temp wires,
etc.) and guide outreach to other subspecialists overnight.
● Access Fellow: Dedicated in-house fellow who covers the Ellison 11 Access Unit and is first-call for access site
complications on cardiac patients across the hospital (bleeding, suspected ischemia distal to access site, any
questions about TR bands, etc.). They will come hold pressure for access site bleeds (resident not expected to hold
pressure for prolonged periods) and will help direct further management.
● CCU Attending: The native CCU Blue attending can and should be contacted for high-level management questions
on established Blue patients, particularly if there is disagreement with the overnight fellow or if a patient is complex
(the overnight fellow most often will not know the patients). Most attendings will plan a scheduled phone check-in at
~10pm but will remain available thereafter. The Blue attending should also be contacted if needing to involve the
HCICU intensivist or Shock team overnight.
● HCICU intensivist: Available in-house overnight and can be consulted for critical care issues on Blue team patients
(need for intubation, vent/sedation issues, difficult procedures, troubleshooting mechanical support devices). Also
holds the Shock/ECMO consult pager and is responsible for Blue/Red triage decisions.
● Overnight Intensivist: In-house intensivist covering MICU boarders in the CCU (for critical care questions on CCU
Blue team cardiology patients, contact the HCICU intensivist). Available for general management questions, to
supervise procedures, to staff new MICU admissions.
● CCU Red APP: Cardiac surgery PA/NP who sits in the Red Team office near the double doors at the CCU entrance.
Can be a useful resource for questions regarding post-op patients and for help troubleshooting MCS devices.
7
Chris Marnell, Krishan Sharma
Introduction to Inpatient Cardiology
TOC
Introduction to the Ellison 10 SDU: Ellison 10 is a 36-bed cardiology unit which admits patients with primary cardiac
disorders and serves as a step-down unit for the Ellison 9 CCU. Patients in the SDU are cared for by two equivalent
housestaff teams (Red and Blue) and an EP team.
SDU Team Structures: Each team is composed of 1-2 day interns, 2 JARs, 1 SAR, and 1 attending. There is also an
embedded cardiology fellow dedicated to teaching both teams. The Blue and Red teams alternate admitting days. The
JAR admitting cycle is: long rounding – long admit – short rounding – short admit.
Overnight Support: please refer to the SDU Night Float guide for full details
● Cardiology Night Teach will see and staff all admissions performed by SDU NF interns (JAR does not have to formally
staff)
● For access site issues 🡪 Access fellow
● For acute clinical change requiring a cardiologist within 30 minutes 🡪 in-house cardiology fellows
● For EP patients 🡪 EP Fellow, they may independently request assessment by other in-house cardiology staff
8
Chris Marnell, Krishan Sharma
Introduction to Inpatient Cardiology
TOC
● Questions for pass-off: Procedure (diagnostic findings and interventions), Access (Where and what size access was
used? High stick? Do you want the sheaths pulled? When? By whom? At what PTT? Duration of bedrest? Closure
devices used? TR band in place?), Anticoagulation (What was used in the case? Do you want heparin or bivalirudin
restarted?), Antiplatelets (Was the patient loaded with a P2Y12? What should be ordered going forward?)
Complications?
● Post-procedure evaluation: Check groin yourself for expansile mass, tenderness, bruit (suggestive of
pseudoaneurysm). Document full bilateral neurovascular exam
● Key complications to watch out for: Access site bleeding, retroperitoneal hematoma, arterial pseudoaneurysm, limb
ischemia, coronary perforation resulting in tamponade
● Contingencies: For an acute access site bleed, hold pressure proximal to arteriotomy (several cm proximal to skin
puncture), page fellow who performed procedure (day) or cardiac access fellow (night)
For any procedural/access questions, first port-of-call for help should be cardiology or interventional cardiology
fellow.
9
Chris Marnell, Krishan Sharma
Introduction to Inpatient Cardiology
TOC
10
Chris Marnell, Krishan Sharma
ACS/IHD
TOC
Classification
Angina pectoris: Chest pain caused by myocardial oxygen demand outstripping myocardial oxygen supply.
● Classically: 1) Substernal, 2) Worsened with exertion, 3) Relieved by rest or nitroglycerin
● Stable angina: Occurs predictably at a certain level of exertion and is relieved with rest or nitroglycerin
Acute coronary syndrome (ACS) This is commonly subdivided into non-ST-elevation ACS (NSTE-ACS) – including
unstable angina or NSTEMI – or STEMI. Important features that can distinguish NSTE-ACS from stable CAD.
o Rest angina, which is usually more than 20 minutes in duration
o New onset angina that markedly limits physical activity
o Angina that is more frequent, longer in duration, or occurs with less exertion than previous angina
● STEMI: Designates an MI in patients with chest discomfort or other ischemic symptoms, who develop EKG changes
denoted in "Diagnosis" Section. Often denotes acutely occluded coronary artery from thrombus arising from plaque
disruption or erosion or rarely thromboembolism
● NSTEMI: MI without ST-segment elevation
● Unstable angina: ACS without biomarker elevation
History
When evaluating patients with chest pain for ACS:
● Typical ischemic chest pain: Commonly meets angina characteristics. OPQRST
mnemonic: HYPERLINK
o Onset: Gradual "https://jamanetw
o Provocation/palliation: Provoked by an activity; not changed with respiration or position ork-com.ezp-
o Quality: Substernal pressure (squeezing, burning or tightness)
prod1.hul.harvard
o Radiation: Radiates to the shoulder, neck, jaw, or either arm (may also be inter-scapular
or epigastric) .edu/journals/jam
o Site: Not felt in one specific spot, but diffuse discomfort a/fullarticle/24688
o Time course: Angina (2-5 min), 96"JAMA 2015;314(18):1955-
1965
● Atypical chest pain: Falls short of meeting all three criteria
o Common presentation of ACS, with 10% of individuals reporting dyspnea alone.
o More common in women, elderly, inferior MI, and patients with diabetes
o May rarely present with fatigue, lethargy, syncope, AMS, CVA/TIA, or GI distress
● Non-ischemic chest pain:
o Quality: Pleuritic, sharp, or reproducible with palpation
o Timing: Occurring with cessation of exercise or lasting only seconds or >6 hrs.
o Context: Without evidence of myocardial damage by biomarkers. Chest pain that is not
ischemic may represent other life-threatening etiologies of chest pain, such as aortic
dissection, PE, or pneumothorax. Alternative diagnoses should be ruled out before
disregarding chest pain.
Diagnosis
A. Physical Exam: Exam findings do not predict ACS reliably
● Signs w/ high pre-test probability for CAD: S4, Levine’s sign, signs for disease in other vascular beds
● High-risk features in ACS: syncope, new HF (S3 gallop, rales, or elevated JVP), possible mechanical complication
such as papillary muscle or ventricular septal rupture (sinus tachycardia, hypotension, elevated JVP, or new systolic
murmur)
12
Erik Dove, Peyton Hanson
Heart Failure
TOC
C. Cardiac Biomarkers
Troponin: At MGH, use a high-sensitivity troponin T (hsTnT) assay. An elevated troponin is defined as: hsTnT ≥10 ng/L (in
F) and ≥12 ng/L (in M).
● Peaks within 12-48 hours and returns to normal within 5-14 days. Reaches peak more quickly when revascularized,
more slowly when not.
● Converting numbers between assays (4th generation to hsTnT assay): for > 0.01 multiply by 1000 to get hsTNT
o For values < 0.1, remember: cTnT value of 0.01 ng/ml 🡪 hsTnT of 30 ng/L, 0.03 ng/ml 🡪 hsTnT of 53 ng/L
How to Use hsTnT:
● DO NOT wait for troponin if high suspicion for ACS (classic pain, ECG changes) 🡪 immediately start treatment
● Order troponin at presentation + 3hr repeat. Check after 1hr if worried
● A rising OR falling value (delta) ≥ 5 w/ symptoms or ECG changes 🡪 consider ACS.
● If baseline troponin >99th percentile (more common in CKD or left ventricular hypertrophy patients; mechanism of
action (MOA) not completely understood), a rise and fall of 20% is suggestive of MI. As with the general population
and hsTnT, the “delta” in troponin values is critical to determine the likelihood of an acute coronary syndrome
CK-MB: CK-MB peaks within 24 hrs and normalizes within 48-72 hrs. At MGH, CK and CK-MB are no longer used
routinely except post-PCI (or post-alcohol septal ablation) to evaluate for infarct size or when reinfarction is suspected.
D. Risk Stratification for non-ACS patients with chest pain: for patients with chest pain who do not meet above
criteria for ACS, use HEART score – estimates 6 week risk of MACE 🡪 score >3 consider for non-invasive or stress
imaging
● When compared to TIMI and GRACE, the HEART score was superior at identifying low risk patients, with
negative predictive value > 98% for MACE with 1 single troponin (Int J Cariol 2017;227:656)
13
Erik Dove, Peyton Hanson
Heart Failure
TOC
E. Non-invasive Testing
Schematic Approach to Noninvasive Cardiac Testing (adapted from UpToDate)
Stress testing
● Indications:
o Diagnose CAD: sx of stable angina in pts with intermediate-high risk of CAD. Not indicated for low risk or
asymptomatic pts
o Evaluate new or changing sx concerning ischemia in pts with known CAD
o Post-revascularization: evaluate pts with ongoing/recurrent angina or asymptomatic pt if incomplete revasc
o Pre-op risk assessment: not routinely indicated (see Perioperative Medicine)
o Newly diagnosed HF or cardiomyopathy likely due to ischemia, functional capacity (for exercise prescription),
viability testing, valvular disorders
● Contraindications: untreated ACS, MI within 2d, high risk or LM CAD, uncontrolled arrhythmia, acute CHF, recent
DVT/PE, acute myo-/peri-/endocarditis, aortic dissection, uncontrolled HTN
● Preparation: NPO 3h prior, longer if imaging or adenosine. Must reverse DNR/DNI for test. Hold caffeine >12h for
adenosine, > 24h for dobutamine.
o “Does the patient have CAD?” ⭢ hold BB and nitrates
o “How well are meds working in known CAD?” ⭢ continue BB and nitrates
● Caveats:
o Majority of vulnerable plaques are angiographically insignificant (<70% stenosis) ⭢ stress testing unable to
identify the presence of these plaques (CTA more sensitive). CTA may be helpful to rule-out LM disease.
o Angiographically significant (>70% stenosis) 3 vessel disease may produce false-negative vasodilator stress test
⭢ ”balanced ischemia”
● Positive test results: optimize medical tx. Decision re: angiography/revascularization varies by pt.
o ISCHEMIA trial: revascularization did not decrease ischemic CV events for pts with stable CAD (NEJM
2020;382:1395)
14
Erik Dove, Peyton Hanson
Heart Failure
TOC
a. Exercise Tolerance Test (ETT) Stress = exercise, Imaging = EKG, SPECT, TTE
i. ETT preferred over pharmacologic testing if pt is able to reach goal exertion (Bruce protocol or modified Bruce)
ii. Assesses exercise duration, METs, BP/HR response, HR recovery, double product (HR x SBP)
iii. Diagnostic: >85% max-predict HR (220-age), peak double product (HRxBP) >20k, HR recovery (HRpeak – HR1min
post-exercise) >12
iv. Increased probability of ischemia: # of leads with STD, degree of max STD, METs when EKG changes
occur, ventricular ectopy during recovery, increased time to recovery of EKG, failure of SBP to rise with exercise
b. Pharmacologic Stress Test Stress = pharmacologic, Imaging = TTE, SPECT, PET, or MRI
i. Choosing an agent:
1. Adenosine/Regadenoson (act via cAMP): stenosed coronary arteries are already maximally dilated, so flow
is “stolen” by healthy vessels (“coronary steal") relative perfusion deficit
a. Side effects: wheezing, bradycardia, HoTN. Caution if ACTIVE bronchospasm, high grade AVB, SSS,
severe AS
b. Regadenoson: decreased respiratory/conduction side effects, more cost-effective in obese pts. Caution if
seizure hx (reversal agent aminophylline seizure risk)
2. Dobutamine: workload induced by positive inotropy and chronotropy via β-1 receptor agonism
a. Extremely high dose of dobutamine is given, dose titrated up to 40 mcg/kg/min
b. Side effects: tachyarrhythmias. Caution if MI<48h, hx of malignant arrhythmia, severe AS, HOCM, severe
HTN, severe PAH, aortic dissection
c. Choosing an imaging modality:
i. Nuclear imaging: utilizes a radioactive tracer to detect areas of perfusion between rest and stress states. More
expensive than TTE and high amount of radiation (SPECT > PET).
ii. PET (92.6% Sn/ 81.3% Sp) is more sensitive and specific than SPECT (88.3% Sn/ 75.8% Sp) with faster image
acquisition. Less widely available and most expensive. Additional uses for imaging in rheumatologic (i.e. cardiac
sarcoid) and oncologic contexts (i.e. cardiac myxoma, metastases)
iii. TTE: Reasonable test characteristics to exclude CAD (76% Sn / 88% Sp). May be helpful for
hemodynamics/valve disorders.
1. Do not use in pts with LBBB, V-pacing or extensive wall motion abnormalities at rest.
Viability Testing
● Up to 20-40% of patients with CAD and LV dysfunction may demonstrate improved function after revascularization.
This is thought to be due to viable myocardium that lacks adequate perfusion (“stunned/hibernating myocardium”). In
contrast, necrotic myocardium is unlikely to benefit from reperfusion. Does not require stressor.
● Indication: determine viability of ischemic myocardial tissue (“hibernating myocardium”).
● Use is controversial since myocardial viability testing did not identify pts w/ a different survival benefit from CABG
compared with medical therapy alone (STITCH trial). Some argue that may have been due to type of viability study
used (i.e. SPECT)
● Imaging modalities: FDG PET, dobutamine TTE, cMRI
15
Erik Dove, Peyton Hanson
Heart Failure
TOC
Rest Imaging
● Coronary CTA: used to evaluate for presence and extent of CAD (JACC 2010;55:2663).
● Unlike stress imaging, CCTA is an anatomic assessment, not a functional assessment.
● CT-Fractional flow reserve (FFR). FFR is most validated as an invasive assessment during coronary
angiography. It can be computed on CCTA to determine the hemodynamic relevance of stenosis. FFR derived
from CCTAs closely mimic invasive FFR and may provide some functional data; however, data are limited and
there remain limitations (e.g. ostial lesions)
● Requires cardiac gating (goal HR 60-70, may need to give BB) and respiratory gating (breath hold for 5+ sec) and
vasodilators (nitroglycerin)
● Indications:
o Low to intermediate risk patients in the ED who present with new CP (i.e. to rule out ACS)
▪ Low risk pts: has high NPV (99%) for CAD rule-out (JACC 2008;52:1724)
▪ Moderate risk pts: reasonable for further risk stratification in pts at intermediate risk of CAD or pts with
equivocal stress test results
o Others: Assess patency of CABG, assess for anatomical abnormalities (e.g. course of anomalous
coronaries), CTO planning
● Findings: 2 yr. ACS risk significantly elevated if high-risk plaque (16%) and/or stenotic disease (6%)
(JACC 2015;28:337)
o Higher sensitivity and specificity for coronary stenosis compared to cMRI (Annals 2010;152:167)
● Limitations: calcified coronaries, prior stents (“blooming artifacts”)
● Coronary Artery Calcium (CAC) Scans: CT modality that provides risk assessment score (CAC or “Agatston”
Score) for coronary artery disease. Either performed alone or concurrently with coronary CTA. Non-gated, non-
contrast chest CT scans can be used to provide a qualitative estimation of CAC (Agatston score).
● Indications: To guide in decision making for primary prevention for asymptomatic adults ≥40 years of age at
intermediate risk (7.5 to 19.9 percent 10-year ASCVD risk) if statin therapy decision remains uncertain.
● Do not use: stand-alone test in evaluation of symptoms of myocardial ischemia, other high risk atherosclerosis
patients e.g. familial hypercholesterolemia.
● Findings:
o Agatston scores ≥100 or >75 percentile for age and gender independent of ASCVD risk 🡪 lifestyle changes, risk
factor modification (BP, smoking DM tx), and statin therapy. (JACC 2019;73:285)
o For those with calcium score of 0, reasonable to defer statin therapy for up to 5 years (Circ 2019; 140:496)
16
Erik Dove, Peyton Hanson
Heart Failure
TOC
Management
A. Myocardial Injury Without Ischemia
● Evaluate for non-ACS causes of elevated troponin (UpToDate)
Preventative therapy: ASA, statin, ACE/ARB (HTN, DM, HFrEF, CKD), CVD risk factor reduction, flu shot
PCI/CABG:
● Elective for refractory or severe symptoms, change in symptom severity, high-risk coronary anatomy, LV dysfunction
(PCI or CABG as appropriate)
● Mortality: No convincing evidence [ISCHEMIA trial (NEJM 2020;382), COURAGE trial NEJM 2007;356] that
revascularization improves mortality in patients with SIHD as defined by study
o ISCHEMIA excluded: LVEF <35%, LM > 50%, “unacceptable angina,” recent onset CCS III
angina/progressive angina/CCS IV angina, ACS within 2 months, PCI within 12 months, NYHA III-IV, recent
stroke, ESRD, valvular disease, many prior CABG patients.
● Symptoms: For patients w/o adequate control of anginal symptoms with optimal medical therapy PCI and CABG have
been shown to improve symptoms in two trials MASS II (J Am Coll Cardiol 2004;43) and ISCHMIA (NEJM 2020%3B
382). The ORBITA trial (Lancet 2018;391) shows no benefit in anginal symptoms with PCI, but has been highly
criticized.
C. ACS (UA/NSTEMI/STEMI)
Pharmacologic Management: All pts (w/o contraindications) should immediately receive the following:
1) ASA 325 mg, chewed/crushed
2) Atorvastatin 80 mg
3) Heparin (ACS protocol)
4) Nitroglycerin – careful in patients with low-normal SBP + large RCA MI
*Decisions regarding P2Y12 inhibitors and other medications should be had in conjunction with cardiology.
Coronary Angiography: All pts w/ ACS should undergo angiography during hospitalization - Optimize medical therapy
(see ACS – PHARMACOLOGIC MANAGEMENT), PCI (see section), or CABG (see section).
● Directly to Cath Lab:
● STEMI
● UA/NSTEMI accompanied by high-risk feature: Cardiogenic shock, refractory CP, electrical instability (frequent
NSVT, unstable SVT, or sustained VT)
● If the patient has UA/NSTEMI that does not meet criteria for immediate coronary angiography, risk stratify.
● TIMI (JAMA 2000;284:835) and GRACE (BMJ 2006;333:1091) At MGH, the TIMI Risk Score is most often used.
17
Erik Dove, Peyton Hanson
Heart Failure
TOC
● TIMI Score of ≥3 or GRACE score of >140 and high clinical suspicion for a Type 1 MI warrants an early invasive
strategy (catheterization ≤ 48hrs), though notably, the TIMCAS NEJM 2009;360 study showed that early
intervention (≤24 hrs) did not differ from delayed intervention (>36 hrs) in preventing death, MI, or stroke in
NSTEMI patients
ACS – pharmacologic management
A. Anti-Platelet
ASA
● Indication: All patients with suspected ACS
● Dose: 325 mg x1, non-enteric coated, chewed/crushed (onset of action 20 min) 🡪 81 mg qD thereafter for lifetime
● MOA: Irreversibly inhibits prostaglandin H synthase (cyclooxygenase-1) in platelets; prevents the formation of
thromboxane A2, a potent vasoconstrictor and platelet aggregator.
● Benefit: Mortality reduction and 50% RR non-fatal reinfarction
● Aspirin allergy: Consider substituting a P2Y12 inhibitor. Less common options: cilostazol, or vorapaxar
o In all cases, ASA desensitization with Allergy/Immunology is recommended when pt stable
P2Y12 Inhibitors
● Indication: STEMI, Type I NSTEMI/UA (regardless of if medical management or an invasive strategy is chosen)
● MOA: Inhibit the G-protein couple receptor P2Y12 that interact with ADP in the process of platelet activation
● Timing: institution-dependent, could delay possible CABG (7 day wash out)
o No significant benefit to pre-loading. Do not start in ACS without cardiology
● Minimum duration (shorter duration associated with worse outcomes - SMART-DATE)
o BMS: DAPT should be continued for at least 1 month (preferably 1 year)
o DES: DAPT should be continued for at least 12 months in patients who underwent PCI for ACS and 6-12
months for patients who underwent PCI for stable ischemic heart disease
o Medical management: at least 1 year
o Post-fibrinolysis: 1 year
o Shorter DAPT durations may be safe in specific circumstances (TWILIGHT, STOPDAPT-2, ONYX ONE)
● Prasugrel (TRITON-TIMI38) or ticagrelor (PLATO) preferred over clopidogrel in ACS. Clopidogrel preferring patients
with high-bleeding risk.
● Continuing DAPT beyond 1 year: Continuation of clopidogrel for longer may be reasonable (Class IIb, LOE A) in pts
who have tolerated minimum course of DAPT w/o a bleeding complication and who are not a high bleeding risk.
o DAPT Trial: continued DAPT reduced rates of stent thrombosis & MACE, but inc rate of mod/severe bleeding.
o PEGASUS-TIMI 54: In patients with MI in the prior 1-3 years, an extended regimen of ticagrelor plus ASA
significantly reduced the risk of CV death, MI
o Can use DAPT score to predict combined ischemic & bleeding risk and guide decision making on continuation
past 6-12 months (http://tools.acc.org/DAPTriskapp/#!/content/calculator/)
o No data to continue DAPT beyond 36 months
● Continuing DAPT less than 1-3 months: pts receiving specific types of DES, high-risk bleeding, check with cardiology
● ACS discharges: always send 1 year of P2Y12 to pharmacy prior to d/c to ensure supply + check coverage
Clopidogrel:
● Dose: 300-600 mg loading dose + 75mg daily
o 600 mg dose vs 300 mg dose: Maximal platelet inhibition is reached within 2 hrs w/ a 600 mg loading dose vs.
15-24h w/ a 300 mg loading dose. This correlates with better outcomes both in those with STEMI (
HORIZONS-AMI, CURRENT-OASIS) and NSTE-ACS (ARMYDA 2) who underwent PCI
● Benefit: Decrease in the primary end point of CV death, non-fatal MI, stroke (PCI-CURE, PCI-CLARITY)
● Important considerations:
o CYP2C19 genotype variants: poor metabolizers of clopidogrel with decreased response based on high
platelet reactivity (unclear relationship to CV events and bleeding). These individuals are at higher risk of
in-stent thrombosis with clopidogrel. Switch to another agent.
▪ Consider genetic testing if patient presents for in-stent thrombosis.
o Generic, much less expensive than other anti-platelet choices; remains the treatment of choice for many
Ticagrelor:
● Dose: 180 mg load + 90 mg BID maintenance
● Pharmacokinetics: Reversible binding (faster acting), shorter duration, BID dosing. Does not require activation by
CYP450 system
● Evidence/Benefit: Compared to clopidogrel: improves primary end point of MI, stroke, vascular death w/o significant
increase in non-CABG related bleeding; reduces in stent thrombosis (PLATO)
18
Erik Dove, Peyton Hanson
Heart Failure
TOC
o Consider in ACS/post-PCI
o Consider in those individuals who have had in stent thrombosis on clopidogrel (“non-responders”)
● Important considerations:
o Side effects: Associated with the development of dyspnea and more rarely, heart block, potentially related to
inhibition of adenosine metabolism. Higher bleeding risk than clopidogrel.
o After 12m, consider ticagrelor 60mg BID for maintenance (or clopidogrel 75mg daily) (PEGASUS-TIMI54)
Prasugrel:
● Dose: 60mg loading dose, followed by 10mg daily
● Pharmacokinetics: Prodrug with more potent inhibitor of platelet aggregation than clopidogrel
● Evidence/Benefit: Reduction in CV death, MI, stroke compared to clopidogrel but high major and life-threatening
bleeding (TRITON-TIMI 38). No significant differences in outcomes compared to ticagrelor (PRAGUE-18
● Important considerations:
o Contraindicated if prior TIA/CVA, weight < 60 kg, or >75 years old
o No role in pre-cath lab management, or long-term medical management
Cangrelor:
● Dose: 30 mcg/kg bolus followed by 4 mcg/min/kg for PCI
● Pharmacokinetics: Intravenous; short half-life (3-5 min)
● Evidence/Benefit: More effective in patients prior to undergoing urgent or elective PCI than clopidogrel in reducing
death, MI, and ischemia-driven revascularization at 48 hours; also, less in-stent thrombosis compared to clopidogrel;
similar rates of severe bleeding (CHAMPION-PHEONIX)
● Important considerations: May be used in pts awaiting CABG versus high-risk PCI eval. Dosing: 0.75 mcg/kg/min)
(BRIDGE)
Switching between P2Y12 inhibitors: Either for cost or bleeding risk (de-escalation, switching to clopidogrel) or need to
switch to a more potent agent (escalation, switching to ticagrelor), switching between P2Y12 is sometimes necessary. The
most common switches are:
● Switching from ticagrelor to clopidogrel: 24 hours after ticagrelor dose, start clopidogrel load
● Switching from clopidogrel to ticagrelor:
o Acute/early phase (<30 days): 180 mg ticagrelor irrespective of when the last clopidogrel dose was, ticagrelor
maintenance dose given ~24 hours after last clopidogrel dose.
o >30 days: 90 mg ticagrelor 24 hours after maintenance dose
Ezetimibe
● Indication: patients with MI who have an LDL >70 despite high dose statin
● Dose: 10 mg daily
19
Erik Dove, Peyton Hanson
Heart Failure
TOC
● MOA: LDL-C lowering through inhibition of an intestinal enterocyte cholesterol transporter thus reducing dietary
cholesterol uptake and ultimately promoting hepatic LDL uptake
● Evidence/Benefit: In patients with recent ACS (prior 10d), addition of ezetimibe to a moderate intensity statin is
associated with a reduction in CV mortality, major CV event or non-fatal stroke when compared to statin therapy
alone. No reduction in all-cause mortality or stroke. Of note, patients already on a high-potency statin were
excluded (IMPROVE-IT).
Icosapent Ethyl
● Indication: Consider for use in addition to maximally tolerated statin therapy in patients with triglyceride levels ≥150
mg/dL and either established CVD or T2DM plus ≥ 2 risk factors for cardiovascular disease
● Dose: 2g BID
● MOA: reduction in hepatic production of triglyceride-rich vLDL
● Evidence/benefit: REDUCE-IT (NEJM 2019;380)
C. Beta Blockers
● Indication: Initiated within 24 hours of STEMI/NSTEMI in patients without contraindication 🡪 reduce the magnitude of
infarction, rate of reinfarction, and frequency of life-threatening ventricular tachyarrhythmias
● Dose/Route: Start with metoprolol tartrate 6.25mg PO q6h and titrate up for goal HR 55–60.
o IV beta blockers should not be routinely given to patients with NSTEMI due to inc risk of cardiogenic shock,
though can consider for HTN mgmt if no contraindication (COMMIT)
● MOA: Reduce the magnitude of infarction, rate of reinfarction, and frequency of life-threatening ventricular
tachyarrhythmias by decreasing myocardial oxygen demand and sympathetic tone. This occurs by decreasing heart
rate, systemic arterial pressure, and myocardial contractility. In addition, prolongation of diastole secondary to the
reduction in heart rate may augment perfusion to ischemic myocardium, particularly to the sub endocardium.
● Evidence/Benefit: No RCTs in STEMI pts post-PCI. Studied in STEMI pts and under certain revascularization
approaches:
o No reperfusion: 25% reduction in mortality at 1 year (however, pts did not receive statin or P2Y12 therapy)
o Fibrinolysis: No high-quality evidence
o PCI: Conflicting data. One observational study showed significant mortality benefit (JACC 2014;7(6)), others
have not (Am J Cardiol 2015;115(11))
● Contraindications (CI): Signs of HF. Relative CI: asthma/reactive airways, PR > 240ms, 2 nd or 3rd degree heart block
D. Nitroglycerin
● Indication: Ongoing ischemic discomfort.
o IV nitroglycerin is indicated within the first 48 hours after NSTEMI for relief of ongoing ischemic discomfort,
control of hypertension, or management of pulmonary congestion.
o IV nitroglycerin should not preclude other mortality-reducing interventions, such as beta-blockers and/or
ACEI. Symptom reduction, not mortality benefit.
● Dose: Sublingual 0.4 mg q5m x3 dose; assess for need of IV if ongoing CP
● MOA: Induces smooth relaxation and vasodilation in peripheral veins and arteries 🡪 reduces cardiac oxygen demand
by decreasing preload, with modest effects on afterload and dilation of coronary arteries
● Contraindications: SBP < 90 or ≥ 30 mm Hg below baseline, suspected RV infarction (V4R elevation), or patients who
have received a PDE-5 inhibitor (e.g. sildenafil, tadalafil) within 24h
● Side effect: headache, may limit IV titration
20
Erik Dove, Peyton Hanson
Heart Failure
TOC
● Dose/Agent: Can start with short-acting captopril for titration and convert long-acting agent prior to discharge. ARBs
with fewer side-effects
● MOA: Attenuate the remodeling of the myocardium, preservation of ischemic preconditioning, reduction of recurrent
MI and ischemia (through reducing afterload and mitigating vasoconstriction induced by angiotensin II) and reduction
in sudden cardiac death through decreased sympathetic activity and reduced ventricular remodeling
● Evidence/Benefit: ISIS-4, CONSENSUS-II, AIRE, SAVE
● Contraindications: Hypotension (SBP < 100 mm Hg or > 30 mm Hg below baseline)
F. Anticoagulation
● Indication: IV anticoagulation for all patients with NSTEMI/STEMI without contraindication.
● Agent/Dose:
o Heparin: Unfractionated heparin (at a dose of 60 U/kg bolus followed by 12 U/kg/hr drip, titrating to goal PTT
50–70) is the anticoagulant of choice, especially if the patient will proceed to catheterization as the activated
clotting time (ACT) can be followed during the procedure, leading to lower bleeding rates
o Bivalirudin, Argatroban: Reserved for patients with HIT
o Enoxaparin: Dose is 1 mg/kg BID. For patients in whom a conservative strategy is selected, Class IIa
recommendation to select enoxaparin or fondaparinux over UFH. This is based on evidence of decrease in
primary composite end-points as compared to heparin (ESSENCE, TIMI11B, and Phase A to Z trials). Of
note, enoxaparin resulted in higher rates of bleeding in patients age > 75 years, though the rate of bleeding
was greatest when patients were transitioned from LMWH to UFH or vice versa (SYNERGY). Prefer last
dose 8h prior to coronary angiography and possible PCI.
● If on existing oral AC: if warfarin, do not start until INR < 2; if DOAC, consult with pharmacy.
● MOA: Treat/stabilize intra-coronary thrombus and thrombogenesis that leads to acute reduction in blood flow
● Length of therapy:
o After PCI: Stop unless pt has another indication for anticoagulation (see triple therapy below)
o Medical management: If PCI is not performed (but CAD seen on angiography), can consider continuing:
▪ Intravenous UFH for at least 48 hours or until discharge; or
▪ Enoxaparin or fondaparinux for duration of the hospitalization, up to 8 days; or
▪ Bivalirudin 0.25 mg/kg per hour for up to 72 hours
o Heparin rebound syndrome: D/C’ing UFH results in a brief period of hypercoagulability during which
patients may experience worsening angina (NEJM 1992;327(3))
▪ Managed through the use of ASA or progressive weaning of UFH drip
▪ In patients who cannot receive ASA, another antiplatelet should be utilized consider UFH wean
● Triple Therapy: Ongoing concurrent DAPT and oral anticoagulant therapy increase the risk of bleeding with no
significant difference in MI, cardiac death, thrombotic events (WOEST, ISAR-TRIPLE)
● MGH common practice: treat with triple therapy (ASA/clopidogrel/AC) for 1-4w and subsequently discontinue
ASA, continuing AC and clopidogrel (preferred over ticagrelor/prasugrel due to increased bleeding risk)
● DOAC is preferred over warfarin in most patients (reduced bleeding risk, no lab monitoring). The following
DOACs have been studied with antiplatelet therapy in atrial fibrillation:
o Dabigatran 150 mg BID (RE-DUAL)
o Apixaban 5 mg BID or 2.5 mg BID in pts w/ at least two of the following: age ≥80 years, body weight ≤60
kg, or serum creatinine ≥1.5 mg/dL (AUGUSTUS)
o Low dose rivaroxaban (15 mg qPM) was studied in PIONEER-AF-PCI RCT trial but was underpowered to
exclude increased risk of stroke therefore some still use the suggested 20 mg qPM for stroke prevention
o Edoxaban 60 mg daily (ENTRUST-AF PCI); however when compared to warfarin + clopidogrel,
edoxaban does not reduce bleeding risk.
● If pt needs triple therapy, give PPI or H2 blocker for GI ppx (PPI may interact with clopidogrel. No clinical impact
suggested, but study terminated early. Expert consensus recommendation).
● Mechanical heart values: DOACs deemed contraindicated due to an association of worse outcomes in this cohort
(RE-ALIGN), <10% of pts included in WOEST and ISAR-TRIPLE studies had mechanical heart valves.
G. Oxygen
● Indication: O2 should be used to maintain SaO2 > 90%. Avoid routine use of supplemental O2 in cardiac patients
without hypoxemia as it is not beneficial and may be harmful (Circulation 2015;131(24))
● Evidence/Benefit: No difference in rate of all-cause mortality or rehospitalization in 1 year when comparing patients
with SaO2 > 90% randomized to supplemental O2 vs ambient air (DETO2X-AMI)
H. Morphine
● Indication: almost never 🡪CP unresponsive to NG gtt is an indication for urgent intervention. Morphine can be used
in refractory pain en route to cath.
21
Erik Dove, Peyton Hanson
Heart Failure
TOC
I. Fibrinolysis
● Indication: STEMI pts who cannot receive primary PCI w/in 120 mins of first medical contact (Catheter Cardiovasc
Interv 2013;82(1)). In UA/NSTEMI there is no benefit of fibrinolysis (possible harm).
● Timing: Time from hospital arrival to initiation of fibrinolysis should be < 30 min. Fibrinolysis has not improved
outcomes in patients who present > 12 hrs after symptom onset and thus is not recommended for patients who are
stable or asymptomatic.
● P2Y12 inhibitors: Patients receiving fibrinolysis benefit from pretreatment with clopidogrel but not GpIIb/IIIa inhibitors.
Ticagrelor may be safe with regards to bleeding (TREAT)
22
Erik Dove, Peyton Hanson
ACS/IHD
TOC
A. Access
Most commonly transradial (TRA) or transfemoral (TFA) approach
● The RIVAL trial demonstrated that TRA was non-inferior to TFA in terms of death, MI, stroke, and non-CABG related
bleeding; in STEMI subset, TRA was associated with a 30-d all-cause mortality benefit1
● Strong evidence to suggest ↓ rates of bleeding and ↓ vascular complications with TRA vs TFA
● AHA endorses a “radial-first” strategy for PCI in ACS2
Radial Access: After catheterization, a clear plastic band (TR Band) with an inflatable pressure pad is secured around the
access site. The sheath (catheter through which procedure was performed) is withdrawn and the inflatable pressure band
is adjusted to prevent access site bleeding, while allowing sufficient blood flow to transport hemostatic factors to the hand
(“patent hemostasis”). There is a specific nursing protocol to deflate and remove the band when hemostasis achieved (at
MGH 2h for diagnostic procedure or 4h post-PCI).
● TR Bands at MGH are managed by the cardiac access fellow, page if any concern for bleeding/expanding hematoma
Femoral Access: After catheterization, the FA sheath can be removed when the activated clotting time is <150-160sec.
Manual pressure is applied and the sheath is removed with ongoing pressure for hemostasis (timing determined by size of
sheath). Vascular closure devices (e.g. Perclose™ > AngioSeal™) may be used in the Cath Lab in fully anticoagulated
patients, patients who have difficulty lying flat and other select scenarios to achieve faster hemostasis. The size of the
sheath should be noted post-catheterization as it correlates with bleeding risk.
Standard Views
24
Erik Dove, Peyton Hanson
Heart Failure
TOC
25
Erik Dove, Peyton Hanson
Heart Failure
TOC
Instant Wave Free Ratio (iFR): adenosine independent resting-pressure derived index. Non-inferior to FFR (iFR-
SWEDEHEART trial)
● Cutoff < 0.89 suggestive of physiologically significant disease and reasonable to consider intervention
● See Fig. 2 for algorithm: https://www.acc.org/latest-in-cardiology/articles/2017/05/25/08/34/ffr-in-2017-current-status-
in-pci-management
Intravascular Ultrasound (IVUS): can assist evaluation of left main disease and/or PCI planning (by measuring vessel
diameter, lesion length and calcification, as well as confirm stent expansion).
● Use of IVUS supported by meta-analyses suggesting reduced MACE, mortality, and complications with IVUS vs.
angiography alone.
● Optical Coherence Tomography (OCT) has higher spatial resolution than IVUS but less depth penetration. Data
suggest equivalence to IVUS; trials ongoing.
D. Aspiration Thrombectomy
2015 ACC/AHA guidelines recommend against aspiration routine thrombectomy in patients undergoing PCI for STEMI.
This is based on three large trials (TOTAL, TASTE, TAPAS) and the subsequent meta-analysis, that did not find improved
outcomes with this strategy.
E. Stent Placement – Bare Metal Stent (BMS) vs Drug Eluting Stent (DES)
BMS: Consider in pts w/ high bleeding risk, need for invasive procedures, or difficulty with adherence to 1yr DAPT
● More likely to develop early in-stent restenosis due to re-epithelization within the first 30 days
● Requires DAPT for at least 1 month (preferred 1 year)
● With reduction in stent thrombosis in newest generation of DES and short-DAPT regimens in high-bleeding risk
individuals, BMS are rarely used.
DES: Contain anti-proliferative drug and polymer that serves as vehicle for the drug and controls the rate of release. The
drug inhibits the growth of the neointima, which is the major cause of restenosis. Associated with increased rate of late
(>30 day) stent thrombosis due to prolonged foreign body exposure
● Requires DAPT for at least 6 months in SIHD (preferred 1 year) so theoretically increases bleeding risk
● The NORSTENT trial comparing 2nd generation DES vs. BMS found no significant difference in mortality, but noted ↓
repeat revascularization in DES group
● Emerging evidence suggests newer generation DES w/ shorter DAPT may be considered (LEADERS FREE II, ZEUS,
ONYX-ONE)
26
Erik Dove, Peyton Hanson
Heart Failure
TOC
27
Erik Dove, Peyton Hanson
Heart Failure
TOC
Post-Cath Care
Pass-off Essentials in SDU/CCU:
● Type of access used for procedure (Arterial v Venous, Femoral v Radial v Jugular)
● Sheaths: What sheaths are in place, by whom they should be removed, removed in post-cath holding area vs. if
closure device used (relevant for bedrest), for how long should bedrest be?
● Medications in cath lab (ex. Anti-platelet loads)
● Drips (heparin, eptifibatide, cangrelor, etc) and parameters to stop
● Planned course for anti-platelet agents & a/c, especially P2Y12 (P2Y12 ordered prior to transfer by cardiology fellow).
● Findings of procedure and devices/stent details (DES v BMS, size, location)
● Complications of procedure
28
Erik Dove, Peyton Hanson
Heart Failure
TOC
B. Bypass Grafts
Arterial: Arterial grafts >> long-term survival compared to vein grafts: >90% patency rate at 10 years, ↑ survival, ↓ MI, ↓
less recurrent angina, ↓ reoperations; arterial ↑ grafts difficulty to harvest compared to SVG so may not be used in
emergent situations
● Internal Thoracic Arteries (ITA) [LIMA/RIMA]: Native take off from the subclavian with end-to-side anastomosis to
coronary, limited by vessel length to proximal touchdown on coronaries (for bypassing proximal stenosis)
o ITA grafts confer 20 yr. survival benefit compared to SVGs alone
o LIMA anatomically favorable for LAD, so most common graft
o RIMA technically challenging, increases pump time; can be used in-situ to bypass RCA or OM w/ critical stenosis
(>90%) or in Y-graft with LIMA to other target vessels.
o Prefer to avoid bilateral ITA in poorly controlled diabetics due to concern for sternal wound healing
● Radial Arteries: 80-85% patency at 5yrs; ↑ patency and ↓ adverse cardiac events compared to SVGs31
o Can bypass severe (>70%) L sided stenosis or critical (>90%) R sided stenosis
o Safe harvest, sometimes numbness/tingling in hand ipsilateral to harvest but generally no motor compromise
● Gastroepiploic Arteries: 15 yr. patency rate >92%, rarely used
Venous: SVG 5-7 yr. patency ~50%
● Requires ASA within 48hrs s/p CABG, continued indefinitely
● Smoking, HTN, HLD, DM ↑ risk for graft failure
Key Points:
● Arterial grafts ↑ patency compared to SVGs; may not be limited to RIMA/LIMA with increased radial artery grafting
● Arterial grafts ↑ difficulty to harvest compared to SVGs, so less conducive in emergencies
D. CABG vs PCI
Syntax Score: Developed for SYNTAX Trial, which randomized patients with 3VD or LMCA to CABG vs PCI
● Syntax Score is a semi-quantitative tool based on coronary angiography that grades anatomic complexity
● ↑ Syntax Score = better outcomes with CABG, while ↓ Syntax Score = non-inferior outcomes compared to PCI
● Multidisciplinary Heart Team meetings at MGH between cardiothoracic surgery, interventional cardiology, and primary
team to decide intervention in complex situations
Specific Considerations:
LMCA Disease: Historically CABG was standard for LMCA disease, some recent randomized trials showing PCI with
DES may be reasonable for certain patients but the data are mixed (SYNTAX, PRECOMBAT, NOBLE, EXCEL, MAIN-
COMPARE)
DM w/ limited overall CAD: No difference in 5yr survival, but rate of MACE was lower in CABG (BARI-2D)
DM w/ advanced CAD: CABG is superior to PCI in the management of patients with DM2 and multivessel disease with
respect to prevention of MI and death, although at a cost of a higher risk of stroke (FREEDOM)
29
Erik Dove, Peyton Hanson
Heart Failure
TOC
CABG + OMT vs. OMT alone in Ischemic Cardiomyopathy: All-cause mortality, cardiovascular mortality and
hospitalization for cardiovascular causes were significantly lower in patients who underwent CABG + medical therapy
than those who received medical therapy only. Benefit seen over time; at least 5 year-survival (STICHES)
Post-MI Complications
30
Erik Dove, Peyton Hanson
Heart Failure
TOC
31
Erik Dove, Peyton Hanson
Heart Failure
TOC
Electrical Complications:
● Bradyarrhythmia/conduction block: may be due to coronary artery occlusion (see below) or Bezold-Jarisch reflex
(Anes 2003;98:1250)
● Tachyarrhythmia: related to creation of re-entrant circuit from scar formation and/or ⭡ automaticity from adrenergic
32
Erik Dove, Peyton Hanson
Heart Failure
TOC
33
Erik Dove, Peyton Hanson
Heart Failure
TOC
Special Populations
34
Erik Dove, Peyton Hanson
Heart Failure
TOC
Microvascular Angina
Definition: Symptoms of angina + signs of ischemia on non-invasive testing, but on evaluation of epicardial coronary
arteries, no obstructive coronary disease
Epidemiology: More likely to be younger and female, more likely to have CV risk factors than the general population
Clinical Presentation: Some patients present with ACS and then are diagnosed with MINOCA. Others have stable
coronary disease that get the diagnosis after persistent and severe symptoms lead to coronary angiography without
evidence of obstructive CAD
Pathophysiology: Various mechanisms proposed, but it has been shown that these patients have higher sensitivity to
vasoconstrictor stimuli and less ability to vasodilate. Other mechanisms include endothelial dysfunction, luminal
obstruction (microthrombi), vascular remodeling, extramural compression, pain sensitivity. Can coincide with other CV
conditions, such as HCM, LVH, aortic stenosis due to one of the many mechanisms proposed
Diagnosis: Stress testing, PET-stress in particular can assess coronary flow reserve/microvascular disease. Coronary
angiography +/- provocative testing.
During angiography:
● Coronary Flow Reserve (CFR): More commonly measured by PET-stress. Can administer intra-coronary
vasodilator and measure the flow reserve during coronary angiography. If the CFR is low (<2-2.5) and there is no
epicardial disease, microvascular dysfunction can be diagnosed
● Provocative testing with Ach: diagnostic if have symptoms + EKG changes without evidence of epicardial disease
Non-invasive
● Fractional flow reserve using CT, PET, or CMR
Treatment:
● Risk factor modification
● Aspirin and statin if have any evidence of atherosclerotic disease
● Anti-anginal: as listed in SIHD
● ACE-I: There is some evidence of benefit. For example, in the WISE trial, women who had received quinapril had
improved CFR after 16 weeks. They also had improved symptoms of angina
● Imipramine: Small study showed 50% reduction in chest pain
35
Erik Dove, Peyton Hanson
Heart Failure
TOC
A. Pre-discharge consults
Once an ACS patient is admitted to the SDU or CCU, they should be admitted using the E10/E11 admission template,
which will standardize their admission orders. Included in that template are consults for:
● PT, Nutrition, smoking cessation (~50% reduction in subsequent cardiac mortality; Arch Intern Med 2000; 160: 939-
44)
● Cardiac rehab (it is cost effective and associated with improvement in clinical outcomes; 20-26% lower mortality rates
in modern era of secondary prevention; Am J Med. 2004; 116:682–92)
● Vaccines: Influenza & Pneumococcal vaccination
o All patients with CVD, regardless of age, should receive PSV23. (For pts previously vaccinated, a
minimum 5-year interval is recommended)
● ***Please make sure that these are ordered prior to the day of discharge.***
B. Assessment of LV function
● Class I recommendation: All patients should have a TTE performed pre-discharge in order to evaluate LV function if
they did not have a ventriculogram performed during catheterization.
● In patients with significant LV systolic dysfunction, a post-discharge plan for re-evaluation ≥ 40 days later should be
made, especially to address potential need for ICD therapy after allowing for recovery from myocardial stunning.
D. Management of Diabetes
● Goal: HbA1c is < 7%. Less stringent goals may be appropriate for some patients.
● SGLT-2 inhibitors: SGLT2-inhibitors empagliflozin, canagliflozin, dapagliflozin decrease cardiovascular mortality and
morbidity in T2DM patients who have CAD/high CVD risk factors (CANVAS, EMPA-REG OUTCOME, subanalysis of
DECLARE-TIMI 58)
o Adverse events: CANVAS showed increased risk of amputations, which occurred more often in those with
prior history of PAD or prior amputation. Similar data has not been seen in EMPA-REG OUTCOME
● GLP-1: liraglutide lowered cardiovascular mortality and all-cause mortality (LEADER)
36
Erik Dove, Peyton Hanson
Heart Failure
TOC
o If you still feel chest pain, take a third tablet and wait 5 more minutes.
o If you rarely require nitroglycerin or if you feel chest pain 5 minutes after taking the third pill, call 911.
o Always tell your doctor if you had to take this medicine
o Keep medicine in the original bottle in a dark, dry place and check the expiration date often.
o If you need to open the bottle, write the date you opened it in permanent marker.
o The bottle is good for only 6 months after it is opened.
● NSAIDs: Patients who routinely took nonsteroidal anti-inflammatory drugs (except for aspirin) before NSTEMI should
discontinue these agents because of increased risks of mortality, reinfarction, hypertension, heart failure, and
myocardial rupture (Class III Recommendation: Harm)
F. Follow up
● Appointments: The patient should be scheduled to see a cardiologist or their PCP within one month of discharge. For
patients who do not have a primary cardiologist, they should be referred to the MGH MI clinic or will be set up with the
PDW fellow on service. They should also be given a phone number to call for their cardiologist (even if an
appointment is not scheduled at the time of discharge) so that they can call if they have symptoms.
37
Erik Dove, Peyton Hanson
Heart Failure
TOC
HEART FAILURE
DEFINITIONS
Cardiomyopathy (CM): disease of myocardium. This can lead to heart failure, but is not synonymous with heart failure
● Dilated CM: dilation or impaired contraction of one/both ventricles
o Left ventricular internal diameter end diastole (LVIDd) >59mm males, >53mm females
● Restrictive CM: nondilated or small ventricle(s) with diastolic dysfunction
● Hypertrophic CM: increased LV mass, with nondilated, hypertrophied LV (IVS >15mm)
● Also: arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D); unclassified CMs
Heart failure (HF): clinical syndrome characterized by typical symptoms and signs that are caused by cardiac
dysfunction. Hemodynamically, it is a failure to provide commensurate cardiac output to the body, or in which output is at
cost of elevated cardiac filling pressures
● Heart failure with preserved ejection fraction (HFpEF) (EF ≥ 50%) accounts for 40-50% of patients with HF
● Heart failure with reduced EF (HFrEF) (EF≤ 40%) accounts for 40-50% of patients with HF
● Heart failure with midrange EF (HFmrEF) (EF 41-49%) accounts for approximately 10-20% of patients with HF
Contractility/Inotropy: inherent capacity of the myocardium to contract independent of changes in preload or afterload.
● ↑ contractile function a/w enhanced ventricular relaxation (lusitropy),
● Frank-Starling curve relates ventricular EDV (preload) to contractility
o ↑ in force of contraction as EDV ↑ with increasing sarcomere length and optimization of the overlap between
actin + myosin filaments
o There is a limit to this optimization after which LVEDV / EDP will increase without additional increase in SV
38
Smirthi Sukumar
Heart Failure
TOC
Symptoms Signs
Shortness of breath Sensitivity: 84-100% S3 gallop Sensitivity: 13%
Specificity: 17-34% Specificity: 99%
Edema, abdominal Sensitivity: 23% Elevated JVP > 6cm Sensitivity: 39%
distention, RUQ pain Specificity: 80% Specificity: 93%
(right sided HF)
Crackles Sensitivity: 60%
Specificity: 78%
Hepatojugular reflex Sensitivity: 24%
Specificity: 96%
39
Smirthi Sukumar
Heart Failure
TOC
Ascites Sensitivity: 1%
Specificity: 97%
▪ Not required for every patient with known HF. Useful in evaluating progressive valvular ,
ischemic, or pericardial disease
▪ Most accurate assessment (especially of RV/LV function and valvular pathologies) is at euvolemia, in
sinus rhythm and at normal rates
CLASSIFICATION
General classification: ACC/AHA, Stages A-D. Grades based on structural changes and symptoms
Functional classification: NYHA, Functional Classes 1-4. Grades severity of symptoms and exercise tolerance
● Since this captures symptomatic only, lowest NYHA grade starts at equivalent point to ACC/AHA B (see graphic)
Severe/decompensating: INTERMACS, Profiles 1-7. Gradations scale from NYHA III (7) to critical cardiogenic shock (1).
● Most commonly applied to advanced HF patients admitted to CCU/CS-ICU, not used in terminology as much day-to-
day as other classifications above
ETIOLOGY
In general, when describing patients with the clinical syndrome of heart failure, try to delineate the type of heart failure
from the underlying condition that led or put them at risk of that syndrome, if identifiable. Example: 62 year old male with
HFrEF (EF 32%) and ischemic cardiomyopathy
You can have heart failure syndrome without a cardiomyopathy, as cardiomyopathy refers only to the disease of
the heart muscle.
41
Smirthi Sukumar
Heart Failure
TOC
42
Smirthi Sukumar
Heart Failure
TOC
ADJUNCT THERAPIES
VASODILATORS ● V-HeFT, A-HeFT
Hydralazine 75mg TID ● Isosorbide mononitrate (Imdur) has not been studied and
Isosorbide dinitrate 40mg TID is not recommended according to ACC/AHA guidelines.
If-CHANNEL BLOCKERS ● SHIFT, BEAUTIFUL, SHIFT-HF
Ivabradine 75 mg TID ● Titrate to HR 50-60 BPM; indicated for patients in sinus
rhythm with HR >70bpm at rest once BB therapy is
maximized
LOOP DIURETICS ● IV > PO for ADHF, can function as a vasodilator and
Furosemide 40-240 mg QD decreases RA and PCWP; starting dose is typically given
Torsemide 10-200 mg QD as 2-2.5 times home PO dose; start with intermittent bolus
Bumetanide 1-5 mg QD and consider continuous infusion if congestions is
refractory to boluses
● Conversions: 40 mg PO Lasix = 20 mg IV Lasix = 20 mg
PO torsemide = 1 mg IV/PO Bumex
THIAZIDE DIURETICS ● Useful in refractory diuresis. Administer 30 minutes before
Hydrochlorothiazide 12.5-100 mg QD loop diuretic for maximum effect.
Metolazone 2.5-10 mg QD
ICD ● SCD-HeFT, MADIT-II, DEFINITE
● Primary prevention of sudden cardiac death in patients with non-ischemic
dilated cardiomyopathy or ischemic heart disease at least 40 days post MI with
EF of 35% or less and NYHA class II-III symptoms on GDMT with expectation
of at least one year survival
● MADIT-CRT, CARE-HF, MIRACLE, RAFT
● CRT is indicated for patients with LVEF 35% or less, sinus rhythm, LBBB with
CRT QRS duration of 150ms or greater and NYHA Class II-IV symptoms on GDMT;
can be useful for patients with reduced EF and LBBB pattern with QRS 120-
149ms or non-LBBB pattern with QRS duration of 150ms or greater
● CRT should also be considered in patients on GDMT with LVEF 35% or less
who are undergoing device implantation with expectation of ventricular pacing
>40% of time
Iron ● FAIR-HF, CONFIRM-HF, IRONOUT-HR, RED-HF, AFFIRM-AHF
● IV iron repletion (ferritin 15-100 or 100-299 with transferrin saturation < 20%)
improves NYHA functional status, 6-min walk test and several quality-of-life
assessments. These improvements not seen with PO iron
44
Smirthi Sukumar
Heart Failure
TOC
HFpEF
Diagnosis may be challenging given normal EF and non-specific signs and symptoms.
● The H2FPEF score was developed to help distinguish HFpEF from other causes of dyspnea.
● European Society of Cardiology (ESC) has developed the HFA-PEFF diagnostic algorithm to diagnose HFpEF.
● For inpatients, RHC (+/- provocative maneuvers such as exercise) should be considered
HFpEF Treatment
● HFpEF patients often elderly and/or highly symptomatic, ∴ aim of therapy 🡪 alleviate symptoms and improve QoL
o SGLT2i (EMPEROR-PRESERVED trial). Improved outcomes for patients w/ and w/o diabetes
o +/- spironolactone (controversial TOPCAT trial)
Diuretics, blood pressure control, address underlying valvulopathy/arrhythmia/ischemia
Cardiogenic shock (CS) is shock [tissue hypoperfusion] secondary to reduced cardiac output of a primary cardiac
etiology. The SCAI stages of CS (A-E) can aid in the timely diagnosis and management.
46
Smirthi Sukumar
Heart Failure
TOC
● Tailored therapy: refers to use a pulmonary artery catheter (PAC) to obtain intracardiac pressures and
hemodynamic measurements to guide therapy.
o PAC (or Swan-Ganz): quad lumen catheter; RIJ, occasionally L 🡪 RA 🡪 RV 🡪 R or L main PA
o Uses: Determine etiology of shock, tailored treatment
o Complications:
▪ Infection/PTX/bleeding, arrhythmia, PA rupture, IJ injury/erosion (long term); pulm. infarct; PE
o Relative CI/indications for cath lab placement:
▪ LBBB: at risk for developing RBBB and then complete heart block
▪ ICD or PPM placed within the last 6 months; temp. wire
▪ Valves: prosthetic/stenotic, or severe tricuspid regurgitation (challenging); TV or pulmonic valve IE
▪ Severe pHTN: systolic PA pressures > 70 mmHg: ↑ risk PA rupture
▪ R. heart mass (thrombus, tumor)
o Placement: assess with waveform & CXR – tip at edge of middle 1/3 of thorax, ideally inferior to level of LA on
CXR (West Zone 3 in V/Q map of lung
47
Smirthi Sukumar
Heart Failure
TOC
CVP/PCWP waveform
a-wave: atrial contraction, lost in AF/flutter, increased in tricuspid/mitral
stenosis, cannon a-waves in junctional, VT and CHB due to contraction
against closed TV/MV
c-wave: closure of TV/MV
v-wave: rapid filling of atria, TR/MR causes fusion of c and v waves into
one wave (called cv-wave) and blunted x descent
x-descent: atrial relaxation
y-descent: early ventricular filling
49
Smirthi Sukumar
Heart Failure
TOC
Acute right ventricular failure: characterized by elevated right heart filling pressures in the setting of acute pulmonary
hypertension (acutely elevated RV afterload).
● The RV is an embryologically, histologically and functionally different structure to the LV. It is more fibrous, with
significantly less muscle mass and capability to functionally compensate for changes in pressures or volumes.
o In the setting of acutely elevated afterload (PA pressure), the RV compensates initially by dilating –
additional pressure is dissipated by creating greater volume
▪ Unlike the LV, additional pressure does not increase RV stroke volume as effectively
▪ As pressure/volume increases, this leads to increased RV wall tension and flattening of the
interventricular septum (IVS)
o The coronary arteries fill during times of low wall tension; as RV WT , myocardial perfusion decreases 🡪
contractility 🡪 RV output
o The same pressure changes that lead to RV WT lead to progressive flattening 🡪 bowing of IVS towards
and into LV
▪ This reduces LV EDV 🡪 reduced preload 🡪 reduced overall cardiac output
▪ Reduced RV output also not providing as adequate preload
o State of low cardiac output 🡪 systemic HoTN 🡪 acidosis, inflammation 🡪 pHTN, coronary O2 delivery
o This type of cardiogenic shock and spiraling deterioration is known as the RV death spiral
50
Smirthi Sukumar
Heart Failure
TOC
Indications Contraindications
Refractory cardiogenic shock (CPO < Anoxic Brain Injury
0.6W, CI < 2.2L/min/m2 or elevated lactate Irreversible End Organ Failure
despite pharmacologic therapy) Prohibitive Vascular Access
High risk interventional procedures DNR
● Type of MCS device is determined by indication, INTERMACS profile, predominant ventricle involved (left, right or
both) and presence of hypoxemia
● If concern for worsening cardiogenic shock on pharmacotherapy, the CARDIOGENIC SHOCK team (staffed by
HCICU Attending) should be paged at 29151 for early consideration of MCS.
● As IABP is the main therapy residents are exposed to, this will be discussed in detail next. Please see chart below for
brief description and comparison of other available MCS options.
B. Indications
Cardiogenic shock and hemodynamic instability after a
STEMI refractory to pharmacological management (Class IIa
based on findings from IABP-SHOCK II)
Mechanical complications of MI (VSD, severe MR)
Refractory ischemia (intractable angina with impaired LV
function or large territory infarct, refractory polymorphic VT)
Prophylactic placement prior to high risk procedures (PCI,
CABG)
Inability to wean from cardiopulmonary bypass after
C. Contraindications
Severe aortic regurgitation
Severe bilateral peripheral arterial disease
Aortic dissection, aneurysm, or intramural hematoma
Severe coagulopathy or sepsis
IABP Waveform
Normal: IABP inflates after AoV closes
(dicrotic notch), leading to diastolic
augmentation > than SBP. Balloon
inflation leads to a sharp and deep V at
the dicrotic notch. Deflation starts
immediately before the opening of the
aortic valve, leading to reduced end-
diastolic pressure.
52
Smirthi Sukumar
Heart Failure
TOC
E. Complications:
● Anemia and thrombocytopenia
● Insertion site complications (bleed, infection, aneurysm, fistula)
● Limb ischemia (daily pulse checks and vascular exam; requires device removal)
● Vascular injury including dissection or pseudoaneurysm (12-40%) or stroke
● Balloon rupture (suspect if blood seen in helium line; device detect, will attempt to aspirate helium from aorta to
prevent gas embolism).
Any suspected complication should prompt an immediate call to the fellow/attending or HCICU Intensivist.
F. Weaning/Removal:
● Depending on hemodynamic status and indication for device, the IABP is programmed to assist every beat (1:1) or
less often (1:2, 1:4, 1:8). Improvements 🡪 decrease cycling frequency
● Thrombosis risk increases at frequencies <1:1 (stasis) – AC required for these
● Wean stepwise, with assessment of hemodynamics, rhythm, symptoms during each phase. Before removal, return to
1:1 x hours due to risk of thrombus dislodgement
● Remove after holding heparin x hours, generally when PTT <40
● Per policy at MGH, IABPs are removed only by fellows or more senior
53
Smirthi Sukumar
Heart Failure
TOC
54
Smirthi Sukumar
Heart Failure
TOC
Devices: LT-MCS most commonly refer to ventricular assist devices (VAD) which are FDA approved to provide support to
the left ventricle (LVAD).
● NB: VADs are also used off-label for RV support (RVAD) or to support both ventricles (BiVAD). RV support
cannot be done outside of an ICU.
● Total artificial heart (TAH) is FDA approved for biventricular support but is not available at MGH.
Principles of VAD
● Blood to LV pump via inflow cannula (surgically
implanted in apex) 🡪 blood to ascending aorta via
outflow graft
● Driveline: Surgically tunneled cable that connects the
pump to an external control unit which operates and
monitors pump function (LVAD speed, flow, power,
pulsatility index). Driveline exits the body through
abdominal wall. The external controller is connected to
a power source.
● The rotor or impeller within the pump propels blood by
spinning at a high speed. Blood flow is proportional to
pump speed though the relationship is not linear. The
speed is set by the provider to ensure adequate LV
unloading. Inadequate loading may result in pulmonary
edema while excessive unloading could lead to suction
events, resulting in hypotension or arrythmias.
● Continuous flow devices do not generate a pulse. However, the interaction with native ventricular function
leads to phasic changes in blood flow through the LVAD called the pulsatility index for the HeartMate II and is
displayed as the waveform for HVAD.
● Given risk of thrombus with continuous flow devices, VADs mandate anticoagulation (heparin 🡪 warfarin)
GI bleeding Common cause of hospital readmissions both early and later. Often no intervenable
source. AC interrupted until bleeding resolves; recurrent bleeding 🡪 lower target INR.
55
Smirthi Sukumar
Heart Failure
TOC
CVA Leading cause of long-term mortality; VAD patients are at risk of both ischemic and
hemorrhagic stroke. Ischemic stroke = emboli from pump thrombosis. Hemorrhagic stroke
= due to the need for chronic AC or risk of hemorrhagic conv. of isch. NCHCT for any
acute mental status or neurologic change.
Arrythmia Burden of arrythmias is high in LT-MCS; reasonably well tolerated with low risk of
immediate hemodynamic collapse since output independent of native myocardium
Aortic insufficiency In ~10 to 53%; fusion of commissures and degenerative changes of cusps from persistent
aortic valve closure. Assessed by TTE.
RHF Risk of developing right heart failure is an important consideration in assessing
candidacy for LVAD; exacerbation of pre-existing RHF, or by changes to RV structure
(septal bowing) due to off-loading of the left ventricle. Assessment of PVR critical.
56
Smirthi Sukumar
Heart Failure
TOC
Immunosuppression: Post-transplant immunosuppression typically consists of a triple regimen that includes calcineurin
inhibitors (such as cyclosporine and tacrolimus), purine synthesis inhibitors (such as MMF and azathioprine) and
corticosteroids.
● MMF is superior to azathioprine in preventing rejection and mortality.
● Steroids prescribed in high doses initially with gradual taper starting at 6 months post-transplantation.
● Patients undergo routine cardiac biopsies to grade level of rejection post-transplant (per below table)
57
Smirthi Sukumar
Heart Failure
TOC
58
Smirthi Sukumar
Heart Failure
TOC
References
1. 2016 ESC Guidelines. PMID: 27207191
2. 2013 ACCF/AHA Guidelines. PMID: 23741058
3. 2017 ACC/AHA/HFSA Guidelines. PMID: 28461259
4. 2021 Update to ACC Guidelines. PMID: 33446410
5. Zipes, Douglas P. et al. Braunwald's Heart Disease : A Textbook of Cardiovascular Medicine. Eleventh edition.
Elsevier, 2019
6. Heart Failure: Diagnosis, Management and Utilization PMID: 27367736
7. Advanced Heart Failure: Epidemiology, Diagnostics and Therapeutic Approaches PMID: 32525126
8. Wireless pulmonary artery hemodynamic monitoring in chronic heart failure: a randomized control trial PMID:
21315441
9. Transcatheter mitral valve repair in patients with heart failure PMID: 30280640
10. Right ventricular function in cardiovascular disease, Part II PMID: 18378625
11. A simple evidence based approach to help guide diagnosis of heart failure with preserved ejection fraction PMID:
29792299
12. How to diagnose heart failure with preserved ejection fraction: the HFA-PEFF diagnostic algorithm PMID:
32133741
13. 2014 ESC Guidelines on the diagnosis and management of acute pulmonary embolism. PMID: 25173341
14. CIBIS-II: "The Cardiac Insufficiency Bisoprolol Study II (CIBIS-II): a randomized trial". The Lancet. 1999.
353(9146):9-13. PMID: 10023943
15. COPERNICUS: Packer M, et al. "Effect of Carvedilol on the Morbidity of Patients With Severe Chronic Heart
Failure". Circulation. 2002. 106(17):2194-9. PMID: 12390947
16. COMET: Poole-Wilson PA, et al. "Comparison of carvedilol and metoprolol on clinical outcomes in patients with
chronic heart failure in the Carvedilol Or Metoprolol European Trial". The Lancet. 2003. 362(9377):7-13. PMID:
12853193
17. MERIT-HF: Fagerberg B, et al. "Effect of metoprolol CR/XL in chronic heart failure: Metoprolol CR/XL
Randomized Intervention Trial in Congestive Heart Failure". The Lancet. 1999. 353(9169):2001-7. PMID:
10376614
18. PARADIGM-HF: McMurray JJV, et al. "Angiotensin-neprilysin inhibition versus enalapril in heart failure". The New
England Journal of Medicine. 2014. 371(11):993-1004. PMID: 25176015
19. PIONEER-HF: Velazquez EJ, et al. "Angiotensin-Neprilysin Inhibition in Acute Decompensated Heart Failure".
The New England Journal of Medicine. 2018. ePub 2018-11-11:1-10. PMID: 30415601
20. SAVE: Pfeffer MA, et al. "Effect of Captopril on Mortality and Morbidity in Patients with Left Ventricular
Dysfunction after Myocardial Infarction — Results of the Survival and Ventricular Enlargement Trial". The New
England Journal of Medicine. 1992. 327(10):669-677. PMID: 1386652
21. CONSENSUS Trial Study Group. "Effects of enalapril on mortality in severe congestive heart failure, results of the
cooperative north Scandinavian enalapril survival study". The New England Journal of Medicine. 1987.
316(23):1429-35. PMID: 2883575
22. SOLVD: Yusuf S, et al. "Effect of Enalapril on Survival in Patients with Reduced Left Ventricular Ejection Fractions
and Congestive Heart Failure". The New England Journal of Medicine. 1991. 325(5):293-302. PMID: 2057034
23. V-HeFT II: Cohn JN, et al. "A Comparison of Enalapril with Hydralazine–Isosorbide Dinitrate in the Treatment of
Chronic Congestive Heart Failure". The New England Journal of Medicine. 1991. 325(5):303-310. PMID: 2057035
24. ATLAS: “Comparative effects of low and high doses of the angiotensin-converting enzyme inhibitor, lisinopril, on
morbidity and mortality in chronic heart failure. ATLAS Study Group” Circulation. 1999 Dec 7;100(23):2312-8.
PMID: 10587334
25. AIRE: “Effect of ramipril on mortality and morbidity of survivors of acute myocardial infarction with clinical
evidence of heart failure. The Acute Infarction Ramipril Efficacy (AIRE) Study” The Lancet. 1993 Oct
2;342(8875):821-8. PMID: 8104270
26. CHARM-ALTERNATIVE: Granger CB, et al. "Effects of candesartan in patients with chronic heart failure and
reduced left-ventricular systolic function intolerant to angiotensin-converting-enzyme inhibitors: the CHARM-
Alternative trial". The Lancet. 2003. 362(9386):772-776. PMID: 13678870
27. VAL-HeFT: Cohn JN, et al. "A randomized trial of the angiotensin-receptor blocker valsartan in chronic heart
failure". The New England Journal of Medicine. 2001. 345(23):1667-75. PMID: 11759645
28. HEAL: Konstam MA, et al. "Effects of high-dose versus low-dose losartan on clinical outcomes in patients with
heart failure". The Lancet. 2009. 374(9704):1840-1848. PMID: 19922995
29. RALES: Pitt B, et al. "The effect of spironolactone on morbidity and mortality in patients with severe heart failure".
The New England Journal of Medicine. 1999. 341(10):709-717. PMID: 10471456
30. EPHESUS: Pitt B, et al. "Eplerenone, a selective aldosterone blocker, in patients with left ventricular dysfunction
after myocardial infarction". The New England Journal of Medicine. 2003. 348(14):1309-21. PMID: 12668699
59
Smirthi Sukumar
Heart Failure
TOC
31. EMPHASIS-HF: Zannad F, et al. "Eplerenone in patients with systolic heart failure and mild symptoms". The New
England Journal of Medicine. 2011. 364(1):11-21. PMID: 21073363
32. DECLARE-TIMI 58: Wiviott SP, et al. "Dapagliflozin and cardiovascular outcomes in type 2 diabetes". The New
England Journal of Medicine. 2019. 380(4):347-357. PMID: 30415602
33. DAPA-HF: McMurray JJV, et al. "Dapagliflozin in Patients With Heart Failure and Reduced Ejection Fraction". The
New England Journal of Medicine. 2019. 381(21):1995-2008. PMID: 31535829
34. EMPA-REG: Zinman B, et al. "Empagliflozin, Cardiovascular Outcomes, and Mortality in Type 2 Diabetes". The
New England Journal of Medicine. 2015. 373(22):2117-28. PMID: 26378978
35. EMPEROR-Reduced: Packer M, et al. "Cardiovascular and renal outcomes with empagliflozin in heart
failure". The New England Journal of Medicine. 2020. 383(15):1413-1424. PMID: 32865377
36. V-HeFT: Cohn JN, et al. "Effect of vasodilator therapy on mortality in chronic congestive heart failure". The New
England Journal of Medicine. 1986. 314(24):1547-52. PMID: 3520315
37. A-HeFT: Taylor AL, et al. "Combination of Isosorbide Dinitrate and Hydralazine in Blacks with Heart Failure". The
New England Journal of Medicine. 2004. 351(20):2049-2057. PMID: 15533851
38. SHIFT: Swedberg K et al. "Ivabradine and outcomes in chronic heart failure (SHIFT): a randomised placebo-
controlled study". The Lancet. 2010. 376(10):875-885. PMID: 20801500
39. BEAUTIFUL: “Ivabradine for patients with stable coronary artery disease and left-ventricular systolic dysfunction
(BEAUTIFUL): a randomised, double-blind, placebo-controlled trial.” The Lancet. 2008 Sep 6;372(9641):807-16.
PMID: 18757088
40. SHIFT-HF: “Influence of Cardiovascular and Noncardiovascular Co-morbidities on Outcomes and Treatment
Effect of Heart Rate Reduction With Ivabradine in Stable Heart Failure (from the SHIFT Trial).” Am J Cardiology.
2015 Dec 15;116(12):1890-7. PMID: 26508709
41. DOSE: Felker GM, et al. "Diuretic strategies in patients with acute decompensated heart failure". The New
England Journal of Medicine. 2011. 364(9):797-805. PMID: 21366472
42. SCD-HeFT: Bardy GH, et al. "Amiodarone or an implantable cardioverter-defibrillator for congestive heart
failure". The New England Journal of Medicine. 2005. 252(3):225-37. PMID: 15659722
43. MADIT-II: Moss AJ, et al. "Prophylactic Implantation of a Defibrillator in Patients with Myocardial Infarction and
Reduced Ejection Fraction". The New England Journal of Medicine. 2002. 346(12):877-883. PMID: 11907286
44. DEFINITE: Kadish A, et al. "Prophylactic Defibrillator Implantation in Patients with Nonischemic Dilated
Cardiomyopathy". The New England Journal of Medicine. 2004. 350(21):2151-2158. PMID: 15152060
45. MADIT-CRT: Moss AJ, et al. "Cardiac-resynchronization therapy for the prevention of heart-failure events". The
New England Journal of Medicine. 2009. 361(14):1329-1338. PMID: 19723701
46. CARE-HF: “The effect of cardiac resynchronization on morbidity and mortality in heart failure.” The New England
Journal of Medicine 2005 Apr 14;352(15):1539-49. PMID: 15753115
47. MIRACLE: Abraham W T, et al. "Cardiac Resynchronization in Chronic Heart Failure". The New England Journal
of Medicine. 2002. 346(24):1845-1853. PMID: 12063368
48. RAFT: Tang AS, et al. "Cardiac-resynchronization therapy for mild-to-moderate heart failure". The New England
Journal of Medicine. 2010. 363(25):2385-2395. PMID: 21073365
49. PEP-CHF: “The perindopril in elderly people with chronic heart failure (PEP-CHF) study”. Eur Heart J 2006; 27:
2338-45. PMID: 16963472
50. CHARMED-PRESEVERED: Yusuf S, et al. "Effects of candesartan in patients with chronic heart failure and
preserved left-ventricular ejection fraction: The CHARM-Preserved Trial". The Lancet. 2003. 362(9386):777-781.
PMID: 13678871
51. I-PRESERVE: “Irbesartan in patients with heart failure and preserved ejection fraction.” The New England Journal
of Medicine. 2008 Dec 4;359(23):2456-67. PMID: 19001508
52. PARAMOUNT: “The angiotensin receptor neprilysin inhibitor LCZ696 in heart failure with preserved ejection
fraction: a phase 2 double-blind randomised controlled trial”. The Lancet. 2012 Oct 20;380(9851):1387-95.
PMID: 22932717
53. PARAGON-HF: Solomon SD, et al. "Angiotensin–neprilysin inhibition in heart failure with preserved ejection
fraction". The New England Journal of Medicine. 2019. 381(17):1609-1620. PMID: 32187481
54. PARALLAX: “Angiotensin receptor neprilysin inhibition versus individualized RAAS blockade: design and rationale
of the PARALLAX trial” ESC Heart Failure 2020 Jun;7(3):856-864. PMID: 32297449
55. TOPCAT: Pitt B, et al. "Spironolactone for heart failure with preserved ejection fraction". The New England
Journal of Medicine. 2014. 370(15):1383-1392. PMID: 24716680
56. RELAX: Redfield MM, et al. "Effect of Phosphodiesterase-5 Inhibition on Exercise Capacity and Clinical Status in
Heart Failure with Preserved Ejection Fraction". JAMA. 2013. 309(12):1268-1277. PMID: 23478662
57. NEAT-HFpEF: Redfield MM, et al. "Isosorbide mononitrate in heart failure with preserved ejection fraction". New
England Journal of Medicine. 2015. 373(24):2314-2324. PMID: 26549714
58. DIG: Gorlin R, et al. "The effect of digoxin on mortality and morbidity in patients with heart failure". The New
England Journal of Medicine. 1997. 336(8):525-533. PMID: 9036306
60
Smirthi Sukumar
Heart Failure
TOC
59. MDPIT: “Diltiazem increases late-onset congestive heart failure in postinfarction patients with early reduction in
ejection fraction. The Adverse Experience Committee; and the Multicenter Diltiazem Postinfarction Research
Group” Circulation. 1991 Jan;83(1):52-60. PMID: 1984898
60. A standardized and comprehensive approach to the management of cardiogenic shock. PMID: 33121700
61. Intra-aortic balloon support for myocardial infarction with cardiogenic shock. PMID: 22920912
62. Percutaneous Mechanical Circulatory Support Devices in Cardiogenic Shock. PMID: 28500136
63. A prospective, randomized clinical trial of hemodynamic support with Impella 2.5 versus intra-aortic balloon pump
in patients undergoing high risk percutaneous coronary intervention. PMID: 22935569
64. Percutaneous Mechanical Circulatory Support versus Intra-aortic balloon pump in cardiogenic shock after acute
myocardial infarction. PMID: 27810347
65. Recover I: a multicenter prospective study of Impella 5.0/LD for post-cardiotomy circulatory support. PMID:
22405676
66. Impella ventricular support in clinical practice: Collaborative Viewpoint from a European expert user group PMID:
26363632
67. Left ventricular assist devices 101: shared care for general cardiologist and primary care. PMID: 31635239
68. Temporary circulatory support for cardiogenic shock. PMID: 32682486
69. A standardized and comprehensive approach to the management of cardiogenic shock. PMID: 33121700
70. 2019 EACTS Expert consensus on long-term mechanical circulatory support. PMID: 31100109
71. Refractory cardiac arrest treated with mechanical CPR, hypothermia, ECMO and early reperfusion. PMID:
25281189
72. Rihal CS et al. 2015 SCAI/ACC/HFSA/STS Clinical Expert Consensus Statement on the Use of
Percutaneous Mechanical Circulatory Support Devices in Cardiovascular Care. J Am Coll Cardiol.
2015;65(19), 2140-2141. PMID: 25861962
73. Vieira JL, Ventura HO, Mehra MR. Mechanical circulatory support devices in advanced heart failure: 2020 and
beyond. Prog Cardiovasc Dis. 2020 Sep-Oct;63(5):630-639 PMID: 3297112
74. Ouweneel et al. Impella CP Versus Intra-Aortic Balloon Pump in Acute Myocardial Infarction Complicated by
Cardiogenic Shock: The IMPRESS trial. J Am Coll Cardiol. 2016: 23127. PMID: 27810347
75. Anderson MB et al. Benefits of a Novel Percutaneous Ventricular Assist Device for Right Heart Failure: The
Prospective RECOVER RIGHT Study of the Impella RP Device. J Heart Lung Transplant.
2015;34(12):1549-560. PMID: 26681124
76. Slaughter MS et al. Advanced heart failure treated with continuous-flow left ventricular assist device. N Engl J
Med. 2009; 361:2241. PMID: 19920051
77. Mehra MM et al. A fully magnetically levitated circulatory pump for advanced heart failure. N Engl J Med. 2017;
376:440-450. PMID: 27959709
78. Sheu JJ et al. Early extracorporeal membrane oxygenator-assisted primary percutaneous coronary
intervention improved 30-day clinical outcomes in patients with ST-segment elevation myocardial infarction
complicated with profound cardiogenic shock. Crit Care Med. 2010;38:1810. PMID: 20543669
79. Mancini DM et al. Value of peak exercise oxygen consumption for optimal timing of cardiac. PMID: 1999029
80. Cardiac Transplantation: Update on a road less traveled. PMID: 3190306
61
Smirthi Sukumar
Arrhythmias & Electrophysiology
TOC
A narrow complex tachycardia (NCT) refers to a tachycardia with QRS duration <120ms and typically implies normal
electromechanical conduction from a supraventricular origin. Generally, it is helpful to organize NCTs by mechanism
(automaticity, re-entry, or triggered activity) and anatomic origin (sinus node, atria, AV node).
PATHOPHYSIOLOGY
Tachyarrhythmias are caused by one of three mechanisms: re-entry, automaticity, or triggered activity
1. Re-entry: most common mechanism of NCT and is thought to be the result of cyclic conduction around an electrical
circuit. Reentry can occur when the following three criteria are met:
1. There are two distinct but parallel conducting pathways connected proximally and distally
2. One pathway must have a substantially longer refractory period than the other pathway
3. The pathway with a shorter refractory period must conduct electrical impulses slower than the other pathway
Initiation: re-entry can be initiated when an appropriately timed premature impulse is introduced to the circuit. If a pre-
mature impulse is blocked by the longer refractory period of one limb of the circuit and preferentially conducts down the
slowly conducting limb of the circuit, enough time can elapse such that the current can travel in retrograde fashion up the
newly repolarized limb and reenter the circuit from the beginning, forming a continuous circuit. Re-entry can also be “set
up” by scar and/or ischemia that produce areas of tissue heterogeneity (though this is more common in ventricular
arrythmias).
2. Automaticity: “automatic” depolarization of electrical foci in different areas of the heart (atria, AV junction, ventricles)
which may be physiologic (e.g. automatic pacemaker function of the SA resulting in normal sinus rhythm or sinus
tachycardia). Abnormal automaticity at specific location(s) of the heart can trigger tachyarrhythmias (both atrial and
ventricular), typically in response to metabolic insults (ischemia, hypoxemia, sympathetic tone, and electrolyte and acid-
base derangements).
3. Triggered activity: shares features of both automaticity and re-entry and occurs when there is interruption of normal
cardiac repolarization by “afterdepolarizations,” early or delayed depolarizations that either proceed (early
afterdepolarizations) or follow (delayed afterdepolarizations) the cardiac action potential. These spontaneous or triggered
beats cause extrasystoles and lead to tachyarrhythmias. In early afterdepolarization, abnormal oscillations in the
membrane potential are seen during phase 2 (plateau) and early repolarization in phase 3 (e.g. long QT syndrome,
Torsades). In delayed afterdepolarization, the oscillations occur after full repolarization (e.g. digitalis toxicity due to
enhanced calcium influx). Either early or delayed after depolarizations can lead to self-sustaining arrhythmic events.
62
Lakotah Hardie, Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Sinus tachycardia
● Presentation: HR 100–150 in older pts. Can be as high as 200 in young patients (max HR ~ 220-age)
o Initiation is gradual (heart rate trend on telemetry increases over seconds)
o P wave axis and morphology unchanged compared to baseline ECG
● Pathophysiology: most frequently secondary to other cause (physiologic) Due to an underlying process (pain, fever,
hypovolemia/shock, CHF, PE, anemia, hyperthyroidism, AV fistula) or drugs (intoxication of caffeine, anticholinergics,
catecholamines, nicotine, or withdrawal from EtOH, benzos, opiates). May be seen in roughly one-third of patients
with acute MI (Circulation 1972;45:681)
o Idiopathic (primary):
▪ Inappropriate Sinus Tachycardia (IST) is idiopathic resting sinus tachycardia, typically with very high sinus
rates on exertion
▪ Paroxysmal Orthostatic Tachycardia Syndrome (POTS) is a separate diagnosis characterized by marked
sinus tachycardia upon standing
▪ Both IST and POTS are diagnoses of exclusion after physiologic ST is ruled out
● Treatment: physiologic treat the underlying cause
o IST or POTS: treatment is primarily for symptoms (tachyarrhythmia-mediated cardiomyopathy is exceedingly
rare). BB is the most common treatment vs less often non-dihydropyridine CCB, catheter ablation of SA node,
ivabradine (off label but can be effective)
63
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Junctional Tachycardia
● Presentation
o These generally include junctional ectopic tachycardia (JET) and accelerated junctional rhythm (which is known
as non-paroxysmal junctional tachycardia of NPJT). Junctional tachycardias can be difficult to distinguish from
other arrhythmias, particularly AVNRT. Retrograde P waves can be observed, and incomplete A-V dissociation is
frequent with intermittent capture of sinus beats by the AV node leading to irregular ventricular rates that can also
mimic atrial fibrillation
o Junctional ectopic tachycardia (JET):
● Rare and relatively benign in adults; more commonly associated with, and often incessant in infants after
cardiac surgery for CHD
● Rapid, occasionally irregular NCT, with rates typically 120 to 220
o Non-paroxysmal junctional tachycardia(NPJT):
● More common in adults than junctional ectopic tachycardia
● Also referred to as accelerated junctional rhythm, rates typically 70 to 130
● Pathophysiology
o Junctional ectopic tachycardia: Arises from an accelerated, ectopic focus in the AV junction (including the His
bundle)
o Non-paroxysmal junctional tachycardia: Most often due to digitalis toxicity (digitalis can enhance phase 4
depolarization of the His-Purkinje fibers) but also seen in acute MI
● Treatment
o Junctional ectopic tachycardia: Acute :IV nodal blockade in the acute setting, Chronic: PO for ongoing
management; ablation can be considered if symptomatic and resistant to medical therapy, but is very risky (can
result in AV block)
o Non-paroxysmal junctional tachycardia: Treatment of underlying condition (digitalis toxicity, MI)
64
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Ladder diagrams of typical (slow-fast) AVNRT (left) and atypical (fast-slow) AVNRT (right). In typical AVNRT, a PAC
triggers re-entry that beings anterograde along the slow pathway (while the fast pathway is refractory). Retrograde
conduction occurs along the fast pathway, generating a “short RP.” In atypical AVNRT, a PVC triggers re-entry with
anterograde conduction via the fast pathway and retrograde atrial depolarization via the slow pathway, generating a “long
RP.”. A = atrial depolarization, V = ventricular depolarization, F = fast pathway, S = slow pathway, ATR = atrium, AVN =
AV node, VENT = ventricle. For more information regarding laddergrams, consider the following link and source
https://ecg-interpretation.blogspot.com/2013/06/ecg-interpretation-review-69-pvc-pjc.html
65
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
AVNRT with retrograde P forming a pseudo r’ in V1 and pseudo-S in II, III, aVF (A). These are absent in NSR (B).
66
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
67
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
68
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Atrial flutter
● Presentation
o Atrial rate 250–350, often 300. Ventricular rate can be around 150 bpm if there is 2:1 A-V conduction.
o Typical (“CTI’”): 90% of patients have typical flutter with counterclockwise reentry around the tricuspid valve.
Sawtooth “F” waves are negative in the inferior leads based on counterclockwise conduction down the right
atrial anterolateral free wall, across the cavotricuspid isthmus, and more slowly up the interatrial septum
o Atypical forms of atrial flutter also exist, with a wide variety of atrial rates and p wave morphologies. These are
more common after catheter/surgical ablation of atrial fibrillation
● Pathophysiology: Macro-reentrant circuit
● Treatment:
o If 2:1 block, can first trial vagal maneuvers. Adenosine may also be trialed and generally will transiently slow
A:V conduction and the ventricular rate but will not terminate atrial flutter (6mg rapid IV push, followed by
12mg push if no response; if administered through a central line, the doses are 3mg-->6 mg).
o Medical management: Anticoagulation and rate/rhythm control, similar to AFib
o Always rate control first with BB, CCB, and/or digoxin before attempting rhythm control with class IA or IC agents
(because these anti-arrhythmics can facilitate embolization of an atrial thrombus as well as faciliatate 1:1 ventricular
conduction with rates in the 300’s. Amiodarone 200mg QD can be used to prevent recurrence. DCCV can be
considered if atrial flutter is associated with hypotension or decompensated heart failure. Typical flutter is most
amenable to catheter ablation
Atrial fibrillation
● Nomenclature:
o Paroxysmal: Recurrent AF (≥ 2 episodes) that terminates spontaneously within 7 days. Episodes of AF of ≤ 48
hours duration that are terminated with electrical or pharmacologic cardioversion should also be classified as
paroxysmal AF episodes.
o Persistent: Continuous AF that is sustained beyond 7 days. Episodes in which a decision to cardiovert the patient
after ≥ 48 hours of AF, but prior to 7 days, should also be classified as persistent AF episodes.
o Long-standing persistent: Duration > 12 months; efforts to terminate, either chemically or electrically, are typically
unsuccessful although catheter ablation may restore sinus rhythm in some patients.
o Permanent: Persistent AF where further attempts at rhythm control are indefinitely deferred
● Pathophysiology: AF results from an abnormal atrial response to reentry and/or rapid focal ectopic firing. In
the case of reentry, fibrillatory activity may be generated from a single localized reentry circuit or result
from multiple functional reentry circuits that vary in time and space. Alternatively, there may be an ectopic
focus that generates regularly firing, rapid discharges.
o Natural history of AF may involve progression from paroxysmal to persistent to long-standing persistent
forms. At the tissue level, this can result from atrial remodeling caused by the arrhythmia itself,
progressive fibrosis, and worsening of underlying heart disease. AF-related electric remodeling results
from altered expression and function of cardiac ion channels, which favor the development of
functional reentry substrates. With AF termination, some of these changes can be reversible, a process
known as reverse remodeling. If left untreated, however, the remodeling and fibrosis can progress
which contribute to persistent AF.
● Management
o Stable AF: rate versus rhythm control. If AF is self-limited, or has a potentially reversible cause,
restoration of normal sinus rhythm should be pursued. Pharmacological or direct current cardioversion
69
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
(DCCV) has a higher success rate when AF has been present for less than 24 hours. Remember the
adage, “AF begets AF” (through irreversible electrical remodeling).
▪ Studies that have examined rate control vs medical rhythm control have not detected differences in
mortality or stroke rates.
▪ Caveats: these studies enrolled predominantly older patients (average age 70), most of whom had
persistent AF in the setting of structural heart disease and were able to tolerate AF from a
symptomatic standpoint; younger patients and those with severe symptoms from AF were
underrepresented. In addition, follow-up was limited to just a few years. Thus, the results of these
trials do not necessarily apply to younger patients without heart disease or those whose
dependency on SR is likely to change appreciably over time.
o Unstable AF 🡪 DCCV: patients with hypotension, hypoxemia, decompensated heart failure, pre-
excitation, worsening ischemia, angina or decreased responsiveness often necessitate emergent
DCCV, ideally preceded by sedation (usually fentanyl/midazolam or propofol depending on patient
stability and coordinated by cardiac anesthesia, CCU or ER team). Alternatively, amiodarone may be
used in conjunction with neo (given reflex bradycardia) in patients who are hypotensive. In addition,
amiodarone may help to maintain SR after DCCV.
o Acute rate control usually achieved through AV nodal blockers (see Table 2).
▪ While in the CCU/SDU, be aware of the negative inotropic effects of BB & CCB
▪ In the case of atrial fibrillation in sepsis, a recent large retrospective analysis showed that IV BBs
were associated with reduced hospital mortality when compared with CCBs, digoxin, and
amiodarone.8
70
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
71
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Rhythm Control Strategies: Before initiating antiarrhythmic drug (AAD) therapy, treatment of precipitating or reversible
causes of AF should be pursued. The goal of AAD therapy include a reduction in the frequency and duration of episodes
of AF as well as an emerging goal of reducing mortality and hospitalizations associated with this condition. See Figure 1
and Table 3.
AATAC (2016): In patients with symptomatic (NYHA II-III) heart failure with reduced ejection fraction (HFrEF, LVEF
●
≤40%) and persistent atrial fibrillation (AF) chosen for a rhythm control strategy, AF ablation was associated with a
36% absolute increase in AF-free survival at 24 months. There was also a 26% absolute reduction in unplanned
hospitalizations and a 10% absolute reduction in overall mortality with AF ablation.10
●
FIRE AND ICE (2016): In patients with symptomatic paroxysmal atrial fibrillation (pAF) resistant to antiarrhythmic
drugs referred for pulmonary vein isolation (PVI), balloon cryoablation is noninferior to radiofrequency ablation in
regards to a primary outcome of composite clinical failure (recurrent AF, prescription of antiarrhythmic drugs, or repeat
ablation) more than 90 days after the index procedure.11
CASTLE-AF (2018): In patients with AF and symptomatic (NYHA II-IV) systolic heart failure (LVEF ≤ 35%), catheter
●
ablation is associated with a 16.1% absolute reduction in death or hospitalization for heart failure when compared to
medical therapy (rate or rhythm control). This difference was driven both by a 11.6% absolute reduction in death and
a 15.2% absolute reduction in hospitalization for heart failure. Catheter ablation was also associated with greater
improvement in LVEF and long-term maintenance of sinus rhythm. 12
●
CABANA (2019): In patients with atrial fibrillation (AF) requiring treatment, catheter ablation was not associated with a
significant reduction in death, disabling stroke, serious bleeding, or cardiac arrest when compared to medical therapy
at 12 months. The results of this trial were incorporated in the AHA/ACC 2019 AF guidelines and as a result AF
ablation in patients without systolic heart failure is likely to remain an elective procedure performed with the primary
purpose of symptom relief in patients who have failed, are intolerant of, or decline anti-arrhythmic therapy. 13
●
EAST-AFNET4 (2020): In patients recently diagnosed with AF (~1month) rhythm control was superior to usual care
and the trial was stopped early, because the primary-outcome composite (CV death, CVA, or hospitalization) was
significantly lower in the rhythm control group. While rhythm control was mostly with AAD, about 8% of participants
underwent catheter ablation by 1 year and 20% by 2 years. This trial has not dramatically altered guidelines as of yet,
but its findings alongside those from CABANA and CASTLE-AF suggest that normal sinus rhythm matters. 36
72
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Maintenance
Drug LOE Loading Dose Onset Major Side Effects
Dose
500 mcg/kg IV 60–200
Esmolol* I-C 5 min ↓ BP/HR; HB; asthma, HF
over 1 min mcg/kg/min IV
2.5–5 mg IV
Metoprolol* I-C over 2 min; up 5 min NA ↓ BP/HR; HB; asthma, HF
to 3 doses
Propranolol* I-C 0.15 mg/kg IV 5 min NA ↓ BP/HR; HB; asthma, HF
0.25 mg/kg IV 2-
Diltiazem* I-B 5–15 mg/h IV ↓ BP; HB, HF
over 2 min 7min
0.075–0.15
3-
Verapamil* I-B mg/kg IV over NA ↓ BP; HB, HF
5min
2 min
↓BP; HB, pulmonary toxicity, skin discoloration,
73
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
in appropriate AFib patients in men with a CHA2DS2VASc of 2 or more and 3 or more in women. In men with a
CHA2DS2VASc of 1 and women with a score of 2, the decision to initiate oral anticoagulation depends on if the extra
point is conferred by age 65-74 as this is the strongest risk factor. Female gender alone does not confer significantly extra
risk for embolic stroke in the absence of other risk factors and does not necessitate anticoagulation by itself. Patients who
have moderate-to-severe mitral stenosis or a mechanical heart valve should continue to be anticoagulated with warfarin. 1
Anticoagulation: Specific Agents
Agent Commentary
Warfarin therapy should be targeted to an INR 2–3, as measured weekly during initiation of therapy and
Warfarin monthly thereafter. On average, warfarin provides an annual reduction of 68% in relative risk for stroke.
Approved as an alternative to warfarin for non-valvular AF, with superiority in the prevention of stroke
Dabigatran compared to warfarin in the RE-LY trial.15 Pro-drug, rapidly converted to active direct thrombin inhibitor.
It is administered in fixed doses without laboratory monitoring of anticoagulation intensity. At a dose of
150 mg bid, it reduced the rate of stroke by 34% with no increase in major bleeding when compared to
warfarin. For those with CrCl 15–30, a dose of 75 mg bid was also approved (dabigatran is not indicated
for patients with CrCl < 15).
A direct-thrombin inhibitor. In ROCKET-AF trial, it proved to be non-inferior to warfarin for the prevention
Rivaroxaban of stroke and systemic embolism, with no difference in risk of major bleeding although ICH and fatal
bleeding occurred less frequently with rivaroxaban. 16 It is administered at a dose of 20 mg daily, with
dose reduction to 15 mg if renal impairment.
Apixaban A direct-thrombin inhibitor, found to be superior to warfarin in preventing stroke or systemic embolism in
the ARISTOTLE trial. It also caused less bleeding and lower mortality.17 It is administered at a dose of 5
mg BID, or 2.5 mg BID if any two of the following: Age ≥ 80 years old, body weight ≤ 60 kg, serum Cr ≥
1.5. In the AVERROES trial in patients with AF thought to be unsuitable candidates for anticoagulation
with VKA, Apixaban reduced the risk of stroke without increasing the risk of major bleeding compared to
aspirin.18
Edoxaban A direct-thrombin inhibitor, found to be equal in efficacy to warfarin in preventing stroke or systemic
embolism in ENGAGE AF-TIMI 48. It caused less bleeding.19 It was also noted to be non-inferior to
warfarin in atrial fibrillation following TAVR, but with more bleeding in the ENVISAGE-TAVI AF trial in
2021.42 It is administered at a dose of 60 mg daily (or 30 mg daily for CrCl 15-50mL/min). Edoxaban is
currently less commonly used given more frequent clinical experience with rivaroxaban and apixaban as
well as equivalence rather than superiority to warfarin.
Aspirin A poor substitute for anticoagulation, though perhaps a plausible alternative in a very low-risk patient
(i.e., CHA2DS2-Vasc = 1) or one at very high risk of bleeding. The relative risk reduction for stroke is
variable, but perhaps around 20%.
Patient with Contraindications to AC: Percutaneous and Surgical Left Atrial Appendage (LAA) Closure
● LAA closure has emerged as a mechanical alternative to pharmacologic stroke prevention in patients with a
contraindication to anticoagulation. There are multiple LAA occlusion devices, including the WATCHMAN, Amulet,
WaveCrest, and LARIAT system devices (Amulet and WaveCrest are currently investigational in the U.S.).
75
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
● PROTECT-AF trial: showed that percutaneous LAA closure with the WATCHMAN device was non-inferior to
warfarin for the prevention of the composite endpoint of ischemic stroke, hemorrhagic stroke, cardiovascular death,
and systemic embolism.22 The PREVAIL Trial subsequently found that LAA occlusion was non-inferior to warfarin
for ischemic stroke prevention, although non-inferiority was not achieved for the overall efficacy endpoint (stroke,
systemic embolism, and cardiovascular/unexplained death).23
● Based on PROTECT & PREVAIL, the WATCHMAN device was approved by the in March 2015 for patients with
nonvalvular AF for whom long-term anticoagulation is indicated but who have a contraindication.
o After placement of an LAA occlusion device, patients are treated with oral anticoagulation + ASA for 6
weeks, followed (if successful LAA closure is documented by TEE at 6 weeks post-implant) by clopidogrel
+ ASA for 6 months, followed by ASA for life. Patients with absolute contraindication to oral anticoagulants
are given DAPT for 6 months.
● In the LAAOS III trial, investigators were able to conclude that surgical occlusion of the LAA in patients with atrial
fibrillation undergoing cardiac surgery and who went on to be anticoagulated had a lower risk of stroke than those
who did not.43
76
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
References:
1. Aliot, Etienne M., et al. "ACC/AHA/ESC Practice Guidelines." Circulation 108 (2003): 1871-1909
2. Robert O., Bonow, Douglas Mann and Peter Libby. Braunwald's Heart Disease: A Textbook of Cardiovascular
Medicine. 9th. Singl Volume. Philadelphia: Saunders, 2011. Print
3. Delacrétaz, Etienne. "Supraventricular tachycardia." New England Journal of Medicine 354.10 (2006): 1039-1051
4. Griffin, Brian P et al. Manual Of Cardiovascular Medicine. Philadelphia: Wolters Kluwer Health/Lippincott Williams
& Wilkins, 2013. Print
5. Spector, Peter, et al. "Meta-analysis of ablation of atrial flutter and supraventricular tachycardia." The American
journal of cardiology 104.5 (2009): 671-677.
6. Fox, David J., et al. "Supraventricular tachycardia: diagnosis and management." Mayo Clinic Proceedings. Vol.
83. No. 12. Elsevier, 2008
7. Appelboam, Andrew, et al. "Postural modification to the standard Valsalva manoeuvre for emergency treatment of
supraventricular tachycardias (REVERT): a randomised controlled trial." The Lancet 386.10005 (2015): 1747-
1753.
8. Page, Richard L., et al. "2015 ACC/AHA/HRS guideline for the management of adult patients with
supraventricular tachycardia: a report of the American College of Cardiology/American Heart Association Task
Force on Clinical Practice Guidelines and the Heart Rhythm Society." Journal of the American College of
Cardiology 67.13 (2016): e27-e115
9. Fogoros, Richard N. Electrophysiologic Testing. Chichester: Wiley-Blackwell, 2012. Print.
10. January, Craig T., et al. "2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: a
report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and
the Heart Rhythm Society." Journal of the American College of Cardiology 64.21 (2014): e1-e76
11. Hohnloser, Stefan H., et al. "Rhythm or rate control in atrial fibrillation—Pharmacological Intervention in Atrial
Fibrillation (PIAF): a randomised trial." The Lancet 356.9244 (2000): 1789-1794
12. Atrial Fibrillation Follow-up Investigation of Rhythm Management (AFFIRM) Investigators. "A comparison of rate
control and rhythm control in patients with atrial fibrillation." New England Journal of Medicine 347.23 (2002):
1825-1833
13. Van Gelder, I. C. "Rate Control versus Electrical Cardioversion for Persistent Atrial Fibrillation Study Group. A
comparison of rate control and rhythm control in patients with recurrent persistent atrial fibrillation." N Engl J
Med 347 (2002): 1834-1840
14. Carlsson, Jörg, et al. "Randomized trial of rate-control versus rhythm-control in persistent atrial
fibrillation." Journal of the American College of Cardiology 41.10 (2003): 1690
15. Opolski, Grzegorz, et al. "Rate control vs rhythm control in patients with nonvalvular persistent atrial fibrillation:
the results of the Polish How to Treat Chronic Atrial Fibrillation (HOT CAFE) Study." Chest 126.2 (2004): 476-486
16. Roy, Denis, et al. "Rhythm control versus rate control for atrial fibrillation and heart failure." New England Journal
of Medicine358.25 (2008): 2667-2677
17. Walkey, Allan J., et al. "Practice patterns and outcomes of treatments for atrial fibrillation during sepsis: a
propensity-matched cohort study." Chest 149.1 (2016): 74-83
18. Klein, Allan L., et al. "Use of transesophageal echocardiography to guide cardioversion in patients with atrial
fibrillation." New England Journal of Medicine 344.19 (2001): 1411-1420
19. Di Biase, Luigi, et al. "Ablation versus amiodarone for treatment of persistent atrial fibrillation in patients with
congestive heart failure and an implanted device: results from the AATAC multicenter randomized
trial." Circulation 133.17 (2016): 1637-1644
20. Kuck, Karl-Heinz, et al. "Cryoballoon or radiofrequency ablation for paroxysmal atrial fibrillation." New England
Journal of Medicine 374.23 (2016): 2235-2245
21. Marrouche, Nassir F., et al. "Catheter ablation for atrial fibrillation with heart failure." New England Journal of
Medicine378.5 (2018): 417-427
22. Mark, Daniel B., et al. "Effect of catheter ablation vs medical therapy on quality of life among patients with atrial
fibrillation: the CABANA randomized clinical trial." JAMA (2019)
23. January, Craig T., et al. "2019 AHA/ACC/HRS focused update of the 2014 AHA/ACC/HRS guideline for the
management of patients with atrial fibrillation: a report of the american college of Cardiology/American heart
association task force on clinical practice guidelines and the heart rhythm society." Journal of the American
College of Cardiology (2019): 25873
24. Connolly, Stuart J., et al. "Dabigatran versus warfarin in patients with atrial fibrillation." New England Journal of
Medicine 361.12 (2009): 1139-1151
25. Patel, Manesh R., et al. "Rivaroxaban versus warfarin in nonvalvular atrial fibrillation." New England Journal of
Medicine 365.10 (2011): 883-891
26. Granger, Christopher B., et al. "Apixaban versus warfarin in patients with atrial fibrillation." New England Journal
of Medicine 365.11 (2011): 981-992
27. Connolly, Stuart J., et al. "Apixaban in patients with atrial fibrillation." New England Journal of Medicine 364.9
(2011): 806-817
77
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
28. Giugliano, Robert P., et al. "Edoxaban versus warfarin in patients with atrial fibrillation." New England Journal of
Medicine 369.22 (2013): 2093-2104
29. Dewilde, Willem JM, et al. "Use of clopidogrel with or without aspirin in patients taking oral anticoagulant therapy
and undergoing percutaneous coronary intervention: an open-label, randomised, controlled trial." The
Lancet 381.9872 (2013): 1107-1115
30. Gibson, C. Michael, et al. "Prevention of bleeding in patients with atrial fibrillation undergoing PCI." New England
Journal of Medicine 375.25 (2016): 2423-2434
31. Reddy, Vivek Y., et al. "Percutaneous left atrial appendage closure vs warfarin for atrial fibrillation: a randomized
clinical trial." Jama 312.19 (2014): 1988-1998
32. Holmes, David R., et al. "Prospective randomized evaluation of the Watchman Left Atrial Appendage Closure
device in patients with atrial fibrillation versus long-term warfarin therapy: the PREVAIL trial." Journal of the
American College of Cardiology 64.1 (2014): 1-12
33. Lubitz, Steven A., et al. "Long-term outcomes of secondary atrial fibrillation in the community: the Framingham
Heart Study." Circulation 131.19 (2015): 1648-1655
34. Gillinov, A. Marc, et al. "Rate control versus rhythm control for atrial fibrillation after cardiac surgery." New
England Journal of Medicine 374.20 (2016): 1911-1921
35. Di Biase, Luigi, et al. "Ablation versus amiodarone for treatment of persistent atrial fibrillation in patients with
congestive heart failure and an implanted device: results from the AATAC multicenter randomized
trial." Circulation 133.17 (2016): 1637-1644
36. Kirchhof, Pauls, Camm A. John, et al. “Early Rhythm-Control Therapy in Patients with Atrial Fibrillation.” New
England Journal of Medicine 383 (2020):1305-1316
37. Sedej, Simon & Pieske, Burkert. (2012). Mechanisms of Ca2+-Triggered Arrhythmias. 10.5772/25906.0
38. Link MS. Clinical practice. Evaluation and initial treatment of supraventricular tachycardia. N Engl J Med.
2012;376:1438–48.’
39. Patel S, Patel A, Olshansky B, et al. TACH-ING AWAY: PERMANENT JUNCTIONAL RECIPROCATING
TACHYCARDIA. J Am Coll Cardiol. 2020 Mar, 75 (11_Supplement_1) 3221.
40. Mahaim-mediated tachycardia using at times the atrioventricular node and other times a left lateral accessory
pathway. Vali, Zakariyya et al. HeartRhythm Case Reports, Volume 7, Issue 10, 641 – 649.
41. Hunter, J., Tsounias, E., Cogan, J., & Young, M. L. (2018). A Case of Lown-Ganong-Levine Syndrome: Due to an
Accessory Pathway of James Fibers or Enhanced Atrioventricular Nodal Conduction (EAVNC)?. The American
journal of case reports, 19, 309–313.
42. Van Mieghem NM, Unverdorben M, Hengstenberg C, et al. Edoxaban versus vitamin K antagonist for atrial
fibrillation after TAVR. N Engl J Med. 2021.
43. Whitlock RP, Belley-Cote EP, Paparella D, et al. Left atrial appendage occlusion during cardiac surgery to prevent
stroke. N Engl J Med 2021;384:2081-2091.
78
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
A wide complex tachycardia (WCT) refers to a tachycardia with QRS duration QRS ≥ 120ms. Generally, the approach to
WCT is to assume it is a ventricular tachycardia (VT) until proven otherwise. VT is the most common and most malignant
of the WCTs, however a broader differential should be considered.
● Ventricular Tachycardia(70% of the time): ectopic or reentrant ventricular impulse spreads electrical activity slowly
through the ventricular myocytes and produces a wide QRS. It originates below or very rarely at the bundle of His.
o Monomorphic VT: has a variety of etiologies (infarct/scar being a common one)
o Polymorphic in the setting of a normal QTc (<500 ms) is often due to ischemia
● Supraventricular Tachycardia (SVT) with Aberrancy( 30% of the time): SVT (e.g. AT, AF) conducts an electrical
impulse into the ventricles along the standard His-Purkinje system, but is slowed along that path as a result of:
o Pre-existing or rate-related aberrant intraventricular conduction (e.g. bundle-branch block)
o Drugs (e.g. class 1C anti-arrhythmics, digoxin), electrolyte abnormalities (e.g. hyperkalemia), or ischemia
o Slowing of electrical conduction through the ventricles results in wide complex QRS
● Supraventricular Tachycardia with Pre-excitation: SVT conducts an electrical impulse into the ventricles through an
aberrant conduction pathway in which depolarization advances slowly through the ventricular myocytes rather than
quickly along the His-Purkinje system (for example, an underlying RBBB)
● Pacemaker-Related Tachycardia
o Pacemaker-mediated tachycardia: “Endless loop” tachycardia in which the aberrant circuit is generated by the
pacemaker
▪ Electricity arising from the ventricular pacing lead conducts retrograde (typically, through the AV node) to the
atria, producing atrial depolarization (seen as a retrograde P wave)
▪ The atrial depolarization is sequentially recognized by the pacemaker, leading to atrial-sensed ventricular
pacing
▪ Another ventricular depolarization can conduct in retrograde fashion again through the AV node and produce
a circuit
o Pacemaker-tracked tachycardia: SVT is tracked by the pacemaker
▪ SVT is sensed by the pacemaker, producing a wide complex beat.
(This rhythm is less common now that devices have become sophisticated at mode switching to non-tracking
modes such as VVI or DDI when the algorithm recognizes SVT or AF
o *Note: Both pacemaker-tracked and pacemaker-mediated tachycardia can be terminated by the application of a
magnet to the pacemaker, which changes the pacing mode to an asynchronous mode (VOO or DOO). This is not
true for most ICDs, in which the magnet will temporarily disable ICD therapy, but will not alter the pacing mode.
Quick reference:
79
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
80
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
a. A typical RBBB looks like an RSR' complex in V1, with R smaller than R', and an RS complex in V6, with
R/S >1. So any complex that looks significantly different than this (for example, a monophasic R, qR
complex, or RSR' complex with R > R') favors a diagnosis of VT.
b. A typical LBBB looks like an rS complex in V1, and an R in V6. So any complex that looks significantly
different than this (for example, a wide R wave or wide notched S wave in V1, or a Q wave in V6) favors a
diagnosis of VT.
QRS morphology:
● A QRS > 160 msec has a 20:1 likelihood ratio of being VT. VT can be narrow (<140 msec) if it arises near the His-
Purkinje system
● If LBBB or RBBB pattern, look for morphologies suggestive of VT:
81
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
● Note: If there is VT with a RBBB pattern + inferiorly directed axis in a patient with a structurally normal heart, consider
LVOT VT. Interestingly, this rhythm can respond to drugs that reduce cAMP, such as adenosine, verapamil or beta-
blockers
● Note: If there is VT with a LBBB pattern + inferiorly directed axis in a patient with a structurally normal heart, consider
RVOT VT. This rhythm also responds to drugs that reduce cAMP
There are many formal criteria for distinguishing VT from other causes of WCT with two others listed below in addition to
the Brugada criteria discussed in depth above. They are more time consuming and unwieldy in comparison to the quick
approach above. If in doubt, assume VT.
1. Vereckei criteria
2. R wave peak time (RWPT)
Clinical Management of WCT
Consider all WCT as VT until proven otherwise. At least 80% of WCT in patients with ischemic or structural heart disease
is VT.
● Pulseless: Immediately start the pulseless VT ACLS algorithm and prepare for unsynchronized DCCV
● Hemodynamically unstable or highly symptomatic: Prepare for synchronized DCCV (+ fentanyl/versed for sedation)
and start amiodarone (150 mg x 1 Q 3–5 min, then drip @ 1 mg/min), and/or lidocaine (100 mg x 1, then drip @ 1–4
mg/min) unless concern for TdP (prolongs QTc)
o Put pads on the patient
o CALL FOR HELP:
▪ Fellow resident(s) and your attending
▪ Ward Cardiology fellow and/or EP Fellow on Call
▪ RICU if anticipate potential need for intubation
▪ Cardiac anesthesia for conscious sedation (though often no time)
o Worry about etiology after stabilization
▪ If regular, monomorphic, and there is some suspicion for SVT, consider adenosine as both a diagnostic (can
slow down rhythm to make easier to dx) and therapeutic intervention
▪ If pacemaker-mediated or tracked WCT→ apply magnet
● Hemodynamically stable:
o Put pads on patient, call for help; you have some time to determine etiology of WCT, but most patients do not
tolerate persistent VT (particularly if critically ill) and will likely develop hemodynamic instability
82
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Monomorphic VT:
● Anti-arrhythmic drugs (AAD): Temporizing measure while reversing underlying cause. Do not reduce mortality or
prevent SCD, may increase mortality when used as monotherapy for VT/VF prevention.
o Recurrent, stable VT: Amiodarone and procainamide
o WPW possible: Lidocaine or procainamide preferred (both reduce accessory pathway conduction)
o Amiodarone: 150 mg IV bolus over 3–5 min (re-bolus as needed), followed by gtt load at 1mg/min for 6hrs
and then 0.5 mg/min for 18 hours.
▪ Effective for both ventricular and supraventricular arrhythmias (Na+ and K+ channel blocker; slows AV nodal
conduction as well) and has superior efficacy over lidocaine
▪ AV nodal blockade effects predominate until 6–7 grams administered. A full “amio load” for VT is 10 grams
(given over a period of days)
▪ AVOID IN TdP: nodal-blocking activity slows HR and K+ channel blocking effect prolongs the QTc, which can
promote TdP
o Lidocaine: 1–1.5 mg/kg IV bolus over 2–3 minutes (often 100mg), followed by 1–4 mg/min infusion (often
start at 1)
o Procainamide: Infuse at 20–50 mg/min until arrhythmia is controlled (stop if hypotension or if QRS complex
widens by 50% of original width, or total of 17 mg/kg is given). Maintenance infusion usually 1–6 mg/min
● Electrolytes: replete Magnesium and potassium
● Electricity: Asynchronous DCCV (for pulseless VT) vs synchronized DCCV (for VT w/ pulse). Other options if
patient has a Pacemaker include:
o Over-drive pacing (anti-tachycardia pacing) at a faster rate than the VT may be useful in some cases (call Ward
fellow and/or EP for help w/ ATP).
Polymorphic VT:
● Torsades/PMVT w/ prolonged QT (on ECG from earlier in day)
o Electrolytes: Repletion of Mg2+ with 1–2 g IV bolus, with a total dose of 2–4 g over 10–15 min, can successfully
terminate TdP within 5 minutes in up to 75% of patients, and 15 minutes in most patients. Replete K+ as well.
o AAD: Lidocaine avoid nodal agents and amiodarone
o Chronotropes: Goal HR 80-100bpm will shorten the QTc and can abort the PMVT. Start IV infusion of
isoproterenol (2–6 mcg bolus followed by 2–20 mg/min OR dopamine at a starting rate of at least 300 mcg/min
o Electricity: Asynchronous DCCV (for sustained PMVT) or over-drive pacing at rates in the range of 80–100
bpm (to suppress pause-dependent initiation of PMVT).
● PMVT with normal QT (on ECG from earlier in day) is ischemia until proven otherwise 🡪 ACS pathway with
emergent cardiology evaluation for cath/revasc, can use AAD as above
83
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
o Caution: Adenosine should not be used in pre-excited AF (presents as irregular, WCT); refer to AVRT section for
management of SVT associated with WPW
● Electricity: DCCV for extremely symptomatic or unstable patients
Pacemaker-Mediated WCT:
● Magnet is 1st line therapy. Magnet will also stop the arrhythmia by mode switching to VOO or DOO to stop
sensing the atria. The magnet does not change the pacing mode for an ICD (it will disable the defibrillator
therapies) and thus will not terminate a PPM-mediated tachycardia in a patient with an ICD.
o EP will need to reprogram pacemaker reprogramming to prevent further episodes of PMT
● AAD: 2nd line: Adenosine, verapamil or beta-blockers can abort retrograde conduction and stop the circuit
Pacemaker-Tracked WCT: Treat SVT as you would any other SVT. Mode switch PPM to a non-tracking mode either by
EP interrogation or with magnet.
Maintenance Therapy
● Devices: ICDs are recommended for secondary prevention of VT/VF unless a reversible trigger is identified/treated
o The AVID trial demonstrated a 10% absolute mortality benefit with this strategy when compared to AAD alone
(see ICD Section for more information)
o Specific guidelines are available for IHD, NICM, and other specific cardiomyopathies (e.g. HCM)
● Medical management:
o Beta-blockers: reduce sympathetic tone, have some direct antiarrhythmic effect, and may reduce mortality in
patients with previous VT/VF
o AADs are often continued unless a reversible VT/VF trigger (e.g. ischemia) is identified and treated, although
there is no established mortality benefit with this strategy
o Patients with HFrEF should be treated with a BB, mineralocorticoid antagonist, ACEi/ARB/neprolysin inhibitor to
reduce SCD and all-cause mortality
● Procedures:
o Patients with VT/VF and ischemic heart disease should be revascularized as appropriate
o VT ablation:
▪ VT ablation is recommended in: patients with sustained VT that recurs despite AAD, when AAD are not
tolerated, incessant VT without a reversible cause
▪ Evidence:
o Among the earliest trials in VT ablation was SMASH-VT (NEJM, 2007) in which VT ablation was
associated with reduced incidence of ICD therapies.
o The VANISH trial (NEJM, 2016) randomized patients to escalation of AAD versus VT in patients
with ischemic cardiomyopathy with recurrent VT. VT ablation was associated with a 10% absolute
reduction in the primary endpoint (composite of death, VT storm, ICD shocks); there was no
difference in mortality
▪ See Chapter 5 – Electrophysiology Studies and Procedures for further details
84
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
References:
1. Antunes, Eduardo, et al. "The differential diagnosis of a regular tachycardia with a wide QRS complex on the 12-
lead ECG: ventricular tachycardia, supraventricular tachycardia with aberrant intraventricular conduction, and
supraventricular tachycardia with anterograde conduction over an accessory pathway." Pacing and clinical
electrophysiology: PACE 17.9 (1994): 1515
2. Trial, Cardiac Arrhythmia Suppre4ssion. "Preliminary report: effect of encainide and flecainide on mortality in a
randomized trial of arrhythmia suppression after acute myocardial infarction." N. Engl. J. Med. 321 (1989): 406-
412
3. Jain, Rahul, et al. "Electrocardiographic features of arrhythmogenic right ventricular dysplasia." Circulation 120.6
(2009): 477
4. Bakker, Annelies LM, et al. "The Lewis lead: making recognition of P waves easy during wide QRS complex
tachycardia." Circulation 119.24 (2009): e592-e593
5. Brugada, Pedro, et al. "A new approach to the differential diagnosis of a regular tachycardia with a wide QRS
complex." Circulation 83.5 (1991): 1649-1659
6. Vereckei, András, et al. "New algorithm using only lead aVR for differential diagnosis of wide QRS complex
tachycardia." Heart rhythm 5.1 (2008): 89-98
7. Exner, Derek V., et al. "Beta-blocker use and survival in patients with ventricular fibrillation or symptomatic
ventricular tachycardia: the Antiarrhythmics Versus Implantable Defibrillators (AVID) trial." Journal of the
American College of Cardiology 34.2 (1999): 325-333
8. Defibrillators, Antiarrhythmics Versus Implantable. "Investigators. A comparison of antiarrhythmic-drug therapy
with implantable defibrillators in patients resuscitated from near-fatal ventricular arrhythmias." N Engl J
Med 337.22 (1997): 1576-1583
9. Sapp, John L., et al. "Ventricular tachycardia ablation versus escalation of antiarrhythmic drugs." New England
Journal of Medicine 375.2 (2016): 111-121
10. Tung, Roderick, et al. "Freedom from recurrent ventricular tachycardia after catheter ablation is associated with
improved survival in patients with structural heart disease: an International VT Ablation Center Collaborative
Group study." Heart rhythm 12.9 (2015): 1997-2007
11. Al-Khatib, Sana M., et al. "2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias
and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart
Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society." Journal of the American
College of Cardiology 72.14 (2018): e91-e220
85
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
A narrow complex tachycardia (NCT) refers to a tachycardia with QRS duration <120ms and typically implies normal
electromechanical conduction from a supraventricular origin. Generally, it is helpful to organize NCTs by mechanism
(automaticity, re-entry, or triggered activity) and anatomic origin (sinus node, atria, AV node).
Protocol: atropine 0.5mg IV q3-5min (max 3mg) 🡪 transcutaneous pacing +/- epinephrine gtt +/- dopamine gtt
Medications:
● atropine 0.5mg IV q3-5min (max 3mg) [avoid in Mobitz II/CHB]
● dopamine 5-15μg/kg/min (β1 predominant; usually 200-800 μg/min)
● epinephrine 2-10μg/min
● isoproterenol 2-10μg/min
● glucagon 3-10mg bolus followed by 3-5mg/h gtt for βB or CCB overdose
Transcutaneous pacing
1. Place Zoll pads, sedate (ideally fentanyl/midazolam with Anesthesia present, but can use morphine/lorazepam if
emergent)
2. set mode to pace, increase rate to 100bpm, and set pacing output (stimulus needed to capture) to 140mA (if code
setting, otherwise start lower), and ensure capture w/ pulse.
3. Intubation is not necessary for pacing, but can be considered for airway protection as part of ACLS protocol
4. Will require placement of transvenous pacing wire See Chapter 4 – Device Therapy
Pathophysiology of Bradyarrhythmias
86
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Sinus bradycardia
● ECG: HR less than 50-60 bpm, normal p wave axis
● Pathophysiology:
o Most often physiologic and asymptomatic, seen in healthy individuals, endurance athletes, and during sleep
where it is not uncommon to see rates briefly in the 30s or pauses of up to 2 seconds
o Pathophysiologic causes include SA node dysfunction, medications, 15-25% of MI (RCA supplies SA node in
60% of individuals), sleep apnea, and exaggerated vagal tone (e.g. vasovagal syncope) (Circulation, 2020;
142:15)
● Treatment:
o Acute, unstable: Call code + initiate ACLS Bradycardia algorithm with immediate placement of temporary
pacing pads on the patient (see management of unstable bradycardia section)
o Chronic treatment: See Chapter 4 – Device Therapy for indications for pacemaker therapy
● When the block is within the AV node (first-degree or Mobitz I), subsidiary pacemakers at the AV junction usually take
over the pacemaker function of the heart, resulting in a relatively stable, non-life-threatening heart rhythm.
● Infra-nodal blocks (within the His bundle) typically result in unstable escape rhythms that may require temporary or
permanent pacing
87
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
88
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
89
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
References:
Fogoros, Richard N. Electrophysiologic Testing. Chichester: Wiley-Blackwell, 2012.
Patterson E, Scherlag BJ et al. Decremental conduction in the posterior and anterior AV nodal inputs. Journal of
Interventional Cardiac Electrophysiology (2002) 7 (2): 137–48.
Tisdale et al. Drug- Induced Arrythmias: A Scientific Statement From the American Heart Association. Circulation 142
(2020
90
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Device Therapy
The purpose of this section is to review the specific indications for temporary pacing; permanent pacemakers (PPMs),
cardiac resynchronization therapy (CRT), and implantable cardioverter-defibrillator (ICD) therapy.
Temporary Pacing
Transcutaneous Pacing
Indications: Formal indications for transcutaneous pacing are provided in Table 1 below. Indications for pad placement (to
allow for transcutaneous pacing instantly if needed) are also included and designated by an asterisk. These patients will
generally proceed to urgent insertion of transvenous pacing wire.
1. Place pads: Note that all MGH defibrillators can provide transcutaneous pacing. Proper pad placement is shown in the
figure below. Ensure that the pads are connected to the defibrillator and that the ECG leads are connected to the patient
(white is right, smoke over fire = white lead on R shoulder, black lead on left shoulder, red lead on left leg) and the
defibrillator.
Anterior pad
Posterior pad (usually
placed over L apex)
2. Administer sedation: Involve anesthesia for all non-ACLS protocol transcutaneous pacing to assist with administration
of medications that may cause respiratory compromise (such as IV midazolam/fentanyl). If pacing must be initiated
immediately, IV Dilaudid +/- IV Ativan may be used.
3. Initiate Pacing: Turn the defibrillator dial to “pacer” and set the pacing rate and output, which is the electrical stimulus
needed to produce capture; a higher output represents more energy and will generally be more painful. A reasonable
initial rate and output are 100 bpm and 100 mA, but note that the upper limit is around 140 mA. In non-emergent
situations where you do not need to ensure immediate capture, it is reasonable to start with a lower output around 40 mA
and increase by 5-10 mA until capture is achieved.
4. Verify Capture: Because the pacing rate should be well above the patients native rate, all beats should be paced.
Appropriate capture is confirmed if pacing spikes precede all QRS complexes (and fire at the set rate, e.g. 100 bpm). If
this is not the case, increase the output by 5-10 mA up to 140 mA. Confirm that paced/captured beats are perfusing with
either palpation or Doppler of femoral pulse.
5. Set Final Output: If capture is achieved immediately (e.g. with an initial output set to 100 mA), find the capture
threshold by decreasing the output slowly until capture is lost. The minimum output needed to maintain output will then be
10% higher than the capture threshold. In healthy patients, theoretical thresholds should range from 40-80 mA
(corresponding to an output range of 44-88 mA or, more practically, 45-90). In clinical practice, thresholds tend to be more
variable, with a range between 20-140 mA.
Transvenous Pacing
91
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Indications: Transvenous pacing is a more durable option than transcutaneous pacing and is employed as a temporary
bridge to recovery or to PPM placement.
Special considerations: In contrast to transcutaneous pacing (which can be performed urgently without input from
cardiology), placement of a temp wire for transvenous pacing must be performed in the cath lab. The pacing lead is
inserted into the right ventricle via the right internal jugular, left subclavian, or femoral veins (less reliable and increased
risk for dislodgement). Temp wires may remain in place for up to 7-10 days but carry a risk of infection. For this reason,
prophylactic antibiotics are indicated during and after implantation.
Additional complications include: lead dislodgement, bleeding, RV perforation, tamponade, thrombophlebitis, PE or air
embolism, pneumothorax, subdiaphragmatic stimulation, arrhythmias (ectopy/asystole), and/or permanent RBBB.
92
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
1. Increase heart rate on device such that all beats are paced
2. Decrease output until pacing ceases and native rhythm is seen, this defines the output threshold of the temporary
pacer
3. Increase output back up to its initial value (or 5-10x the threshold) and turn the rate back to its initial value
4. Do not touch sensitivity, unless troubleshooting
5. Document the threshold, output (in mA), and patient’s native (underlying) rhythm
Note that for RV pacing, the ECG should show LBBB, with left superior axis-usually upright in I and aVL. Any change in
pacing morphology is lead displacement until proven otherwise. If there is concern for change in lead position, perform
threshold testing again and get a STAT CXR.
1. Oversensing: pacer senses noise artifact (background activity) as a QRS complex and does not pace. Decrease
sensitivity or turn off sensing (asynchronous pacing, VOO).
2. Battery or connection problem: Replace battery and check connections.
93
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
2. Lead migration (epicardial lead migration can result in ventricular perforation). Troubleshoot with unipolar ECG:
connect V1 electrode to distal-most pacing electrode. Normal endocardial contact should result in negative QRS
deflection with ST elevation. A positive or biphasic QRS is suggestive of lead migration.
3. Physiologic effect: Such as ischemia, hypoxia, acidosis, alkalosis, hyperglycemia, hypercapnia, medication
related. Attempt to reverse the underlying condition
4. Battery or connection problem. Replace battery and check connections.
Failure to sense: The device does not detect intrinsic cardiac activity and fires inappropriately. Pacing spikes can be
seen on top of or after native QRS (increases risk of R on T).
Permanent Pacing
Permanent pacing is indicated for symptomatic or dangerous forms of bradycardia that are not due to transient or
reversible causes.
AVB
o 2°AVB causing symptomatic bradycardia
o 2° or 3°AVB during exercise without evidence of myocardial ischemia
o 3°AVB with HR≥40 if cardiomegaly or LV dysfunction present or if block below AV node
o 3° or adv. 2°AVB causing symptomatic bradycardia or VT/VF
o 3° or adv. 2°AVB and awake/asymptomatic in NSR w/ pause ≥3s, infranodal escape or
escape HR<40
o 3° or adv. 2°AVB and awake/asymptomatic in AF w/ bradycardia and pause ≥5s
o Persistent 3° or advanced 2°AVB occurring after AV node ablation or cardiac surgery
Chronic Bifascicular Block
Advanced 2° or 3°AVB
Type 2° with alternating bundle branch block
AV Block after Acute MI
Persistent and symptomatic 2° or 3°AVB
Persistent 2° infranodal AVB with alternating BBB or 3° infranodal AVB
Hypersensitive Carotid Sinus Syndrome/Neurocardiogenic Syncope
Recurrent syncope due to spontaneous carotid sinus stimulation with asystole ≥3 seconds
Pacemaker modes:
94
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
95
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Note: AVM: AV delay management, SND: Sinus node dysfunction, AF: Atrial fibrillation, CRT: Cardiac
resynchronization therapy.
AAI: Single right atrial lead paces unless native atrial activity is sensed. Useful for patients with isolated sinus node
dysfunction, manifesting as sinus bradycardia or sinus pauses. Rate-responsiveness can be added for patients who
cannot accelerate their heart rates (AAI-R). Patients must have intact AV conduction. A major disadvantage is that there is
no protection from ventricular bradyarrhythmias in the setting of AV block which develops in 0.6–5% of patients with sick-
sinus syndrome per year. Pacemaker upgrade is often a higher-risk procedure than de novo implantation of a dual-
chamber system. In patients with sinus node dysfunction, the presence of bundle-branch block at implantation is a
relatively accurate predictor of subsequent AV block, and dual chamber pacemaker should be considered instead.
VVI: Single right ventricular lead with paces unless ventricular activity is sensed. Very common mode for prevention of
ventricular bradyarrhythmias or asystole. Virtually all devices currently in use are capable of VVI(R) pacing. Unlike AAI
pacing, VVI pacing is effective even in patients with impaired AV conduction or in patients at risk for future development
of AV block. VVI(R) pacing in particular is indicated in patients with chronic atrial fibrillation with a slow ventricular
response. The drawback to VVI pacing is that this mode cannot maintain AV synchrony and lack of AV synchrony can
result in pacemaker syndrome as well as increased risk of atrial fibrillation.
DDD: Right atrial and right ventricular leads which are both capable of pacing in the absence of native activity sensed in
either respective chamber, but inhibited if it is. Provides closest mimic of physiologic pacing conditions and maintains AV
synchrony which reduces the risk of AF in sick sinus syndrome, resulting in a lower incidence of thromboembolic events,
improves hemodynamics by preserving the atrial kick, and helps to avoid pacemaker syndrome. Results in one (or more
at any given time) of four different rhythms: normal sinus rhythm, atrial pacing with intact AV conduction, AV sequential
pacing, and atrial sensing and ventricular pacing (P-synchronous ventricular pacing).
96
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
1.
2. 3.
4.
1: a-paced, v-sensed. 2: a-sensed, v-sensed (normal conduction). 3: a-paced, v-paced. 4: a-sensed, v-paced.
Subsection References:
1. Tracy, Cynthia M., et al. "2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008
guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of
Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm
Society." Journal of the American College of Cardiology 61.3 (2013): e6-e75
2. Kusumoto, Fred M., et al. "2018 ACC/AHA/HRS guideline on the evaluation and management of patients with
bradycardia and cardiac conduction delay: a report of the American College of Cardiology/American Heart
Association Task Force on clinical practice guidelines and the Heart Rhythm Society." Journal of the American
College of Cardiology (2018): 25701
3. Trohman, Richard G., Michael H. Kim, and Sergio L. Pinski. "Cardiac pacing: the state of the art." The
Lancet 364.9446 (2004): 1701-1719
4. Fitzpatrick, Adam, and Richard Sutton. "A guide to temporary pacing." BMJ: British Medical Journal 304.6823
(1992): 365
5. Gammage, Michael D. "Temporary cardiac pacing." Heart 83.6 (2000): 715-720
97
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Overview: These devices have been shown to be 97% effective in terminating arrhythmias with defibrillation,
cardioversion or antitachycardia pacing (ATP). Additionally, all ICDs can function as a pacemaker (though note that the
reverse is not true). ICDs can be single-chamber (RV-lead) or dual chamber (RA/RV-lead) devices. They can be
distinguished from dual chamber PPMs on CXR by presence of coils.
Indications: Per AHA/ACC guidelines, 1-3 implantation of ICD should be considered in patients on optimal medical therapy
who have an estimated survival of at least 1 year (with good functional status). There are Class I indications for both
primary and secondary prevention, as well as Class II indications for conditions that increase the risk of SCD.
Class I
Secondary Prevention: Survivors of cardiac arrest due to VF or sustained VT without reversible causes
Primary Prevention:
- Ischemic cardiomyopathy and LVEF ≤ 30-40% at least 40 days post-MI (DINAMIT) or at least 3 months post-
CABG (CABG-PATCH)
- LVEF ≤ 30% and NYHA Class I, II, or III (MADIT-II)
- LVEF ≤ 35% and NYHA Class II or III (SCD-HeFT)
- LVEF ≤ 40% and inducible VF or sustained VT at EP study (MUSTT)
- Non-ischemic cardiomyopathy with at least 3 months of documented
- CHF, LVEF ≤ 35%, and NYHA Class II or III (SCD-HeFT)*
- Syncope of unknown origin and VT/VF induced at EP study
- Spontaneous sustained VT (+/- hemodynamic instability) with structural heart disease (*DANISH Study: RCT of
non-ischemic heart failure with EF ≤ 35%, showed no overall mortality benefit from ICD versus optimal medical
therapy, however risk of SCD was reduced and a mortality benefit was observed in the subgroup that was <68
years old)
Class II
For numerous conditions that increase risk of SCD, with limited RCT data
- Hypertrophic cardiomyopathy: LV thickness >/= 30mm, family history of SCD, abnormal exercise BP
- Arrhythmogenic right ventricular cardiomyopathy (ARVC): extensive RV, LV involvement
- Long-QT syndrome: syncope and/or VT while on beta-blockers
- Brugada syndrome: syncope and/or VT
- Catecholaminergic polymorphic VT: syncope and/or sustained VT on beta-blockers
- Cardiac sarcoid, giant cell myocarditis, Chagas disease
- Unexplained syncope, significant LV dysfunction, and non-ischemic dilated cardiomyopathy
- Sustained VT with normal ventricular function
- Non-hospitalized patient awaiting heart transplant
98
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
CRT is an important therapy among patients with HFrEF which portends a robust mortality and reverse remodeling
benefit. Initially shown to improve exercise capacity, clinical symptoms, and LVEF in the MIRACLE Study. Further
investigation demonstrated a mortality benefit in patients with HFrEF and widened QRS.
CRT implantation involves RA, RV, and coronary sinus (for epicardial pacing of LV) leads. This allows for optimization of
A-V synchrony as well as V-V synchrony, which has been shown to improve stroke volume. For this reason, CRTs
represents an important component of guideline directed medical and device therapy. CRT devices may only provide BiV
pacing (CRT-P) or may allow for BiV pacing plus defibrillation with ICD function (CRT-D).
Indications for CRT are divided into two groups, those in sinus rhythm (table 4) and those who are in persistent or
permanent atrial fibrillation who either cannot undergo ablation or who have received ablation and failed (table 5). For
those who fall into those mentioned groups with atrial fibrillation, further consideration can be for AV junction ablation and
simultaneous CRT which can both improve symptoms and reduce mortality (see figure 5 for further decision making and
patient selection for ablation and/or CRT) in patients who either cannot tolerate or who do not respond to other methods of
rate and rhythm control with the understanding that AVJ ablation will effectively render them pacemaker dependent. Most
of the evidence for either group of patients are based on RCT’s who enrolled patients who are NYHA II-IV with LVEF 35%
or less with some exceptions in the atrial fibrillation groups detailed below who have received optimal medical therapy or
OMT for at least 3 months after being diagnosed with HFrEF or at least 40 days following diagnosis of a myocardial
infarction in addition to treatment of any reversible cardiomyopathy such as ischemic or tachycardia-induced
cardiomyopathy. Additional recommendations for those with pre-existing ICD’s can be found in the 2021 ESC guidelines
on cardiac pacing and cardiac resynchronization therapy from which the below tables are pulled. 15
99
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
100
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
101
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
102
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
References:
1. Tracy, Cynthia M., et al. "2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008
guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of
Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm
Society." Journal of the American College of Cardiology 61.3 (2013): e6-e75
2. Kusumoto, Fred M., et al. "2018 ACC/AHA/HRS guideline on the evaluation and management of patients with
bradycardia and cardiac conduction delay: a report of the American College of Cardiology/American Heart
Association Task Force on clinical practice guidelines and the Heart Rhythm Society." Journal of the American
College of Cardiology (2018): 25701
3. Higgins, Steven L. "Impact of the Multicenter Automatic Defibrillator Implantation Trial on implantable cardioverter
defibrillator indication trends." The American journal of cardiology 83.5 (1999): 79-82
4. Klein, Helmut U., and Sven Reek. "The MUSTT study: evaluating testing and treatment." Journal of Interventional
Cardiac Electrophysiology 4.1 (2000): 45-50
5. Moss, Arthur J., et al. "Prophylactic implantation of a defibrillator in patients with myocardial infarction and
reduced ejection fraction." New England Journal of Medicine 346.12 (2002): 877-883
6. Bardy, Gust H., et al. "Amiodarone or an implantable cardioverter–defibrillator for congestive heart failure." New
England Journal of Medicine 352.3 (2005): 225-237
7. Hohnloser, Stefan H., et al. "Prophylactic use of an implantable cardioverter–defibrillator after acute myocardial
infarction." New England Journal of Medicine 351.24 (2004): 2481-2488
8. Myerburg, Robert J. "Implantable cardioverter–defibrillators after myocardial infarction." New England Journal of
Medicine359.21 (2008): 2245-2253
9. Tang, Anthony SL, et al. "Cardiac-resynchronization therapy for mild-to-moderate heart failure." New England
Journal of Medicine 363.25 (2010): 2385-2395
10. Bristow, Michael R., et al. "Cardiac-resynchronization therapy with or without an implantable defibrillator in
advanced chronic heart failure." New England Journal of Medicine 350.21 (2004): 2140-2150
11. Cleland, John GF, et al. "The effect of cardiac resynchronization on morbidity and mortality in heart failure." New
England Journal of Medicine 352.15 (2005): 1539-1549
12. Curtis, Anne B., et al. "Biventricular pacing for atrioventricular block and systolic dysfunction." New England
Journal of Medicine 368.17 (2013): 1585-1593
13. Ruschitzka, Frank, et al. "Cardiac-resynchronization therapy in heart failure with a narrow QRS complex." New
England Journal of Medicine 369.15 (2013): 1395-1405
14. Olgin, Jeffrey E., et al. "Wearable cardioverter–defibrillator after myocardial infarction." New England Journal of
Medicine379.13 (2018): 1205-1215.
15. Michael Glikson et al, ESC Scientific Document Group, 2021 ESC Guidelines on cardiac pacing and cardiac
resynchronization therapy: Developed by the Task Force on cardiac pacing and cardiac resynchronization therapy
of the European Society of Cardiology (ESC) With the special contribution of the European Heart Rhythm
Association (EHRA), European Heart Journal, Volume 42, Issue 35, 14 September 2021, Pages 3427–3520.
.
103
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
The purpose of this section is to provide background/basic information on three commonly employed EP procedures:
synchronized cardioversion and defibrillation, diagnostic EP studies, and catheter ablations.
Cardioversion (DCCV) and defibrillation are non-invasive interventions that can terminate tachyarrhythmias by delivering
an electrical shock to the heart. Cardioversion is performed by synchronizing the shock to a QRS complex while
defibrillation is unsynchronized and delivers a random shock during the cardiac cycle.
Emergent DCCV and defibrillation are indicated in cases of clinical instability. Elective DCCV may also be performed as
an effective treatment for SVTs.
DEFIBRILLATION
VT (pulseless) 360 150-200
VF 360 150-200
¹includes reentry arrhythmias AVRT and AVNRT (attempt adenosine first)
²includes:
AF/AFL of ≥48h (or unknown) duration and anticoagulated for 3–4 weeks (INR 2–3)
AF/AFL of <48h duration, for which anticoagulation is optional depending on risk
AF/AFL of unknown duration with CHF and absence of thrombus in LA on TEE acute onset AF/AFL with
hemodynamic compromise: MI, pulmonary edema, hypotension
*
All MGH defibrillators are biphasic which are typically effective at lower energy levels. Large patients may be
difficult to cardiovert/defibrillate even at 360 J biphasic.
1. Patients who are pregnant: Can be performed without affecting the rhythm of the fetus. Fetal heart rate monitoring
during the procedure is recommended.
2. Patients who have pacemakers/ICDs: Can be performed but the electrode should be > 12 cm from the pulse
generator and an anteroposterior electrode orientation is recommended. The pacemaker/ICD should be interrogated
afterward to ensure proper functioning.
104
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Defibrillation:
1. Turn the mode selector to DEFIB (red area)
a. The unit displays DEFIB 120J SEL on the monitor
b. The default energy selections for adult patients are Shock 1: 120J, Shock 2: 150J, Shock 3: 200J. You
can use energy select (UP and DOWN arrow keys) to change settings
2. If the monitor shows a shockable rhythm, press the CHARGE button on the front panel. If patient is pulseless,
continue CPR while charging
3. Ensure all are clear from the bed then press and hold the illuminated SHOCK button
4. If patient is pulseless, resume CPR for 2 minutes before the next pulse and rhythm check
For non-emergent and routine procedures: NPO for at least 6-8h. Some patients may require periprocedural
anticoagulation to prevent thromboembolism. TEE is frequently used to look for the presence of a LA thrombus.
Complications: After shocking, ST & T wave changes may occur and typically resolve within 5 minutes. However, the
following complications may also occur:
● Arrhythmias/Conduction abnormalities:
o Self-limited and include sinus tachycardia, PAC, PVC, NSVT, VT, VF, and LBBB.
o May cause hemodynamic instability requiring further intervention
● Thromboembolism: Approximately 1% with anticoagulation, 5% without anticoagulation
● Myocardial necrosis: Severity depends on strength and number of shocks delivered
● Myocardial dysfunction/stunning:
o Global LV dysfunction can be present until 48 hours after shock
o Interpret cardiac studies within this 48h window with caution
● Pulmonary edema: Rare complication seen in patients with AF and/or LV dysfunction
● Hypotension: Transient, may last several hours, typically fluid-responsive
● Cutaneous burns:
o Seen in 20-25% of patients
o May use prophylactic steroid cream or topical NSAIDs at the site of electrodes
105
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
EPS is an elective, invasive, intracardiac catheter-based intervention that measures specific intervals within the
cardiac cycle to better understand the etiology, clinical significance, and management of brady- and tachy-
arrhythmias.
Indications:
1. Bradyarrhythmias: acquire corroborative data in symptomatic patients with episodic bradyarrhythmia or define level
of AV conduction abnormality
2. Tachyarrhythmias: define mechanism of NCT or WCT or reproduce a clinically defined tachycardia for mapping
and ablation
3. ICD implantation and programming: search for EPS-induced VT or VF in patients with structural heart disease,
NSVT, or reduced LVEF to justify ICD implantation and define optimal ICD parameters
4. Syncope of unknown etiology: to assess SN function, AV conduction, and search for VT/VF in patients with
syncope and organic heart disease
5. After cardiac surgery: to assess the effect of cardiac surgery on arrhythmia risk
Contraindications: active ACS, bacteremia, ADHF (not caused by the arrhythmia), critical AS, severe HOCM, severe left
main or 3-vessel CAD, presence of a major bleeding diathesis, presence of an acute lower extremity DVT (if femoral vein
cannulation is required).
Performed in the EP lab and patients are given at least conscious sedation. Standard ECG leads and defibrillation pads
are applied. Multiple sites of venous (and at times, arterial) access are acquired for intracardiac catheter placement.
Femoral access is most common. Catheters with electrode tips are placed in specific locations to both pace and record.
Mapping
106
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Indications
Contraindications are generally the same as for EPS (see prior section).
Indicated for patients with symptomatic AF who have either failed pharmacologic therapies for rhythm control or cannot
tolerate medication side effects. Among patients with HFrEF and NYHA II-III symptoms with persistent AF who are chosen
for a rhythm control strategy, PVI leads 36% absolute increase in AF-free survival at 24 months, 26% absolute reduction
in unplanned hospitalizations, and a 10% absolute reduction in overall mortality with AF ablation as compared to
amiodarone.
Can be accomplished with radiofrequency (RFA) or cryoablation. In patients with drug refractory, symptomatic paroxysmal
AF, cryoablation is non-inferior, reduces procedural time, but increases fluoroscopy time as compared to RFA. The
procedure aims to electrically isolate the antral portion of the pulmonary veins, a region identified as containing >90% of
the ectopic beats involved in the generation and maintenance of AF. As a result, cavotricuspid isthmus line ablation can
be performed during the same procedure if atrial flutter is co-occurring.
Efficacy at 1 year has been reported to be as high as 80-85% for paroxysmal AF but decreases to 50-60% for persistent
AF.
Anticoagulation:
As with any cardioversion, there is a risk of thromboembolism with PVI. All patients should be on anticoagulation peri-
procedurally. Patients with AF >48 hours should be on therapeutic anticoagulation for at least 3 weeks or have a TEE
prior to PVI. Anticoagulation should continue for at least two months after the procedure, although based on patient
wishes, risk factors/stratification, and aggressive post-ablation monitoring some EP physicians will choose to discontinue
anticoagulation.
Complications:
1. Complete heart block 5. Pericarditis
2. Thromboembolism 6. Pulmonary vein stenosis
3. Cardiac tamponade 7. Phrenic nerve paralysis
4. Acute MI 8. Atrioesophageal fistula
107
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
References:
1. American Heart Association, American Heart Association, and American Heart Association. "guidelines for
cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal
patients: neonatal resuscitation guidelines." Pediatrics 117.5 (2006): e1029-e1038
2. Jacobs, Ian, et al. "Part 6: defibrillation: 2010 international consensus on cardiopulmonary resuscitation and
emergency cardiovascular care science with treatment recommendations." Circulation 122.16_suppl_2 (2010):
S325-S337
3. Lown, Bernard, Raghavan Amarasingham, and Jose Neuman. "New method for terminating cardiac arrhythmias:
use of synchronized capacitor discharge." JAMA 256.5 (1986): 621-627
4. Knight, B. “Basic principles and techniques of cardioversion and defibrillation.” UpToDate 2018
5. Knight, B. “Cardioversion for specific arrhythmias.” UpToDate 2019
6. Kadish, Alan H., et al. "ACC/AHA clinical competence statement on electrocardiography and ambulatory
electrocardiography: A report of the ACC/AHA/ACP–ASIM Task Force on Clinical Competence (ACC/AHA
Committee to Develop a Clinical Competence Statement on Electrocardiography and Ambulatory
Electrocardiography) Endorsed by the International Society for Holter and Noninvasive Electrocardiology." Journal
of the American College of Cardiology 38.7 (2001): 2091-2100
7. Zipes, Douglas P., et al. "Guidelines for clinical intracardiac electrophysiological and catheter ablation procedures:
a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines
(Committee on Clinical Intracardiac Electrophysiologic and Catheter Ablation Procedures), developed in
collaboration with the North American Society of Pacing and Electrophysiology." Journal of the American College
of Cardiology 26.2 (1995): 555-573
8. Homoud, M. “Invasive cardiac electrophysiology studies.” UpToDate 2019
9. Dagres, Nikolaos, et al. "Complications of atrial fibrillation ablation in a high‐volume center in 1,000 procedures:
Still cause for concern?." Journal of cardiovascular electrophysiology 20.9 (2009): 1014-1019
10. Haissaguerre, Michel, et al. "Spontaneous initiation of atrial fibrillation by ectopic beats originating in the
pulmonary veins." New England Journal of Medicine 339.10 (1998): 659-666
11. Oral, Hakan, et al. "Circumferential pulmonary-vein ablation for chronic atrial fibrillation." New England Journal of
Medicine354.9 (2006): 934-941
12. Pappone, Carlo, and Vincenzo Santinelli. "Atrial fibrillation ablation: state of the art." The American journal of
cardiology96.12 (2005): 59-64
13. Ganz, L. “Overview of catheter ablation of cardiac arrhythmias.” UpToDate 2019
14. Passman, R. “Catheter ablation to prevent recurrent atrial fibrillation.” UpToDate 2018
15. Kuck, Karl-Heinz, et al. "Cryoballoon or radiofrequency ablation for paroxysmal atrial fibrillation." New England
Journal of Medicine 374.23 (2016): 2235-2245
16. Di Biase, Luigi, et al. "Ablation versus amiodarone for treatment of persistent atrial fibrillation in patients with
congestive heart failure and an implanted device: results from the AATAC multicenter randomized
trial." Circulation 133.17 (2016): 1637-1644
108
Kyle Saysana
Arrhythmias & Electrophysiology
TOC
MECHANISM
↑ AUTOMATICITY RE-ENTRY
S
Sinus Tachycardia Sino-Atrial Nodal Reentrant Tachycardia
I
● Secondary cause (HoTN, pain, fever, ● Reentry within or around SA node
N
PE, CHF, acute MI, fistula, etc.) vs drug ● Paroxysmal, abrupt on/off
U
effect/toxicity/withdrawal ● P wave morphology similar to baseline
S
● HR 100-150 in older pts; up to 200 in ● Treat w/ vagal maneuvers, Valsalva;
N
younger pts nodal blockade; ablation
O
● Gradual onset
D
● Treat underlying cause
E
Non-Reentrant AVNRT
Junctional Tachycardia ● Reentry thru dual pathways in AV node
● JET (junctional ectopic tach): infants > ● Abrupt on/off, rates 150-220
adults, particularly post-cardiac surgery ● Retrograde P, can be buried in QRS
A for CHD; due to ectopic focus; high ● Acute: vagal, adenosine
V morbidity ● Mgmt: BB, CCB; ablation is definitive
N ● NPJT (non-paroxysmal JT): adults >
O children; “accelerated junctional AVRT
D rhythm”; a/w digoxin toxicity and acute ● Reentry thru acc. pathway + AV node
E MI; difficult to distinguish from AVNRT ● Narrow = orthodromic (antegrade thru
w/o EP study; treat underlying cause AV node, retrograde thru acc. path);
use nodal blockade
● Wide/pre-excitation = antidromic; avoid
nodal blockade (see WCT chapter)
● Definitive tx: ablation
109
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
110
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
● There are many different pacing modes, which are designated by a 1-5 letter code. In the chart below, “position”
refers to letter position
o As an example, in the 4-letter code DDDR, the D in position I implies that both chambers (the atria and
the ventricles) can be paced, the D in position II indicates that both chambers (the atria and the
ventricles) can be sensed, the D in position III indicates that the device can respond to sensing by
either triggering activity or inhibiting itself, and the R in position IV indicates that the device allows for
rate modulation
111
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
● Emergent DCCV and defibrillation are generally indicated in cases of clinical instability. Planned DCCV may also
be performed as an effective treatment for SVTs
● The delivered shock depolarizes all or most excitable cardiac tissue and induces a refractory period that
terminates reentrant activity
● Cardioversion is performed by synchronizing the shock to a QRS complex (thus requires a rhythm in which the
QRS complex is present) and restores sinus rhythm in 70–95% of patients. The procedure is as follows:
21. Turn the mode selector to DEFIB (red area). Select the desired energy using the up and down arrow keys in the
front panel. In general:
a. Narrow, regular: 50 – 100 J (atrial flutter often converts with 50 J)
b. Narrow, irregular: 120 – 200 J (atrial fibrillation typically requires 150 J)
c. Wide, regular: 100 J
d. Wide, irregular: 150 – 200 J (defibrillation dose)
22. Press the Sync On/Off button
a. Confirm that a Sync marker appears on the monitor above each detected R wave to indicate where
discharge will occur
b. If necessary, use the LEAD and SIZE buttons to establish settings that yield the best display
23. Press the CHARGE button on the front panel
24. Ensure all are clear from the bed then press and hold the illuminated SHOCK button on the front panel. The
defibrillator will discharge with the next detected R wave
25. If additional shocks are necessary, increase the energy level as needed
● Defibrillation is unsynchronized and delivers a random shock during the cardiac cycle.
5. Turn the mode selector to DEFIB (red area)
a. The unit displays DEFIB 120J SEL on the monitor
b. The default energy selections for adult patients are Shock 1: 120J, Shock 2: 150J, Shock 3: 200J. You
can use energy select (UP and DOWN arrow keys) to change settings
6. If the monitor shows a shockable rhythm, press the CHARGE button on the front panel. If patient is
pulseless, continue CPR while charging
7. Ensure all are clear from the bed then press and hold the illuminated SHOCK button on the front panel
8. If patient is pulseless, resume CPR for 2 minutes before the next pulse and rhythm check
● An electrophysiology study (EPS) is an elective, invasive, intracardiac catheter-based intervention that
measures specific intervals within the cardiac cycle. The purpose is to better understand the etiology,
clinical significance, and management of brady-and tachy-arrhythmias
● A common procedure in EP is Pulmonary Vein Isolation (PVI), performed with either radiofrequency (RFA)
or cryoablation, which involves electrical isolation of the antral portion of the pulmonary veins, which
contains >90% of the ectopic beats involved in AFib.
PVI is indicated in patients with symptomatic atrial fibrillation who have either failed pharmacologic therapies for
rhythm control or cannot tolerate medication side effects. Importantly, ablation does not negate the need for
therapeutic anticoagulation
The purpose of this section is to provide background/basic information on three commonly employed EP procedures:
synchronized cardioversion and defibrillation, diagnostic EP studies, and catheter ablations.
112
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Cardioversion (DCCV) and defibrillation are non-invasive interventions that can terminate tachyarrhythmias by delivering
an electrical shock to the heart. Cardioversion is performed by synchronizing the shock to a QRS complex while
defibrillation is unsynchronized and delivers a random shock during the cardiac cycle.
Emergent DCCV and defibrillation are indicated in cases of clinical instability. Elective DCCV may also be performed as
an effective treatment for SVTs.
DEFIBRILLATION
VT (pulseless) 360 150-200
VF 360 150-200
¹includes reentry arrhythmias AVRT and AVNRT (attempt adenosine first)
²includes:
AF/AFL of ≥48h (or unknown) duration and anticoagulated for 3–4 weeks (INR 2–3)
AF/AFL of <48h duration, for which anticoagulation is optional depending on risk
AF/AFL of unknown duration with CHF and absence of thrombus in LA on TEE acute onset AF/AFL with
hemodynamic compromise: MI, pulmonary edema, hypotension
*
All MGH defibrillators are biphasic which are typically effective at lower energy levels. Large patients may be
difficult to cardiovert/defibrillate even at 360 J biphasic.
1. Patients who are pregnant: Can be performed without affecting the rhythm of the fetus. Fetal heart rate monitoring
during the procedure is recommended.
2. Patients who have pacemakers/ICDs: Can be performed but the electrode should be > 12 cm from the pulse
generator and an anteroposterior electrode orientation is recommended. The pacemaker/ICD should be interrogated
afterward to ensure proper functioning.
113
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
114
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Defibrillation:
9. Turn the mode selector to DEFIB (red area)
a. The unit displays DEFIB 120J SEL on the monitor
b. The default energy selections for adult patients are Shock 1: 120J, Shock 2: 150J, Shock 3: 200J. You
can use energy select (UP and DOWN arrow keys) to change settings
10. If the monitor shows a shockable rhythm, press the CHARGE button on the front panel. If patient is pulseless,
continue CPR while charging
11. Ensure all are clear from the bed then press and hold the illuminated SHOCK button
12. If patient is pulseless, resume CPR for 2 minutes before the next pulse and rhythm check
For non-emergent and routine procedures: NPO for at least 6-8h. Some patients may require periprocedural
anticoagulation to prevent thromboembolism. TEE is frequently used to look for the presence of a LA thrombus.
Complications: After shocking, ST & T wave changes may occur and typically resolve within 5 minutes. However, the
following complications may also occur:
● Arrhythmias/Conduction abnormalities:
o Self-limited and include sinus tachycardia, PAC, PVC, NSVT, VT, VF, and LBBB.
o May cause hemodynamic instability requiring further intervention
● Thromboembolism: Approximately 1% with anticoagulation, 5% without anticoagulation
● Myocardial necrosis: Severity depends on strength and number of shocks delivered
● Myocardial dysfunction/stunning:
o Global LV dysfunction can be present until 48 hours after shock
o Interpret cardiac studies within this 48h window with caution
● Pulmonary edema: Rare complication seen in patients with AF and/or LV dysfunction
● Hypotension: Transient, may last several hours, typically fluid-responsive
● Cutaneous burns:
o Seen in 20-25% of patients
o May use prophylactic steroid cream or topical NSAIDs at the site of electrodes
115
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
EPS is an elective, invasive, intracardiac catheter-based intervention that measures specific intervals within the
cardiac cycle to better understand the etiology, clinical significance, and management of brady- and tachy-
arrhythmias.
Indications:
6. Bradyarrhythmias: acquire corroborative data in symptomatic patients with episodic bradyarrhythmia or define level
of AV conduction abnormality
7. Tachyarrhythmias: define mechanism of NCT or WCT or reproduce a clinically defined tachycardia for mapping
and ablation
8. ICD implantation and programming: search for EPS-induced VT or VF in patients with structural heart disease,
NSVT, or reduced LVEF to justify ICD implantation and define optimal ICD parameters
9. Syncope of unknown etiology: to assess SN function, AV conduction, and search for VT/VF in patients with
syncope and organic heart disease
10. After cardiac surgery: to assess the effect of cardiac surgery on arrhythmia risk
Contraindications: active ACS, bacteremia, ADHF (not caused by the arrhythmia), critical AS, severe HOCM, severe left
main or 3-vessel CAD, presence of a major bleeding diathesis, presence of an acute lower extremity DVT (if femoral vein
cannulation is required).
Performed in the EP lab and patients are given at least conscious sedation. Standard ECG leads and defibrillation pads
are applied. Multiple sites of venous (and at times, arterial) access are acquired for intracardiac catheter placement.
Femoral access is most common. Catheters with electrode tips are placed in specific locations to both pace and record.
Mapping
116
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
Indications
Contraindications are generally the same as for EPS (see prior section).
Indicated for patients with symptomatic AF who have either failed pharmacologic therapies for rhythm control or cannot
tolerate medication side effects. Among patients with HFrEF and NYHA II-III symptoms with persistent AF who are chosen
for a rhythm control strategy, PVI leads 36% absolute increase in AF-free survival at 24 months, 26% absolute reduction
in unplanned hospitalizations, and a 10% absolute reduction in overall mortality with AF ablation as compared to
amiodarone.
Can be accomplished with radiofrequency (RFA) or cryoablation. In patients with drug refractory, symptomatic paroxysmal
AF, cryoablation is non-inferior, reduces procedural time, but increases fluoroscopy time as compared to RFA. The
procedure aims to electrically isolate the antral portion of the pulmonary veins, a region identified as containing >90% of
the ectopic beats involved in the generation and maintenance of AF. As a result, cavotricuspid isthmus line ablation can
be performed during the same procedure if atrial flutter is co-occurring.
Efficacy at 1 year has been reported to be as high as 80-85% for paroxysmal AF but decreases to 50-60% for persistent
AF.
Anticoagulation:
As with any cardioversion, there is a risk of thromboembolism with PVI. All patients should be on anticoagulation peri-
procedurally. Patients with AF >48 hours should be on therapeutic anticoagulation for at least 3 weeks or have a TEE
prior to PVI. Anticoagulation should continue for at least two months after the procedure, although based on patient
wishes, risk factors/stratification, and aggressive post-ablation monitoring some EP physicians will choose to discontinue
anticoagulation.
Complications:
1. Complete heart block 5. Pericarditis
2. Thromboembolism 6. Pulmonary vein stenosis
3. Cardiac tamponade 7. Phrenic nerve paralysis
4. Acute MI 8. Atrioesophageal fistula
117
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
References:
17. American Heart Association, American Heart Association, and American Heart Association. "guidelines for
cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal
patients: neonatal resuscitation guidelines." Pediatrics 117.5 (2006): e1029-e1038
18. Jacobs, Ian, et al. "Part 6: defibrillation: 2010 international consensus on cardiopulmonary resuscitation and
emergency cardiovascular care science with treatment recommendations." Circulation 122.16_suppl_2 (2010):
S325-S337
19. Lown, Bernard, Raghavan Amarasingham, and Jose Neuman. "New method for terminating cardiac arrhythmias:
use of synchronized capacitor discharge." JAMA 256.5 (1986): 621-627
20. Knight, B. “Basic principles and techniques of cardioversion and defibrillation.” UpToDate 2018
21. Knight, B. “Cardioversion for specific arrhythmias.” UpToDate 2019
22. Kadish, Alan H., et al. "ACC/AHA clinical competence statement on electrocardiography and ambulatory
electrocardiography: A report of the ACC/AHA/ACP–ASIM Task Force on Clinical Competence (ACC/AHA
Committee to Develop a Clinical Competence Statement on Electrocardiography and Ambulatory
Electrocardiography) Endorsed by the International Society for Holter and Noninvasive Electrocardiology." Journal
of the American College of Cardiology 38.7 (2001): 2091-2100
23. Zipes, Douglas P., et al. "Guidelines for clinical intracardiac electrophysiological and catheter ablation procedures:
a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines
(Committee on Clinical Intracardiac Electrophysiologic and Catheter Ablation Procedures), developed in
collaboration with the North American Society of Pacing and Electrophysiology." Journal of the American College
of Cardiology 26.2 (1995): 555-573
24. Homoud, M. “Invasive cardiac electrophysiology studies.” UpToDate 2019
25. Dagres, Nikolaos, et al. "Complications of atrial fibrillation ablation in a high‐volume center in 1,000 procedures:
Still cause for concern?." Journal of cardiovascular electrophysiology 20.9 (2009): 1014-1019
26. Haissaguerre, Michel, et al. "Spontaneous initiation of atrial fibrillation by ectopic beats originating in the
pulmonary veins." New England Journal of Medicine 339.10 (1998): 659-666
27. Oral, Hakan, et al. "Circumferential pulmonary-vein ablation for chronic atrial fibrillation." New England Journal of
Medicine354.9 (2006): 934-941
28. Pappone, Carlo, and Vincenzo Santinelli. "Atrial fibrillation ablation: state of the art." The American journal of
cardiology96.12 (2005): 59-64
29. Ganz, L. “Overview of catheter ablation of cardiac arrhythmias.” UpToDate 2019
30. Passman, R. “Catheter ablation to prevent recurrent atrial fibrillation.” UpToDate 2018
31. Kuck, Karl-Heinz, et al. "Cryoballoon or radiofrequency ablation for paroxysmal atrial fibrillation." New England
Journal of Medicine 374.23 (2016): 2235-2245
32. Di Biase, Luigi, et al. "Ablation versus amiodarone for treatment of persistent atrial fibrillation in patients with
congestive heart failure and an implanted device: results from the AATAC multicenter randomized
trial." Circulation 133.17 (2016): 1637-1644
118
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
MECHANISM
↑ AUTOMATICITY RE-ENTRY
S
Sinus Tachycardia Sino-Atrial Nodal Reentrant Tachycardia
I
● Secondary cause (HoTN, pain, fever, ● Reentry within or around SA node
N
PE, CHF, acute MI, fistula, etc.) vs drug ● Paroxysmal, abrupt on/off
U
effect/toxicity/withdrawal ● P wave morphology similar to baseline
S
● HR 100-150 in older pts; up to 200 in ● Treat w/ vagal maneuvers, Valsalva;
N
younger pts nodal blockade; ablation
O
● Gradual onset
D
● Treat underlying cause
E
Non-Reentrant AVNRT
Junctional Tachycardia ● Reentry thru dual pathways in AV node
● JET (junctional ectopic tach): infants > ● Abrupt on/off, rates 150-220
adults, particularly post-cardiac surgery ● Retrograde P, can be buried in QRS
A for CHD; due to ectopic focus; high ● Acute: vagal, adenosine
V morbidity ● Mgmt: BB, CCB; ablation is definitive
N ● NPJT (non-paroxysmal JT): adults >
O children; “accelerated junctional AVRT
D rhythm”; a/w digoxin toxicity and acute ● Reentry thru acc. pathway + AV node
E MI; difficult to distinguish from AVNRT ● Narrow = orthodromic (antegrade thru
w/o EP study; treat underlying cause AV node, retrograde thru acc. path);
use nodal blockade
● Wide/pre-excitation = antidromic; avoid
nodal blockade (see WCT chapter)
● Definitive tx: ablation
119
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
120
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
● Permanent pacing is indicated for irreversible symptomatic bradycardia, chronotropic incompetence, or unstable
conduction disorders
● There are many different pacing modes, which are designated by a 1-5 letter code. In the chart below, “position”
refers to letter position
o As an example, in the 4-letter code DDDR, the D in position I implies that both chambers (the atria and
the ventricles) can be paced, the D in position II indicates that both chambers (the atria and the
ventricles) can be sensed, the D in position III indicates that the device can respond to sensing by
either triggering activity or inhibiting itself, and the R in position IV indicates that the device allows for
rate modulation
121
Lakotah Hardie and Kyle Saysana
Arrhythmias & Electrophysiology
TOC
122
Lakotah Hardie and Kyle Saysana
Valvular Heart Disease
TOC
Etiology: clinical risk factors for calcific AS mirror risk factors for CAD.
• AS is more common in men, advanced age, CKD (in part related to metastatic calcification), and vascular disease
• Rheumatic heart disease is responsible for the majority of cases in the developing world.
• Radiation-induced valve disease is increasingly recognized after mediastinal radiation therapy
• Aortic sclerosis, or calcification of the valve with no hemodynamic effects, is increasingly common with age, though
over 5 years, only 10-15% of these patients will develop AS.
Figure 1. Aortic Stenosis. CVPhysiology.com. Note increased LVEDP, LA pressures and decreased systemic pressures.
Presentation:
• Symptoms generally occur only in severe AS; LV remodeling often precedes symptom onsetAngina: results from
several mechanisms including increased total LV oxygen demand in setting of LV hypertrophy, decreased coronary
perfusion gradient due to elevated LVEDP, reduced diastolic perfusion time during tachycardia (later AS); ~ 50% of
angina in AS have CAD
• Syncope: exercise-induced decrease in total peripheral resistance is uncompensated because CO is restricted by
stenotic valve. 15% present with syncope.
• Heart failure: LV hypertrophy from chronic afterload results in diastolic dysfunction and/or systolic dysfunction
123
Kate Khan, Omar Khan
Valvular Heart Disease
TOC
o Systolic ejection murmur at the R upper sternal border that radiates to the neck; occasionally a different quality of
murmur which radiates to the apex (Gallavardin phenomenon).
o Decreases with maneuvers that decrease preload, such as standing and Valsalva. The severity of valvular
disease may not correlate with the intensity of the murmur.
o Indicators of severe AS include a late-peaking murmur, delayed carotid upstroke (pulsus parvus et tardus),
radiation to the left clavicle, and obliteration of the A2 component of S2.
• Diagnostics
o EKG may show LVH, LAE, LAFB or LBBB
o Transthoracic echocardiography (TTE) is used to evaluate valve anatomy and structure, valve gradients, and
LV structure and function. Multiple views can show reduced leaflet excursion with a small aortic orifice. Short axis
views “en-face” to the valve can demonstrate a bicuspid valve. Doppler echocardiography permits measurement
of jet velocity and calculation of the LV-Aortic gradient, which are the standard parameters used for evaluation of
severity (See Table 1).
o Cardiac catheterization may be indicated for hemodynamic assessment if clinical and echo data are
inconclusive or to evaluate for CAD prior to aortic valve replacement.
o Particularly for patients with suspected LFLG severe AS, need to optimized blood pressure control prior to
diagnostic studies to accurately assess AS severity
Grading AS Severity
Echocardiographic criteria (Table 1).
Table 1: 2020 ACC/AHA guidelines for grading of severity in aortic stenosis
Clinical progression is separated into stages A-D. Patients with bicuspid aortic valve or valvular sclerosis are termed
Stage A, or “at risk.” Mild-to-moderate AS defines Stage B, or “progressive AS.” Patients with Severe AS may be
categorized as Stage C (asymptomatic) or Stage D (symptomatic).
▪ Conversely, a normal valve may appear to have a low area if the ejection fraction and the flow across it are low
(so termed “pseudo-severe” AS).
▪ In “pseudo-severe” AS, the increased cardiac output may “stent” open the valve, and increase calculated
aortic valve area (to an AVA >1cm2), while the mean AV gradient does not change or may even be reduced.
AVR is NOT indicated in these patients
124
Kate Khan, Omar Khan
Valvular Heart Disease
TOC
Medical Management
Only AVR has been shown to improve symptoms and mortality in AS patients. Guidelines for medical management
involve treatment of HTN in patients at risk of developing AS (stage A) or with asymptomatic AS (stages B and C).
• Hypertension: Reduces the “double load” of afterload related to both the stenotic valve and systemic vasculature. No
optimal regimen. ACEi may reduce fibrosis; caution as diuretics can reduce preload and CO, vasodilators can reduce
coronary perfusion, and BB can reduce myocardial contractility. Up-titrate cautiously.
• Volume status: Volume status can be difficult to optimize in hospitalized patients with severe aortic stenosis.
Adequate preload is necessary to ensure sufficient cardiac output. Nitrates and diuretics should be used with caution
to avoid rapidly decreasing preload. High afterload can further potentiate high wall tension and diastolic dysfunction,
resulting in flash pulmonary edema. Initial management of hypovolemia in patients with severe AS may require small
fluid boluses (250cc) with careful monitoring of respiratory status. Patients with severe stenosis may easily “tip over”
with volume overload and can rapidly experience pulmonary edema.
• Statin therapy: has not been shown to reduce progression of AS and therefore is not indicated for prevention of
hemodynamic progression of AS.
Interventional Management:
▪ Bioprosthethic vs mechanical valve: depends on age (if <50, mechanical preferred for durability), contraindications
to anticoagulation (required lifelong for mechanical valve), and surgical risk (mechanical valve only done surgically)
▪
▪ TAVR versus SAVR: Recommendation of transcatheter AVR versus surgical AVR is made in multi-disciplinary
consultation between surgery and structural cardiology.
▪ Use of the Society of Thoracic Surgeons (STS score) risk calculator is helpful in determining surgical risk. An
STS score <4% qualifies as low risk, 4-8 % as intermediate risk, >8% as high risk
▪ Main risk factors that are not included in STS risk score are frailty, pulmonary hypertension, liver
disease, and porcelain aorta. Consider these in addition to STS.
TAVR was initially shown to be effective in patients with prohibitively high surgical risk (PARTNER trial), but has
since been shown to be non-inferior to surgical AVR in patients with:
▪ High surgical risk (PARTNER 1A, CoreValve trials), intermediate and low surgical risk (PARTNER-2,
PARTNER-3, SURTAVI, NOTION, STACCATO, Siontis GCM trials)
▪ TAVR has a slightly lower mortality risk and is associated with a shorter hospital length of stay, more rapid return
to normal activities, lower risk of transient or permanent AF, less bleeding, and less pain than SAVR. However, it
does have a higher risk of paravalvular leak, valve reintervention, and need for permanent pacemaker (PARTNER
3, NOTION)
125
Kate Khan, Omar Khan
Valvular Heart Disease
TOC
References:
1. Lester SJ et al. The natural history and progression of aortic stenosis. Chest. 1998;113(4):1109-14
2. Makkar RR et al. Transcatheter aortic-valve replacement for inoperable severe aortic stenosis. N Engl J Med.
2012;366(18):1696-704
3. Kodali SK et al. Two-year outcomes after transcatheter or surgical aortic-value replacement. N Engl J Med.
2012;366(18):1686-95
126
Kate Khan, Omar Khan
Valvular Heart Disease
TOC
4. Adams DH et al. Transcatheter aortic-valve replacement with a self-expanding prosthesis. N Engl J Med.
2014;370(19):1790-8
5. Popma JJ et al. Transcatheter aortic valve replacement using a self-expanding bioprosthesis in patients with
severe aortic stenosis at extreme risk of surgery. J Am Coll Cardiol. 2014;63(19):1972-81
6. Leon MB et al. Transcatheter or surgical aortic-valve replacement in intermediate-risk patients. N Engl J Med.
2016;374(17):1609-20
7. Hansson NC et al. Transcatheter aortic valve thrombosis: Incidence, predisposing factors, and clinical
implications. J Am Coll Cardiol. 2016;68(19):2059-2069
8. Tay EL et al. A high -risk period for cerebrovascular events exists after transcatheter aortic valve implantation.
JACC Cardiovasc Interv. 2011;4(12):1290-7
9. Makkar RR et al. Possible subclinical leaflet thrombosis in Bioprosthetic aortic Valves. N Engl J Med.
2015;373(21):2015-24.
10. Auffret V et al. Serial changes in cognitive function following transcatheter aortic valve replacement. J Am Coll
Cardiol. 2016;68(20):2129-2141
11. Spaziano M et al. Imaging and functional testing to assess clinical and subclinical neurological events after
transcatheter or surgical aortic valve replacement: a comprehensive review. J Am Coll Cardiol. 2014;64(18):1950-
63
127
Kate Khan, Omar Khan
Valvular Heart Disease
TOC
Acute and chronic AR have different etiologies, pathophysiological consequences, natural histories, and management
strategies. In general, AR occurs when the aortic valve does not close (or coapt) completely and blood flows back into
the LV from the aorta during diastole. As shown in Figure 1, this results in a rapid descent of aortic diastolic pressure
(AP tracing). Backflow from Aorta to LV → Increase in LVEDV → increased LV preload → increased SV and SBP.
Increased SBP and decreased DBP as described above result in characteristic wide pulse pressure.
Acute AR:
Etiology and Epidemiology
Results from abnormalities of the valve, such as infective endocarditis, or cusp prolapse, or acute abnormalities of the
aorta, primarily aortic dissection. Acute AR may also occur among patients with mechanical or bioprosthesis if there is
prosthetic leaflet dysfunction.
Pathophysiology:
In the uncompensated heart with acute AR, an increase in LVEDV (without acute LV enlargement) results in a large
increase in left-sided pressures, which leads to congestive heart failure and pulmonary edema. Marked and rapid volume
overload pushes the LV to the edge of its Frank-Starling curve. The LV cannot acutely increase total stroke volume,
resulting in a decline in forward stroke volume and cardiac output.
Acute AR may be an emergency; if a patient is symptomatic from acute AR, surgical consultation should be
pursued.
Chronic AR:
Etiology/Epidemiology:
The most common causes of chronic AR in the United States and other developed countries are bicuspid aortic valve,
thoracic aneurysms and calcific valve disease. Rheumatic heart disease is the leading cause in many developing
countries. AR also arises from primary diseases causing dilation of the ascending aorta or sinuses of Valsalva (e.g.
genetic syndromes such as Marfan’s Syndrome, systemic rheumatic disorders such as ankylosing spondylitis, and
infectious aortitis).
The 2018 AHA/ACC Adult Congenital Cardiology guidelines provide a Class IIa recommendation to screen first-degree
relatives of individuals with bicuspid aortic valve for valvular disease (present in 10% of relatives) and aortopathy (present
in 32% of relatives).1
Over time, however, the LV continues to dilate and the hypertrophy is unable to keep pace, which eventually leads to a
decline in both LV contractility and ejection fraction (EF). Interstitial fibrosis increases, compliance declines, and LVEDP
rises, and symptoms of congestion ensue. Bradycardia is poorly tolerated because slow HRs increase diastolic filling (and
regurgitant volume) time, which reduces effective forward stroke volume.
Radiographic evaluation:
▪ TTE is indicated in patients with signs or symptoms of AR for accurate diagnosis of the cause of regurgitation;
assessment of regurgitant severity, LV size and LV systolic function; and for determining timing of valve
intervention. See Figure 2 and Table 1 below for echocardiographic/clinical grading criteria.
▪ If TTE images are sub-optimal in moderate/severe AR, or clinical and echocardiographic findings are discrepant,
can consider TEE or cardiac MRI (less useful in acute setting), to distinguish between moderate and severe MR
Note: In cases of acute AR when aortic dissection is suspected, the sensitivity and specificity of TTE for
dissection is only around 60 to 80%. In these instances, TEE or CTA should be employed, as the
sensitivities and specificities for diagnosing aortic dissection exceed 95% for these modalities.
▪ AR is quantified by measuring the jet width in the LVOT and vena contracta (the narrowest width of the
regurgitant jet before the LVOT) on Doppler as well as the regurgitant orifice size and regurgitant volume. With
severe AR, Doppler shows steep deceleration in the jet velocity due to elevated LV end-diastolic pressure and
prolonged diastolic flow reversal in the descending aorta.
▪ LV systolic and diastolic diameters, EF, and aortic width on TTE are key parameters that influence decision for
surgical intervention. Depressed ejection fraction and increased LVESV are associated with development of
HF symptoms or death in asymptomatic patients.
Hemodynamics
*Note: Stage A refers to “at-risk” patients, such as patients with bicuspid aortic valve, diseases of the aortic sinus or
ascending aorta, history of rheumatic fever, or infective endocarditis.
Surveillance:
Repeat TTE should be performed to re-evaluate asymptomatic patients with AR, with recommended frequency
determined by the severity of regurgitation (Severe: every 6-12 months, Moderate: every 1-2 years, Mild: every 3-
5 years).
Management
Medical Management
Acute or decompensated AR: The management of acute severe AR (most often resulting from infectious endocarditis or
aortic dissection) consists of hemodynamic temporization and urgent surgical evaluation. Propensity-matched cohort
studies among patients with infective endocarditis have demonstrated a 6% absolute risk reduction with early versus
delayed surgery.2
Medical therapy:
• IV afterload reduction with agents like nitroprusside. Avoid vasoconstrictors which may increase diastolic
regurgitant flow
• Inotropic support with dobutamine/milrinone as needed
• Chronotropic support with overdrive pacing or isoproterenol to reduce diastolic regurgitation time as needed.
• Beta blockers are generally avoided in severe AR (blocks compensatory tachycardia, increasing diastolic
regurgitation time), except in the case of acute aortic dissection where it is first line for impulse control therapy
• Diuretics as needed to reduce LV filling pressures
• Consider PA line placement for guidance of above therapies
Valvular intervention
- Proceed to AVR if patient is symptomatic, LVEF <50%, LV end-systolic dimension >50 mm, and/or if concomitant
CT surgical procedure is being planned (i.e. CABG or other valve). See Figure 3.
- Outcomes are optimal when surgery is performed before LVEF decreases below 55%.
- In asymptomatic patients with LV systolic dysfunction, postoperative outcomes are better if AVR is
performed before onset of symptoms.
Additional Notes:
o Once AR has been diagnosed, follow up evaluations should focus on the identification of symptoms.
Symptomatic AR is an indication for surgical intervention, and intervening as early as possible—before LV
systolic failure ensues or worsens—is paramount. Without AVR, these patients have a very high mortality rate
(25% for NYHA class III/IV, 6% for NYHA Class II), and post-operative survival is significantly higher in patients
who are still NYHA I/II at time of surgery compared with NYHA class III/IV (10 year survival of 80% vs 45%).4
o Even in symptomatic patients with severely depressed systolic function, surgery is recommended over medical
therapy. The sooner AVR is performed after depressed EF is noted, the more systolic recovery occurs.
References:
1. Stout KK et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J am Coll
Cardiol. 2019;73(12):1494-1563
2. Lalani T et al. Analysis of the impact of early surgery on in-hospital mortality of native valve endocarditis: use of
propensity score and instrumental variable methods to adjust for treatment-selection bias. Circulation.
2010;121(8):1005-13.
3. 2014 AHA/ACC guideline for the management of patients with valvular heart disease. J Am Coll Cardiol. 2014 Jun
10;63(22):2438-88
4. Klodas E et al. Optimizing timing of surgical correction in patients with severe aortic regurgitation: role of
symptoms. J Am Coll Cardiol. 1997;30(3):746-52
Acute.
Etiologies: Acute ischemia (most often inferior STEMI) with papillary muscle rupture/dysfunction, infective endocarditis,
spontaneous chordal rupture in myxomatous mitral valve disease
Chronic.
(1) Primary (degenerative). Valvular incompetence via pathology of a component of the valve itself (leaflets/chordae
tendinae/papillary muscles/annulus).
o Most common cause in United States is MVP and ischemic heart disease. Additional etiologies:
infective endocarditis, rheumatic heart disease, collagen vascular disease, annular calcification
o Young patients: myxomatous degeneration
o Elderly patients: fibroelastic degeneration of valve
(2) Secondary (functional). Valve is normal but becomes displaced via papillary muscle displacement (usually from dilated
ventricle and severe LV dysfunction). Annulus dilates, leaflet becomes tethered, and valve leaflets cannot coapt.
In those who are symptomatic, exertional dyspnea, fatigue, and atrial fibrillation are the most common manifestations.
Severe MR results in poor clinical outcomes (survival rates 33% at 8 years without MVR, mortality 5% per year). Most
deaths are due to heart failure. There is a substantial rate of sudden death, highlighting the importance of ventricular
arrhythmias.
Evaluation: 1. Distinguish between acute and chronic. 2. Identify etiology. 3. TTE to confirm and grade severity.
Acute.
History: New onset dyspnea, PND, orthopnea, edema. Exam: New apical holosystolic murmur radiating to left axilla.
Rales, edema, JVD, cardiogenic shock.
Chronic.
History: Progressive exertional dyspnea, exercise intolerance.
Exam: Apical holosystolic murmur radiating to left axilla (half patients with chronic secondary MR lack audible
murmur, especially if low systolic LA-LV pressure gradient i.e. ↑LA pressure, ↓systemic arterial pressure) decreased with
maneuvers which decrease venous return (Valsalva). Specific findings per etiology: mid-systolic click (MVP), S3 gallop,
lateral PMI (LV dilatation), rales (pulmonary edema). AF highly associated with chronic MR with LA dilatation.
• Other possible findings: Early diastolic rumble, left sided S4, soft S1 and widely split S2. Sharp and high volume
carotid upstroke when severe. Cardiac impulse brisk, hyperdynamic, laterally displaced.
TTE is most common modality for diagnosis of MR. Echo criteria are used to grade MR (Tables below). ACC/AHA
guidelines support the use of TTE for initial evaluation (EF, LV and LA sizes, and PA pressure). Consider TEE as well
before surgical intervention if TTE not sufficient for mechanism of MR and feasibility of repair (although sedation during
TEE can reduce degree of MR via vasodilation and afterload reduction).
The criteria for grading MR are shown in Table 1-2. The clinical progression of the disease is separated into stages A-D.
Stage A is mild MR. Stage B is moderate MR. Stage C-D are severe MR. For severe, chronic MR, only one
echocardiographic criteria must be met.
- Mild valve
thickening and - Rheumatic valve changes - Rheumatic valve changes with leaflet
leaflet restriction with leaflet restriction and restriction and loss of central coaptation
loss of central coaptation
- Prior IE - Prior IE
Angiographic 1+ to 2+ 3+ to 4+
grade
Stage A B C D
Definition At risk of MR Progressive MR Asymptomatic Symptomatic
severe MR Severe MR
Valve Anatomy Normal valve leaflets, - Regional wall - Regional wall motion abnormalities and/or LV
chords, and annulus in a motion abnormalities dilation with severe tethering of mitral leaflet
patient with CAD or with mild tethering of Annular dilation with severe loss of central
cardiomyopathy mitral leaflet coaptation of the mitral leaflets
Annular dilation with
mild loss of central
coaptation of the
mitral leaflets
Valve
Hemodynamics
Doppler MR jet - No or small central jet
area area <20% LA
Associated Normal or mildly dilated LV Regional wall motion Regional wall motion abnormalities with
Cardiac Findings size with fixed (infarction) or abnormalities with reduced LV systolic function
inducible (ischemia) reduced LV systolic LV dilation and systolic dysfunction
regional wall motion function attributable to primary myocardial disease
abnormalities LV dilation and
Primary myocardial disease systolic dysfunction
with LV dilation and systolic attributable to
dysfunction primary myocardial
disease
Symptoms Symptoms attributable to coronary ischemia or HF may be HF symptoms attributable to MR
present that respond to revascularization and appropriate persist even after revascularization
medical therapy and optimization of medical
therapy
Decreased exercise tolerance
Exertional dyspnea
Not all criteria for each valve hemodynamic criteria need to be present in each patient. Parameters should be integrated with other
clinical evidence. ERO, effective regurgitant orifice; IE, infective endocarditis; LA, left atrium; LV, left ventricular; LVESD, left
ventricular end-systolic dimension; MVP, mitral valve prolapse.
Management
Acute MR:
Surgical repair > transcatheter repair. Consider early revascularization if ischemia.
Additional notes: This usually requires urgent and early surgical repair. Worse prognosis than chronic MR. Early
revascularization (if ischemic) may arrest local maladaptive LV modeling, restore valve competence, and obviate
the need for surgical repair. Surgical repair generally preferred over transcatheter repair.
Medical management to stabilize for surgery. Reduce afterload, maintain CO and diminish pulmonary congestion with IV
nitroprusside, dobutamine, or intra-aortic balloon pump.
Additional notes: If surgery will proceed, the next determination is mitral valve repair vs replacement followed by the
decision of mechanical vs bioprosthetic valve. Among patients with severe symptomatic MR, mortality is as high as 8%
per year, and the mainstay of therapy is surgery.
Mitral valve repair is preferred over replacement if valve morphology is amenable, as valve repair is associated with
superior post-operative survival and ventricular function. Involvement of the anterior leaflet (or both leaflets) may preclude
repair and require replacement. Repair further eliminates the risks and pitfalls associated with prosthetic valves, including
prosthesis degeneration. Around half of patients with bioprosthetic MVR will need another replacement within 15 years.
For those who are symptomatic despite GDMT and have prohibitive surgical risk, transcatheter mitral valve
repair with MitraClip can be considered
Medical management:
• Treat patients with symptomatic chronic primary MR stage D and LVEF<60% with GDMT as bridge to surgery or
if prohibitive surgical risk.
• Treat concomitant hypertension per standard therapy.
• Patients with asymptomatic chronic primary MR stages B and C with normal LV systolic function do not receive
medical treatment.
Chronic secondary MR: In contrast to primary MR, the utility of surgical intervention in chronic secondary MR is generally
low due to the pathophysiology of this disease. If concomitant HFrEF, treat with GDMT.
There are no Class I indications for MV surgery for chronic secondary MR. Class II recommendations:
▪ MV surgery is reasonable: undergoing CABG concurrently for myocardial ischemia
▪ MV surgery can be considered: preserved LV systolic function (LVEF >50) with severe persistent symptoms
(NYHA class III, IV) despite therapy for HF and comorbidities.
▪ MV surgery can be considered: reduced LV systolic function (LVEF <50) with severe persistent symptoms (NYHA
class III, IV) on optimal GDMT
▪ Cardiac resynchronization therapy (CRT) can also be helpful in reducing the severity of functional MR if otherwise
clinically indicated. 1
▪ If surgery will proceed, mitral valve replacement is preferred over repair.
There are no Class I indications for TEER for chronic secondary MR. Class II recommendations:
• In patients with chronic severe secondary MR related to LV dysfunction LVEF<50% with NYHA class II, III, or IV
symptoms while on optimal GDMT, TEER is reasonable with appropriate anatomy on TEE and LVEF 20-50%,
LVESD <70, PASP <70mmHg.
Monitoring:
Repeat TTE if there are changes in symptoms.
In the absence of clinical change, repeat TTE every 6–12 months for patients with severe MR for evaluation of
EF (more frequently if evidence of LV dilatation), every 1-2 years for moderate MR, every 3-5 years for mild MR.
Guidelines for anticoagulation after MVR (institutional preferences may vary, check with fellow/attending). Note that if
reversal of anticoagulation is urgently needed, FFP can be used (Class II) and is preferred over Vitamin K since it is
thought to reduce the incidence of thrombotic complications (though no good data).
o Bioprosthetic MVR: 81 mg ASA, 3 month of Coumadin for INR 2–3 after surgery (lifetime if rheumatic MR or if
EF<30–35, hypercoag, hx clot, AFib)
o Mechanical MVR: 81 mg ASA, INR 2.5–3.5 for life with IV heparin when INR <2.5
References:
1. van Bommel RJ et al. Cardiac resynchronization therapy as a therapeutic option in patients with moderate-severe
functional mitral regurgitation and high operative risk. Circulation. 2011;124(8):912-9.
2. 2020 AHA/ACC guideline for the management of patients with valvular heart disease. J Am Coll Cardiol. 2014 Jun
10;63(22):2438
Etiologies: rheumatic fever, then senile calcific MS. Other rare cause is congenital (parachute, supramitral ring, part of
Shone’s complex)
• Rheumatic → progressive, thickening of leaflet edges, fusion of commissures, chordal shortening and fusion.
Latent phase between initial illness and valve stenosis.
• Senile calcific → benign calcification of annulus extends into leaflets → narrowing of annulus and rigidity of
leaflets without commissural fusion. Can also be caused by radiation induced valve disease, often in survivors of
Hodgkin’s lymphoma.
• ↓LA flow to LV → ↑LA pressure, ↑pressure in pulmonary vasculature, ↑right sided pressures.
• Nonspecific presentation: exertional dyspnea, poorly tolerated AF from loss of atrial kick or rapid heart rate,
unexplained cardio embolism, hemoptysis from increased pulmonary congestion, hoarseness from LA
compression of recurrent laryngeal nerve (Ortner’s Syndrome). Clinical history of childhood rheumatic fever or
characteristic cardiac exam.
• Generally diagnosed echocardiographically
• If untreated: progressive pulmonary edema → pulmonary hypertension and right heart failure (60% of cases)
→death. Mortality can also result from systemic thromboembolic events in the context of atrial fibrillation.
Evaluation
TTE is indicated to 1. Diagnose MS. 2. Determine etiology. 3. Grade severity. 4. Typify valve morphology to determine
suitability for percutaneous mitral balloon commissurotomy (PMBC)
Exam:
Loud S1 leading to softer S1 as leaflets become more calcified, opening snap heard best at base, low pitched diastolic
rumble (most prominent at apex in left lateral decubitus during held expiration using stethoscope bell),
The criteria for grading MS are shown in Table 1. The clinical progression of the disease is separated into stages A-D.
Table 1. 2020 ACC/AHA Guidelines for Staging MS
Stage A B C D
Definition At risk of MS `Progressive MS Asymptomatic Symptomatic
severe MS Severe MS
Valve Anatomy Rheumatic valve Rheumatic valve changes with
changes with commissural fusion and diastolic doming
Visual appearance Mild valve commissural fusion of mitral valve leaflets
doming and diastolic
during doming of mitral
diastole valve leaflets
Mitral Valve Area
(planimetered) >1.5cm2 ≤1.5cm2
Valve Hemodynamics
Normal Increased
transmitral transmitral flow
flow velocity velocities
Mitral Valve Area (by
continuity equation) >1.5cm2 ≤1.5cm2
Hemodynamic
Consequences
Pulmonary artery
systolic pressure Normal >50 mmHg
Symptoms None Decreased exercise tolerance
Exertional dyspnea
The transmitral mean pressure gradient should be obtained to further determine hemodynamic effect of MS and is
usually >5-10mm Hg in severe MS; However, due to the variability of the gradient with HR and output, it is not
included in criteria for severity. LA left atrial; MS, mitral stenosis; PASP, pulmonary artery systolic pressure
Repeat TTE should be performed to re-evaluate asymptomatic patients with MS. For Very Severe: every year, Severe:
every 1-2 years, Progressive MS: every 3-5 years. The mean rate of progressive valve narrowing is ~0.1cm2 per year
with appreciable variability.
Management
Anticoagulate in rheumatic MS with AF and prior embolic events. It has been shown to reduce incidence of arterial
embolization. There is evidence for VKA, but not for NOACs (patients with MS were excluded from AF trials).
If rheumatic MS and AF with RVR, or with symptomatic resting/exertional sinus tachycardia, use a beta blocker.
Additional notes: Negative dromotropes (e.g. beta blockers) can be beneficial in patients with rheumatic MS and
either AF with RVR or with symptomatic resting or exertional sinus tachycardia. Studies of beta blockers and
ivabradine demonstrated only benefit in younger patients without chronotropic incompetence; thus although there
is theoretical benefit in reducing HR and prolonging diastolic filling (thereby preventing a rise in mean mitral
gradient and increased LA pressures), this is balanced by limitation of stroke volume and chronotropic
incompetence. Rheumatic disease itself can lead to fibrosis of conduction tracts and predispose to hard to control
AF. Beta blockade is used in pregnant pts with MS to blunt the physiologic tachycardia of pregnancy and prolong
diastole, prevent pulmonary edema.
Diuretics are a mainstay of medical management to keep LA pressures down and manage pulmonary edema prior to
intervention.
PMBC or open/closed commissurotomy are mainstay of therapy over medical management. Mitral valve replacement is
an option only if no other options with severe limiting symptoms.
Additional notes: PMBC involves puncturing the septum during cardiac catheterization to access the stenotic
mitral valve via RA to LA. A balloon is then inflated to force open the valve a separate the fused commissures;
this is also what occurs during surgical commissurotomy. Other approaches with expanding roles in MS include
transcatheter mitral valve replacement (TMVR) and mitral valve bypass. IN TMVR, for patients who cannot
receive PMBC or surgery, an expandable transcatheter valve is inserted into the MV leaving the native MV tissue
in place. In MV bypass a conduit is sewed from the LA to the LV apex and a prosthetic valve is incorporated into
the conduit, avoiding direct manipulation of the mitral annulus.
Recommendations:
• PMBC is recommended: patients with NYHA class II, III, IV, and severe rheumatic MS and favorable valve
morphology with less than 2+ MR in the absence of LA thrombus, if at a Comprehensive Valve Center.
• Mitral valve surgery is indicated: patients with NYHA III or IV with severe rheumatic MS who are not candidates
for PMBC, have failed a previous PMBC, require other cardiac procedures, or do not have access to PMBC.
• PMBC is reasonable: asymptomatic patients with severe rheumatic MS and favorable valve morphology with less
than 2+MR in the absence of LA thrombus with elevated PASP>50mmHg, if at a comprehensive Valve Center
• PMBC may be considered: asymptomatic patients with severe rheumatic MS and favorable valve morphology
with less than 2+MR in the absence of LA thrombus who have new onset of AF, if at a Comprehensive Valve
Center.
• PMBC may be considered: NYHA II, III, IV with rheumatic MS, mitral valve area >1.5, if evidence of
hemodynamically significant rheumatic MS with PAWP >25 or mean mitral valve gradient >15 during exercise, if
at a Comprehensive Valve Center
• PMBC may be considered: NYHA III, IV with severe rheumatic MS with suboptimal anatomy and are not
candidates for surgery, if at a Comprehensive Valve Center.
Calcific MS (or mitral annular calcification) is increasingly frequent in USA, but is usually high risk for any intervention
given extensive calcification, advanced age, and comorbidities. Intervention only in highly symptomatic (NYHA III or IV)
with severe MS (mitral valve area <1.5) attributable to extensive mitral annular calcification, with discussion of procedural
risk and patient preferences and values. PMBC is not often performed here; feasibility studies show that TMVR may have
a role. Nonrheumatic MS can also occur after radiation therapy or after a mitral valve repair with a small annuloplasty ring.
Evaluation
TTE is indicated to 1. establish diagnosis. 2. characterize severity. 3. measure velocity of TR for estimation of systolic RV
to RA gradient 4. identify mechanism responsible for TR.
• TTE can show:
o RV or RA dilation and RV function, tricuspid annulus dilation, paradoxical interventricular septal
movement
o Degree of pulmonary hypertension via estimation of RV to RA systolic gradient from TR jet peak velocity
using modified Bernoulli equation
o Doppler color flow using vena contracta method
Exam:
Clinical findings can be absent in TR and when present are often related to the underlying cause of TR, or its sequela,
right-sided heart failure. In severe TR there may be a sensation of pulsation in the neck.
• Right-sided HF: painful hepatosplenomegaly, ascites, peripheral edema. When severe, cachexia, cyanosis,
sometimes jaundice.
• Jugular veins: distended and prominent veins, distinct “c-v” wave from systolic regurgitation, pulsatile jugular vein
(which can be confused with carotid arterial pulse), Kussmaul’s sign (JVD more prominent with inspiration),
systolic thrill felt over jugular vein when severe.
• Other findings: dynamic RV heave, dullness and prominent pulsation over left second intercostal space,
peripheral edema, pleural effusions (if secondary to left-sided problem), hepatomegaly with possible thrill
(transmission of systolic murmur)
• Auscultation: Holosystolic murmur best heard at left mid sternal border or subxiphoid area. No radiation and
generally no palpable thrill. Murmur is soft/absent when TR is severe. Increases with inspiration/leg
raise/exercise/hepatic compression. Standing decreases intensity of murmur. S3 often heard if RV very dilated;
S4 possible if significant RV hypertrophy.
Stage B C D
Definition Progressive TR Asymptomatic Symptomatic
severe TR Severe TR
Valve Hemodynamics
<0.40cm2 ≥0.40cm2
ERO
Dense continuous wave signal with triangular
shape
Hepatic vein systolic flow reversal
Hemodynamic Consequences
Management
Treatment of primary TR with medical management alone are poor. Management of secondary TR is of underlying
pulmonary hypertension or myocardial disease. Surgery can be considered in a subset of patients with isolated primary or
secondary TR (without pulmonary hypertension or dilated cardiomyopathy).
VASCULAR MEDICINE
Venous Thromboembolism
Deep Vein Thrombosis
A. Definition/ Classification
Clot formation within the deep venous system. Typically classified as:
● Proximal/ “above the knee” (external iliac, popliteal, femoral veins including superficial femoral)
● Distal/ “below the knee” (ant. tibial, post. tibial, peroneal, gastrocnemius and soleus vein)
● Upper extremity (includes the internal jugular vein)
B. Clinical Presentation
Patients may be asymptomatic or complain of unilateral leg pain, swelling, erythema, or warmth.
1. Always consider DVT in signs, symptoms and diagnosis of PE.
Risk Factors
Anatomic risk factors
Iliac vein compression (aka “May-Thurner
syndrome”): compression of the left iliac vein
between the right iliac artery and lumbar spine within
the deep pelvis. Compression on the right is also
possible.
C. Diagnostics
● Diagnosis of an acute DVT in the lower extremity begins with an assessment of the pre-test probability for that patient
and incorporates US and D-dimer based upon this pre-test probability assessment 1.
o Consider Well’s Criteria for DVT to risk stratify likelihood of DVT.
● Venous doppler US is the most practical test to make the diagnosis of DVT.
D. Management
● Therapeutic anticoagulation is mainstay of treatment in absence of contraindications (i.e. increased bleeding risk)
● Always indicated in patients with lower extremity proximal DVT, Upper extremity DVT, and in some cases of distal
DVT
o Proximal lower extremity DVTs are associated with a high risk of PE and mortality
1. Medical therapy:
● Direct oral anticoagulants (DOACs) (apixaban, rivaroxaban, edoxaban and dabigatran) are considered first-line
agents for VTE in the majority of patients.
● Warfarin favored in patients with APLAS (TRAPS)2.
● LMWH was considered the only preferred method of AC in active malignancy until recently where DOACs have
shown to have similar safety and effectiveness in patients with active malignancy who do not have gastric cancer or
gastroesophageal lesions (ASH 2021 Guideline)3
146
Krishan Sharma
Vascular Medicine
TOC
147
Krishan Sharma
Vascular Medicine
TOC
● Duration:
o First episode of provoked VTE: Minimum of three months of therapeutic anticoagulation4.
o If persistent risk factors (e.g. immobility, obesity, HRT) and low risk of bleeding: Minimum 6-12 months.
o If unprovoked VTE, recurrent provoked VTE, active malignancy: Indefinite AC may be considered.
o Careful consideration of bleeding risk should always be considered when planning duration of therapy.
.
Pulmonary Embolism
A. Definitions
● Acute: acute obstruction within the pulmonary arteries by material (e.g. thrombus, tumor, air, fat) from elsewhere in
the body
● Chronic thromboembolic pulmonary hypertension (CTEPH): symptomatic post-embolic pulmonary obstruction
leading to pulmonary hypertension.
B. Clinical Presentation
● Presenting symptoms: dyspnea, tachypnea, chest pain, pleuritic pain, palpitations, pre-syncope, cough, hemoptysis,
low grade fever, syncope, and cardiac arrest.
● Signs: tachycardia (sinus most common, also new RV strain patter, RAD), hypoxemia, hypotension; e/o LE DVT
Image from StatPearls Jan 2021
C. Diagnostics
● CT chest angiogram is the gold standard test to diagnose PE.
● V/Q scan is an alternative for those with contraindications.
● Consider Transthoracic echo (TTE) and lower extremity ultrasound (LENIs) for risk stratification.
Pre-test probability:
There are several externally validated scoring systems to assess for the pretest probability of pulmonary embolism. 9 They
include the original Wells score, the Geneva score and the pulmonary embolism rule-out criteria (PERC) score10.
1. Wells Criteria for PE (10,11,12) (100% Sn, 85% Sp) Highly sensitive when used to exclude the diagnosis of PE in patients
with low clinical probability of PE (score ≤2). Critiques of the Wells score point to its subjective criteria that cannot
otherwise be standardized across users.
2. Geneva score(10,13) Developed as an objective measurement of pre-test probability but has not been demonstrated to be
superior to the Wells score.
148
Krishan Sharma
Vascular Medicine
TOC
3. PERC score(10,14) (97% SN, 22% Sp) To be used only in instances of low pre-test probability by either the Wells score
or Geneva score.
Labs:
● D-dimer 15: D-dimer has a high sensitivity (80%-100%) and low specificity (23%-63%). Obtain when the pre-test
probability for PE is low (i.e., a rule out test).
● Can be elevated in many different diseases associated with fibrinolysis (cancer, inflammation/infection, DIC,
arterial thrombus, aortic dissection, CHF, ESLD, and ESRD). Use Age-adjusted D-dimer16 for patients >50 years
of age (age x 10ng/mL = cut off value in ng/mL). Normal levels cut off to rule out PE is <500 ng/mL.
Imaging:
● CTPE17 (98% sp, 86% sn) Most definitive diagnostic test. Contraindicated (relative) in pregnancy, renal insufficiency or
contrast allergy.
● V/Q scan17 (93% sp, 83% sn) Alternative image modality in those with contraindications to CTPE
● LENI17 (86% sp, 44% sn): Obtained in conjunction with CTPE or V/Q scan or in some instances to aid in treatment
decisions when chest imaging cannot be obtained promptly (e.g. pregnancy).
● TTE: Obtained with CTPE or V/Q scan. Utilized in risk stratification.
Risk Stratification
Risk stratification of PE depends on the presence or absence of hemodynamic instability, increased respiratory effort
and right heart strain. Overall patient risk taken into context as well as alluded to in the PESI score
● RV strain can be assessed via multiple modalities
o Cardiac biomarkers (NT-proBNP, hsTnT),
o EKG (RBBB, R axis deviation, anterior T wave inversions, S1Q3T3),
o CT-PE (RV/LV ratio >0.9), or
o TTE (RV dilation, hypokinesis, or McConnell’s sign [diffuse RV wall hypokinesis with apical sparing]).
Intermediate-high:
● +Troponin and RV
dysfunction on Echo or CT
imaging
● NB: the presence/ absence of hemodynamic instability does not always correlate with the anatomic location or the
degree of clot burden.
● The Pulmonary Embolism Severity Index (PESI)14 helps to risk stratify patients who can safely be treated outpatient
once a diagnosis is made.
Management
Low risk: Initiate therapeutic oral AC provided there is no contraindications and risk of bleeding is low.
● If no other reason for hospitalization and adequate home support can discharge home, direct from ED
Intermediate risk: Initiate AC; monitor closely for clinical signs of deterioration. PERT c/s recommended.
● Generally recommended that most patients without hypotension are not treated with thrombolytic therapy. 4
● Thrombolysis or catheter-based therapies can be considered weighing the risks and benefits of the procedure.
● If persistent dyspnea, consult PERT
High-risk:
Consult PERT ASAP.
● Consider systemic thrombolysis 🡪 decreased all-cause mortality but increased risk of major bleeding and intracranial
hemorrhage4
● Catheter-directed thrombolysis
149
Krishan Sharma
Vascular Medicine
TOC
● For those with high bleeding risk, invasive stabilizing measures (e.g., ECMO) and surgical thrombectomy.
PERT: The Pulmonary Embolism Response Team (x47378) at MGH is a rapid response, multidisciplinary team that can
be activated for prompt assessment and management of patients with intermediate to high-risk PEs.
Catheter-associated thrombosis
Clinical considerations
● Incidence as high as 14-18%.
● Duplex ultrasound is the diagnostic imaging modality of choice.
● Current guidelines recommended that systemic anticoagulation be administered for at least three months after the
catheter has been removed.
● If central access is required, the CVC can remain in place as long as it is appropriately positioned and functioning
correctly without evidence of infection.
● If the catheter remains for > 3 months on therapeutic AC, LMWH prophylaxis is recommended until the line is
removed.4
150
Krishan Sharma
Vascular Medicine
TOC
B. Risk Factors
Age ≥70 yo, Male, black, Smoking, DM, H/o Atherosclerosis, HTN, HLD, Elevated homocysteine
C. Clinical presentation
Classic claudication: exertional buttock, hip, thigh, calf, or foot pain that is relieved with rest.
D. Diagnosis
Screening
The USPSTF has stated there is insufficient evidence to recommend routine screening for PAD with an ABI. 24 However,
the detection of PAD may identify patients at risk for atherosclerosis at other sites and can result in early interventions to
lower overall CVD risk.
Diagnostics
The diagnosis of PAD can be suspected clinically based on history of symptoms, risk factors for PAD, and physical exam
findings. Ankle-brachial index (ABI) should be used to confirm the diagnosis.
151
Lakotah Hardie
Vascular Medicine
TOC
E. Management
Management of PAD: Management depends on presentation (asymptomatic, intermittent claudication or critical limb
ischemia). Revascularization is an option for patients with significant or disabling symptoms unresponsive to other
treatment options.
Revascularization is recommended for patients with limb-threatening ischemia such as ischemic rest pain or ulceration
and patients with significant or disabling symptoms unresponsive to above.29 Options include percutaneous intervention or
open surgical intervention depending on the location and extent of disease. Most patients are offered endovascular
intervention.
152
Lakotah Hardie
Vascular Medicine
TOC
Options for revascularization include endovascular therapy or bypass surgery. There has been persistent, widespread
clinical uncertainty about the best means by which to revascularize patients with chronic limb threatening ischemia. In
modern practice most patients receive endovascular therapy first. The BEST-CLI trial is currently enrolling patients and is
designed to compare best endovascular therapy with best open surgical treatment in patients eligible for both treatments.
Amputation may be considered in patients with sepsis, limb paralysis, uncorrectable flexion contracture, or significant
necrosis of weight-bearing parts of the foot.
153
Lakotah Hardie
Vascular Medicine
TOC
B. Risk Factors and Etiology: RAS likely accounts for 1-5% of patients with hypertension. Prevalence is higher in patients
with acute, severe, or refractory HTN.31 Risk factors include age, HLD, HTN, and elderly patients with comorbidities
such as diabetes, PAD, or CAD.32
1. Atherosclerotic RAS (ARAS) accounts for 90% of all RAS.33 Patients with ARAS are likely to have atherosclerotic
disease in other areas of the body including CAD and PAD.
2. Fibromuscular Dysplasia (FMD) is a non-inflammatory, non-atherosclerotic vascular condition that commonly
affects the renal arteries. 34 According to the FMD registry, the majority of patients affected with FMD are female
(90%) and the mean age at diagnosis was 52 years.35 In this registry FMD was identified in the renal artery in
66% of patients. Similar data exist in the international / European registry.
3. Other non-atherosclerotic forms of RAS include aneurysms, congenital/ traumatic AV fistulas, vasculitis,
neurofibromatosis, trauma, embolization, post-radiation therapy, dissection, and extrinsic compression of the
renal arteries due to tumors. 36
C. Clinical Presentation: Patients present with severe or resistant hypertension. Physical exam findings of include a
unilateral systolic-diastolic abdominal/ flank bruit (Sn 40% Sp 99). Labs may show HypoK and metabolic acidosis.
Suspect in non-obese patients <30 y.o. with no family history of HTN. RAS is associated with an unexplained rise in
serum Cr > 50% within 1 week of ACE/ ARB initiation, however unilateral RAS is not a contraindication to these
medications.
D. Diagnostics Testing for renovascular disease is indicated for patients who present with clinical findings consistent with
secondary hypertension with moderate-high pre-test probability.
E. Management
● Medical therapy remains the mainstay of treatment for RAS. Treatment focuses on lowering cardiovascular risk
through smoking cessation, glucose control, cholesterol reduction, ASA, and blood pressure control. Choice of anti-
HTN medication should follow published guidelines as if for non-RAS patients.
o Importantly, ACE and ARB are not contraindicated in RAS. ~50% of patients with RAS will usually have a
mild increase in creatinine after the administration of an ACE/ARB. ACE/ARB may be of benefit given
elevated RAAS activity in RAS.
● Revascularization: Options for revascularization include percutaneous angioplasty +/- stenting and surgical
revascularization. Several RCT’s, including, the CORAL trial demonstrated that renal artery stenting plus medical
therapy did not confer a significant benefit with respect to the prevention of clinical events. 37 However, some patients
do benefit from revascularization and it is recommended for those who have a high likelihood of benefit. 38 That
includes patients with hemodynamically significant atherosclerotic RAS AND at least one of the following 1) recurrent
“flash” decompensated HF; 2) refractory ACS; 3) typically new-onset resistant HTN; or 4) acutely progressive CKD
stage IV. Revascularization strategy may vary depending on etiology of RAS (i.e., atherosclerotic RAS vs FMD).
154
Lakotah Hardie
Vascular Medicine
TOC
Aortic Aneurysm
A. Definition
Thoracic Aortic Aneurysm (TAA): is a full-thickness dilation of a segment of the
thoracic aorta that is at least 50% larger than the expected diameter of that
segment. The normal aortic diameter varies according to age, sex, and body size. 39
Abdominal Aortic Aneurysm (AAA): A full-thickness dilation of a segment of the
abdominal aorta typically ≥3 cm. Classified by location (suprarenal, pararenal, or
infrarenal) and morphology (fusiform vs saccular). 40,41
B. Risk Factors
Thoracic Aortic Aneurysm (TAA):
Sporadic (Degenerative) Genetically mediated
Hypertension (Most common. Marfan syndrome
occurring in 60% of pts w/ Ehlers-Danlos syndrome CT image of ascending thoracic aortic
TAA2) Loeys-Dietz syndrome aneurysm. Adapted from JACC
2010;55:e27.
Male Turner syndrome
Age Familial TAA & dissection
Smoking history Bicuspid aortic valve disease
Hypercholesterolemia
Prior aortic dissection
Aortitis (vasculitis/ infection)
Risk of rupture:
TAA: The risk of rupture or dissection increases abruptly as thoracic aneurysms approach 6 cm because at this size,
distensibility of the aorta rapidly falls. The annual risk of rupture is 2% for TAAs <5 cm and 7% for TAAs >6 cm. 43
AAA: Risk of rupture increases with aneurysm size with <1% annual rupture risk for AAA <4 cm in diameter and >10%
annual rupture risk for AAA >6 cm in diameter. 42 Rupture is often lethal with mortality cited as 85-90%44. Female sex
appears to be an independent risk factor for rupture in AAA. Women make up ~20% of AAAs compared to men but
constitute approximately ~30% of all AAA ruptures.45
C. Clinical Presentation
TAA and AAA are typically clinically silent, however, patients who are symptomatic can present with symptoms due to
compression of surrounding structures. 46 Patients with
● Ascending aorta/aortic arch
o Heart failure due to aortic regurgitation
o Myocardial ischemia or infarction due to compression of a coronary artery
o Dysphagia due to esophageal compression
o Hoarseness due to left recurrent laryngeal nerve compression
o Hemidiaphragmatic paralysis due to phrenic nerve compression
o Wheezing, cough, dyspnea due to compression of the tracheobronchial tree
o SVC syndrome
● Descending aorta: Back pain from erosion into the adjacent spine
● Abdominal: Pulsatile abdominal mass, Constant abdominal pain or back pain
155
Lakotah Hardie
Vascular Medicine
TOC
D. Diagnosis
Typically, an incidental finding on imaging studies (e.g., CT, CXR, echo)
TAA:
1. CTA or MRA: Helpful in determining size and extent of the aneurysm. Patients with known connective tissue
disease syndromes or first-degree relatives with TAA should undergo complete aortic imaging.39
2. TTE: Indicated when aortic root or ascending aortic aneurysm is present to evaluate for the presence of a
bicuspid aortic valve and aortic regurgitation.
AAA:
1. Transabdominal US: Preferred for screening and surveillance of infra-renal AAA. Initial test if suspect AAA. High
sensitivity at 98% and specificity of 99%42.
2. CT imaging: Preferred for supra-renal AAA, stable symptomatic patients, preop planning and often post-op
surveillance.
E. Management
TAA: The decision point for management rests on whether there is any indication for surgical repair. Repair options
include open surgery or endovascular repair.
AAA: Options for repair include open surgical repair or endovascular repair (EVAR) if indicated. EVAR 1, DREAM, OVER
all show initial survival benefit with EVAR but similar long-term mortality at 8-10 years. Patients who undergo EVAR
require long-term surveillance with CT for development of endoleaks 49,50.
156
Lakotah Hardie
Vascular Medicine
TOC
F. Surveillance
TAA: Asymptomatic patients with thoracic aortic aneurysm who do not meet indications for repair should undergo long-
term monitoring. Imaging modalities used for surveillance include TTE, CTA, or MRA.
AAA: The U.S. Preventive Services Task Force (USPSTF) recommends a one-time screening by abdominal
ultrasound for men aged 65 to 75 years who have ever smoked Society guidelines also recommend ultrasound
screening for AAA in first-degree relatives of patients with AAA who are >65 years of age.43,51 Once detected, it is
recommended that monitoring continue until aneurysm diameter exceeds 5.5 cm.
157
Lakotah Hardie
Vascular Medicine
TOC
Aortic Dissection
A. Definition
Tearing of the aortic intima resulting in separation of the arterial wall into
two layers forming a false lumen that can propagate both distally and
less commonly in a retrograde fashion. The Stanford system is the most
widely used classification system that guide management decisions.
B. Risk Factors
Hypertension, collagen disorders (Marfan Syndrome, Ehlers-Danlos
Syndrome type IV, Loeys-Dietz Syndrome, aortic surgery/
instrumentation (CABG, coronary cath, TAVR), preexisting aortic Stanford Type A Type B
aneurysm, congenital heart defects (bicuspid aortic valve, aortic DeBakey I II III
coarctation), and pregnancy.
Aortic dissection classifications. Adapted from Ital J
C. Clinical Presentation Vasc End Surg 2015;22:141.
● Symptoms: Ripping/ tearing chest pain, inter-scapular back pain,
abdominal or per umbilical pain (may be migratory as dissection
propagates caudally), hypertension, hypotension, syncope, AMS.
● Physical exam: Asymmetric BP (SBP variation >20 mm Hg between arms) or pulse deficit
● Image findings: CXR with mediastinal widening on CXR (60-90% of cases). EKG findings are 31% normal, 42%
nonspecific ST-T changes, 15% ischemia, and 5% acute MI
Complications of aortic dissection are mainly from propagation and include aortic valve regurgitation, cardiac tamponade,
obstruction of coronary artery ostia, coronary artery dissection, and end-organ failure due to abdominal aortic branch
vessel obstruction.
End organ complications Presentation
Acute MI (RCA most common53) Chest pain; ST-T changes, + trop
Acute stroke Focal neurologic deficits, headache
Spinal cord ischemia Acute paraplegia
Mesenteric ischemia Abdominal pain, bloody diarrhea,
Limb ischemia 6 P’s (pallor, paresthesia, pain etc.)
D. Diagnostics
Imaging remains the mainstay of diagnosis. Diagnostic tools have been developed to better predict likelihood of aortic
dissection given variable nature of presentation.
D-dimer: Recent studies have demonstrated that a D-dimer <500ng/mL can effectively identify patients who do not have
acute aortic dissection. (97% sn, 56% sp, 96% NPV).54
Imaging: CTA most common initial test. If the patient is hemodynamically unstable, TEE. (98% sn & 63-96% sp).
Study Sens (%) Spec (%) Comment
CXR 68-90 n/a Mediastinal widening (64%), displaced intimal calcification (9%),
pleural effusion (16%), abnl aortic contour (76%), normal (12%)
TTE 30-80 83-96 Pros: Useful for complications: AI, tamponade, LV, valves
Cons: Only proximal aorta is visualized
Chest 97-100 83-100 Pros: Available, fast and holistic evaluation of chest
CTA* Cons: IV contrast, cannot evaluate coronaries or aortic valve
reliably, site of tear often not defined
MRA 98-100 87-100 Pros: Test of choice for following chronic dissection
Cons: Long image acquisition time
158
Lakotah Hardie
Vascular Medicine
TOC
TEE 97-100 98-100 If high pretest probability and chest CTA contraindicated.
Evaluate LV, AV, prox coronaries, and pericardial effusion
*Consider ordering CTA chest with neck, abdomen, pelvis w/ leg run offs
**Surveillance imaging should be obtained at 3, 6, 12 months and annually thereafter.
E. Management
Acute management consists of the following:
● Access: A-line in arm with highest pressure. Frequent BP checks of both sides; CVC; intubate
o Rule out tamponade / CHF, then volume resus 🡪 take emergently to the OR.
● Goal HR <60: IV BB (e.g., labetalol, esmolol). Reduce HR before blood pressure to prevent reflex tachycardia/
inotropy and minimize shear stress. CCB may be used as an alternative
o Caveat: reducing HR in the setting of acute severe AI will increase the diastole/systole ratio, may precipitate
cardiogenic shock.
● Goal SBP 100-120 mmHg: If SBP remains elevated after appropriate HR control, antihypertensive drip should be
started (nitroprusside or nicardipine)
o Avoid use of hydralazine - can increase hydraulic shear
● Urgent surgical consultation should be considered for all patients
o Type A Dissection: Stat page CT surgery. Typically treated surgically with sternotomy, aortic root
replacement with graft +/- valve repair/replacement since they are at high risk for complications.
▪ If untreated, mortality is 1-2%/hour for the 48hrs in acute proximal dissections.
▪ Surgery: mortality is 10-20%; medical therapy only: mortality is >50%.55
o Type B Dissection: usually treated medically. Surgical interventions are reserved for patients who develop
complications related to the dissection.
159
Lakotah Hardie
Vascular Medicine
TOC
References
1. Wells, P. S., Anderson, D. R., Bormanis, J et al. (1997). Value of assessment of pretest probability of deep-vein
thrombosis in clinical management. The Lancet, 350(9094), 1795–1798. https://doi.org/10.1016/s0140-
6736(97)08140-3
2. Pengo V, Denas G, Zoppellaro G, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid
syndrome. Blood. 2018;132(13):1365-1371. doi:10.1182/blood-2018-04-848333
3. Lyman, GH, Carrier M, AY C, et al. American Society of Hematology 2021 guideline for management of venous
thromboembolism: Prevention and treatment in patients with cancer. Blood Adv. 2021;5(4):927-974. (2021). Blood
advances, 5(7), 1953-1953.
Doi:10.1182/blood advances.2021004734
4. Kearon, C., Akl, E. A., Ornelas, J., et al. (2016). Antithrombotic Therapy for VTE Disease. Chest, 149(2), 315–
352. https://doi.org/10.1016/j.chest.2015.11.026
5. Enden, T., Haig, Y., Kløw, N.-E., et al. (2012). Long-term outcome after additional catheter-directed thrombolysis
versus standard treatment for acute iliofemoral deep vein thrombosis (the CaVenT study): a randomised
controlled trial. The Lancet, 379(9810), 31–38. https://doi.org/10.1016/s0140-6736(11)61753-4
6. Vedantham, S., Goldhaber, S. Z., Julian, et al. (2017). Pharmacomechanical Catheter-Directed Thrombolysis for
Deep-Vein Thrombosis. New England Journal of Medicine, 377(23), 2240–2252.
https://doi.org/10.1056/nejmoa1615066
7. Comerota, A. J., Kearon, C., Gu, et al. (2019). Endovascular Thrombus Removal for Acute Iliofemoral Deep Vein
Thrombosis. Circulation, 139(9), 1162–1173. https://doi.org/10.1161/CIRCULATIONAHA.118.037425
8. Holly, B. P., Funaki, B., & Lessne, M. L. (2018). Inferior Vena Cava Filters. Clinics in Chest Medicine, 39(3), 645–
650. https://doi.org/10.1016/j.ccm.2018.04.015
9. Squizzato, A., Donadini, M. P., Galli, L., et al. (2012). Prognostic clinical prediction rules to identify a low-risk
pulmonary embolism: a systematic review and meta-analysis. Journal of Thrombosis and Haemostasis, 10(7),
1276–1290. https://doi.org/10.1111/j.1538-7836.2012.04739.x
10. Kline, J. A., Courtney, D. M., Kabrhel, et al. (2008). Prospective multicenter evaluation of the pulmonary embolism
rule-out criteria. Journal of Thrombosis and Haemostasis, 6(5), 772–780. https://doi.org/10.1111/j.1538-
7836.2008.02944.x
11. Anderson, D., Rodger, M., Ginsberg, et al. (2000). Derivation of a Simple Clinical Model to Categorize Patients
Probability of Pulmonary Embolism: Increasing the Models Utility with the SimpliRED D-dimer. Thrombosis and
Haemostasis, 83(03), 416–420. https://doi.org/10.1055/s-0037-1613830
12. Khetpal, R., & Shibli, M. H. (2010). Predictability of Wells Score for the Diagnosis of Acute Pulmonary Embolism.
Chest, 138(4), 938A. https://doi.org/10.1378/chest.10949
13. Le Gal, G., Righini, M., Roy, P.-M., et al. (2006). Prediction of Pulmonary Embolism in the Emergency
Department: The Revised Geneva Score. Annals of Internal Medicine, 144(3), 165. https://doi.org/10.7326/0003-
4819-144-3-200602070-00004
14. Aujesky, D., Obrosky, D. S., Stone, R. A., et al. (2005). Derivation and Validation of a Prognostic Model for
Pulmonary Embolism. American Journal of Respiratory and Critical Care Medicine, 172(8), 1041–1046.
https://doi.org/10.1164/rccm.200506-862oc
15. Crawford F, Andras A, Welch K, et al. D‐dimer test for excluding the diagnosis of pulmonary embolism.
Cochrane Database of Systematic Reviews 2016, Issue 8. Art. No.: CD010864. DOI:
10.1002/14651858.CD010864.pub2. Accessed 10 February 2021
16. Schouten Henrike J, Geersing G J, Koek H L et al. Diagnostic accuracy of conventional or age adjusted D-dimer
cut-off values in older patients with suspected venous thromboembolism: systematic review and meta-
analysis BMJ 2013; 346 :f2492
17. Moore, A. J. E., Wachsmann, J., et al. (2018). Imaging of acute pulmonary embolism: an update. Cardiovascular
Diagnosis and Therapy, 8(3), 225–243. https://doi.org/10.21037/cdt.2017.12.01
18. Chatterjee, S., Chakraborty, A., Weinberg, I., et al. (2014). Thrombolysis for Pulmonary Embolism and Risk of All-
Cause Mortality, Major Bleeding, and Intracranial Hemorrhage. JAMA, 311(23), 2414.
https://doi.org/10.1001/jama.2014.5990
19. Wells PS, Hirsh J, Anderson DR, et al. Accuracy of clinical assessment of deep-vein thrombosis. Lancet. 1995
May 27;345(8961):1326-30. doi: 10.1016/s0140-6736(95)92535-x. Erratum in: Lancet 1995 Aug
19;346(8973):516. PMID: 7752753.
20. Haller, E. P., Nestler, D. M., Campbell, et al. (2014). Point-of-Care Ultrasound Findings of Acute Pulmonary
Embolism: McConnell Sign in Emergency Medicine. The Journal of Emergency Medicine, 47(1), e19–e21.
https://doi.org/10.1016/j.jemermed.2014.01.032
21. Mismetti, P., Laporte, S., Pellerin, et al. (2015). Effect of a Retrievable Inferior Vena Cava Filter Plus
Anticoagulation vs Anticoagulation Alone on Risk of Recurrent Pulmonary Embolism. JAMA, 313(16), 1627.
https://doi.org/10.1001/jama.2015.3780
22. Fowkes FG et al. Comparison of global estimates of prevalence and risk factors for peripheral artery disease in
2000 and 2010: a systematic review and analysis. Lancet. 2013;382(9901):1329-40.
160
Lakotah Hardie
Vascular Medicine
TOC
23. Hirsh AT et al. ACC/AHA 2005 practice guidelines for the management of patients with peripheral arterial disease
(lower extremity, renal, mesenteric, and abdominal aortic). Circulation. 2006;113(11):e463-654.
24. USPTF. Screening for Peripheral Artery Disease and Cardiovascular Disease Risk Assessment With the Ankle-
Brachial Index: US Preventive Services Task Force Recommendation Statement. JAMA. 2016;320(2):177-183
25. Vemulapalli S et al. Supervised vs unsupervised exercise for intermittent claudication: A systematic review and
meta-analysis. Am Heart J. 2015;169(6):924-937.
26. Murphy TP et al. Supervised Exercise, Stent Revascularization, or Medical Therapy for Claudication Due to
Aortoiliac Peripheral Artery Disease: A Randomized Clinical Trial. J Am Coll Cardiol. 2015;65(10):999-1009
27. Bhatt DL et al. Clopidogrel and aspirin versus aspirin alone for the prevention of atherothrombotic events. N Engl
J Med. 2006;354(16):1706-17
28. Eikelboom JW et al. Rivaroxaban with or without Aspirin in Stable Cardiovascular Disease. N Engl J Med.
2017;377(14):1319-1330
29. Norgren L et al. Inter-Society Consensus for the Management of Peripheral Arterial Disease (TASC II). J Vasc
Surg. 2017;S5067
30. Tafur-Soto JD et al. Renal artery stenosis. Cardiology Clinics 2015;33(1):59-73.
31. Gupta R et al. Renal artery stenosis: new findings from the CORAL trial. Curr Cardiol Repo. 2017;19:75.
32. Hansen KJ et al. Prevalence of renovascular disease in the elderly: a population-based study. J Vasc Surg.
2002;36(3):443–451.
33. Safian RD et al. Renal-artery stenosis. N Engl J Med. 2001;344(6):431–442.
34. Olin JW et al. Fibromuscular dysplasia: State of the science and critical unanswered questions: A scientific
statement from the American Heart Association. Circulation 2014;129:1048-78.
35. Olin JW et al. The United States registry for fibromuscular dysplasia: Results in the first 447 patients. Circulation
2012;125(25):3182-90.
36. Garovic VD et al. Renovascular hypertension: Balancing the controversies in diagnosis and treatment. Cleveland
Clinic J of Med. 2005;72(12): 1135–1146.
37. Cooper CJ et al. Stenting and medical therapy for atherosclerotic renal artery stenosis. NEJM 2014;370:13-22.
38. Prince M et al. When and how should we revascularize patients with atherosclerotic renal artery stenosis? JACC
Cardiovasc Interv 2019;12(6):505-17.
39. Elefteriades JA et al. Thoracic aortic aneurysm: Clinically pertinent controversies and uncertainties. JACC 2010;
55:841-57.
40. Kent, KC, et al. Analysis of risk factors for abdominal aortic aneurysm in a cohort of more than 3 million
individuals. J Vasc Surg. 2010; 52:539-48.
41. Lederle, FA et al. Prevalence and associations of abdominal aortic aneurysm detected through screening.
Aneurysm detection and management (ADAM) veterans’ affairs cooperative study group. Ann Intern
Med.1997;126:441-9.
42. Chaikof EL, et al. The society for vascular surgery practice guidelines on the care of patients with an abdominal
aortic aneurysm. J Vasc Surg. 2018; 67:2-77.
43. Davies RR, et al. Yearly rupture or dissection rates for thoracic aortic aneurysms: simple prediction based on size.
Ann Thorac Surg 2002; 73:17-28.
44. Coady MA, et al. Familial patterns of thoracic aortic aneurysms. Arch Surg 1999; 134:361-7.
45. Vouyouka AG et al. Arterial vascular disease in women. J Vasc Surg. 2007; 46:1295-1302.
46. Isselbacher EM. Thoracic and abdominal aortic aneurysms. Circulation 2005; 111:816-28.
47. Hiratzka LF et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and
management of patients with thoracic aortic disease. JACC 2010; 55:e27-129.
48. Pasternak B et al. Fluoroquinolone use and risk of aortic aneurysm and dissection: nationwide cohort study. BMJ
2018; 360:k6
49. The United Kingdom EVAR trial investigators. Endovascular versus open repair of abdominal aortic aneurysm.
NEJM. 2010; 362:1863-71.
50. The UK small aneurysm trial participants. Mortality results for randomised controlled trial of early elective surgery
or ultrasonographic surveillance for small abdominal aortic aneurysms. Lancet. 1998; 352:1649-55.
51. Norman PE, et al. Population based randomised controlled trial on impact of screening on mortality from
abdominal aortic aneurysm. BMJ. 2004; 329:1259-64.
52. Bickerstaff LK, et al. Thoracic aortic aneurysms: a population-based study. Surgery 1982;92:1103-8.
53. Lentini et al. Aortic dissection with concomitant acute myocardial infarction: From diagnosis to management. J
Emerg Trauma Shock. 2011; 4:273-8
54. Shimony A, Filion KB, Mottillo S, Dourian T, Eisenberg MJ. Meta-analysis of usefulness of d-dimer to diagnose
acute aortic dissection. Am J Cardiol. 2011 Apr 15;107(8):1227-34. doi: 10.1016/j.amjcard.2010.12.027. Epub
2011 Feb 4. PMID: 21296332.
55. Nienaber et al. Aortic dissection: new frontiers in diagnosis and management. Circ. 2003; 108: 628–635
56. Nazerian P, Mueller C, Soeiro AM, Leidel BA, Salvadeo SAT, Giachino F, Vanni S, Grimm K, Oliveira MT Jr,
Pivetta E, Lupia E, Grifoni S, Morello F; ADvISED Investigators. Diagnostic Accuracy of the Aortic Dissection
Detection Risk Score Plus D-Dimer for Acute Aortic Syndromes: The ADvISED Prospective Multicenter Study.
161
Lakotah Hardie
Vascular Medicine
TOC
Circulation. 2018 Jan 16;137(3):250-258. doi: 10.1161/CIRCULATIONAHA.117.029457. Epub 2017 Oct 13.
PMID: 29030346
57. Cigarroa et al. Diagnostic imaging in the evaluation of suspected aortic dissection. Old standards and new
directions. NEJM 1993; 328: 35–43
162
Lakotah Hardie
TOC
164
Lakotah Hardie
Vascular Medicine
TOC
o The USPSTF recommends one-time screening by abdominal ultrasound for men aged 65 to 75 years
who have ever smoked (i.e., 100 or more lifetime cigarettes)
● Patients with AAA are often asymptomatic but may present with a sensation of pulsation near the umbilicus,
constant abdominal or back pain.
o The classic triad of ruptured AAA (severe acute pain, pulsatile abdominal mass, and hypotension) is
present in only 50% of cases
● In stable patients with a high suspicion for ruptured AAA, CT aortography is the initial test of choice
● In patients who presents with abdominal or back pain and some suspicion for AAA, abdominal ultrasound is the
initial test of choice
● Indications for repair include:
o Symptomatic AAA regardless of diameter
o Asymptomatic AAA if Diameter ≥5.5 cm, Saccular abdominal aneurysms, Rapid growth rate
● Repair options include open surgical repair or endovascular repair (EVAR)
● Medical management is focused on risk modification
o There is no clear evidence that pharmacotherapy (statins, beta blockers, ACE inhibitors) reduce AAA
growth
● Surveillance imaging should be performed using transabdominal ultrasound
Aortic Dissection
● Aortic dissection occurs when a tear in the aortic intima exposes the medial layer to the systemic pressure of
intraluminal blood
o Type A dissections involve the ascending +/- descending aorta. Type B dissections involve only the
descending aorta
● Risk factors for aortic dissection include HTN (most common), cocaine, heavy lifting, pregnancy, collagen
disorders (Marfans/ Ehlers Danlos type IV), and prior aorta surgery/ minimally invasive intervention.
● Most patients with aortic dissection present with abrupt-onset severe, sharp chest pain that
can radiate to the back
o Exam findings include a pulse deficit, SBP variation >20 mm Hg between arms, and findings associated
with complications/distal propagation
o Complications include: Acute AI, tamponade, and end-organ ischemia secondary to branch-vessel
involvement
● CTA is the most commonly performed imaging modality available
o If hemodynamically unstable, TEE is the preferred diagnostic test given its high sensitivity and specificity
o The addition of a negative D-dimer <500ng/mL can help to effectively rule-out dissection in addition to low
ADD-RS score (<1)
● Type A dissections require emergent surgical evaluation for consideration of sternotomy and
aortic root replacement with graft +/- valve repair/replacement. PAGE CT SURGERY
● In contrast, Type B dissections are typically managed medically with surgical intervention
reserved for patients who develop critical complications
o Use IV beta blockers first to aggressively reduce HR to goal <60 (reduces reflex tachycardia and
decreases shear stress)
o Once HR goal achieved, use vasodilators (nitroprusside, nicardipine) to reduce SBP (goal SBP 100-120
mm Hg)
● Longer-term management of Type B dissection involves ongoing therapy with anti-hypertensives (goal BP
<120/80) to reduce incidence of late complications
o Beta blockers or nondihydropyridine CCB and ACE are preferred over hydralazine
165
Lakotah Hardie
TOC
166
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
The 2018 ACC/AHA Adult Congenital Heart Disease (ACHD) guidelines presented a new classification system (1).
The ACHD anatomic and physiological (ACHD AP) classification system uses both anatomic complexity and
physiologic/functional status.
● Anatomic classification:
o Class I (simple)
o Class II (moderate complexity)
o Class III (great complexity)
● Physiological classification:
o stages A-D (similar to the NYHA heart failure classification system)
o considers patient’s functional status and presence of valve disease, pulmonary hypertension,
arrhythmias, aortic dilatation, end-organ function, or cyanosis.
167
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
PFO is defined as failure of the foramen ovale (opening between the atria in fetal circulation that allows blood to bypass the
pulmonary vasculature) to close in the first few months of life, allowing a flap-like communication between the RA and LA
Epidemiology/Presentation
● PFOs are common (25-30% of the general population) and generally asymptomatic
● PFOs are more common (~40%)2 in patients presenting with cryptogenic stroke, especially in those who
suffer strokes before age 55
● Very rarely do PFOs result in right-to-left shunting and hypoxia, or orthodeoxia (positional desaturation)
● PFOs should be screened for in the setting of a cerebral ischemic event in young patients or hypoxemia of
uncertain origin
o Associated atrial septal aneurysms (ASA) confer highest risk for recurrent PFO-related stroke5
Imaging: Echocardiogram with color flow Doppler or agitated saline contrast (a “bubble study”) is the diagnostic test
of choice.
● TEE has higher sensitivity than TTE for the detection of PFO
Management
● Primary prevention of stroke in patients with a PFO is not indicated3
● Secondary prevention in patients with ischemic stroke/TIA and found to have PFOs and without alternative
explanation for stroke despite thorough evaluation:
o For patients aged ≤60 yrs: percutaneous PFO device closure in addition to
antiplatelet therapy, rather than antiplatelet therapy alone4
o For patients > 60 yrs: data less clear, as the risks of intervention must be balanced with changing
technology (including percutaneous suture options)6
▪ This does not apply to selected patients with strong clinical evidence of paradoxical
embolus (ex: pts with acute DVT, PE, or other VTE)
References:
1. Stout KK et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J am Coll
Cardiol. 2018;73(12):1494-1563.
2. Lechat et al. Prevalence of patent foramen ovale in patients with stroke. N Engl J Med. 1988; 318:1148-1152.
3. Messé et al. Practice advisory update summary: Patient foramen Ovale and secondary stroke prevention: Report of the
Guideline Subcommittee of the American Academy of Neurology. Neurology. 2020; 94 (20).
4. Mojadidi et al. Cryptogenic Stroke and Patent Foramen Ovale. J Am Coll Cardiol. 2018; 71:1035-1043.
5. Turc et al. Atrial septal aneurysm, shunt size, and recurrent stroke risk in patients with patent foramen ovale. J Am Coll
Cardiol. 2020; 75(18):2312-2320.
6. Gaspardone and Sgueglia. Cryptogenic stroke over 60 years of age: should patent foramen ovale be closed? Eur
Heart J Supplements. 2020; 22: E82-E86
169
Max Quinn
Congenital Heart Disease
TOC
Epidemiology/Presentation
● Most common type of congenital heart lesion
● 2:1 female to male predominance
● Typically, left-to-right atrial shunt (due to RV compliance)
o Can eventually lead to R-sided volume overload, RV enlargement and pHTN (rarely with large
defects, right-to-left shunting and Eisenmenger Syndrome)
▪ In right-to-left shunting, increased risk of paradoxical embolization and stroke
Exam: Large ASDs may result in a fixed and split S2 that does not vary with inspiration. A soft systolic crescendo-
decrescendo systolic pulmonary flow murmur may be heard due to increased flow over the pulmonary valve.
Precordial lift may be felt in cases or RV enlargement. Prominent P2 may be present at the apex if resting
pulmonary hypertension has developed.
Imaging:
● TTE generally first step, sinus venosus defects can be difficult to diagnose by TTE but RV enlargement
without other explanation should prompt additional imaging
● TEE, cardiac CT, or MRI may additionally be performed to help define the margins of the defect (an
important consideration for device closure of secundum ASD) and to identify associated pulmonary
venous anomalies
Management
ASD repair (primum or secundum, either transcatheter or surgical) is indicated (Class I recommendation),1 if:
● The patient exhibits impaired functional capacity, has RA and/or RV enlargement, and net left-to-right
shunting large enough to cause physiologic sequelae
ASD closure is NOT recommended (Class III: Harm) if:
● PA systolic pressure > 2/3 systemic pressure and PVR >2/3 SVR
● There is net right-to-left shunt
Note: All sinus venosus, primum, and coronary sinus defects must be repaired surgically. For isolated secundum
defects, percutaneous closure is the most common approach for repair, with extremely high success rates (surgical
repair reserved for very large defects or patients with deficient septal rims).
References:
1. Stout KK et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J am Coll
Cardiol. 2019;73(12):1494-1563.
170
Anna O’Kelly
Congenital Heart Disease
TOC
VSDs results from the failure of septal development. There are four main types:
● Supracristal (5%): Result from deficiency of the septum inferior to the aortic and pulmonary valves. Aortic
valve cusp can prolapse into the VSD, leading to progressive AR and occasionally aortic sinus dilatation
● Perimembraneous (60-70%): Most common, located near the membranous septum below the AV
● Muscular (10%): Can be small or large, single or multiple, and may spontaneously close. In adults, they
are generally small
● Inlet (rare): Results from deficiency of the inlet septum located beneath both mitral and tricuspid valves.
Does not result in mitral or tricuspid regurgitation unless associated with an AV canal defect and septum
primum defect (often seen with Down’s syndrome)
Epidemiology/Presentation
● VSDs are among the most common congenital heart entities in early childhood, but only account for 10% of
ACHD. Most close spontaneously.
● Small restrictive VSDs rarely cause chamber enlargement
● Moderate VSDs result in left heart volume loads (via L to R shunting)
● Large VSDs can lead to L-to-R shunting—enough to cause biventricular failure, RV volume overload,
pHTN, and even reversal of flow (R-to-L shunting, i.e. Eisenmenger Syndrome)
● Note: Congenital VSDs differ from post-infarct VSDs; which typically are muscular, large, and impact RV
function primarily.
Exam: Small VSDs may be associated with a high-pitched pansystolic murmur. Larger VSDs tend to cause lower-
frequency murmurs or may not be audible. These should augment with hand-grip (due to increased left ventricular
afterload and increased shunt fraction).
**The presence of a diastolic murmur in a patient with known restrictive VSD must be further evaluated (aortic
regurgitation or aortic fistula).
Imaging: TTE is generally sufficient to define the lesion and assess for biventricular size and function.
Management
● Class I indication for repair1:
o Evidence of LV volume overload and hemodynamically significant shunt AND PA systolic pressure < ½
systemic systolic pressure and PVR < 1/3 SVR
● Class II indication for repair1:
o Worsening aortic regurgitation due to VSD (Class IIa)
o History of infective endocarditis caused by VSD (if not otherwise contraindicated) (Class IIb)
o There is net L-to-R shunt when PA systolic pressure > ½ systemic systolic pressure and/or PVR >
1/3 SVR
● Repair contraindicated (Class III: Harm)1:
o PA systolic pressure > 2/3 systemic pressure, PVR > 2/3 SVR, and/or there is net R to L shunt
References:
1. Stout KK et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J am Coll
Cardiol. 2018;73(12):1494-1563.
171
Max Quinn
Congenital Heart Disease
TOC
Coarctation of the Aorta (CoA) is characterized by discrete or diffuse narrowing of the aorta. The typical location is in
the region of the ligamentum arteriosum, adjacent to the origin of the L subclavian artery.
Epidemiology/Presentation2
● Accounts for ~5% all congenital heart defects
● Occurs more commonly in males
● Can be associated with other congenital cardiac lesions
o In children, CoA is often associated with complex congenital heart lesions
o In adults, bicuspid aortic valve is the most common associated defect
● Obstruction to blood flow causes increased afterload on the LV leading to LV hypertrophy
● There is also differential hypertension, with higher BP in vessels proximal to the obstruction and relative
hypotension distally (patients may suffer from claudication).
● Patients are at increased risk of aortic dissection, particularly with pregnancy. 10% of CoA cases have
aneurysms along the Circle of Willis (as below, should be screened with a one-time MR/CTA).
Exam: Differential hypertension can be pressure (> 20mmHg BP difference between arm and leg). Lower extremities
may have diminished or late pulses (brachiofemoral delay). Systolic bruit may be heard along the scapular region.
Systolic click of a bicuspid aortic valve may be heard if present (about 50-60%)
Imaging:
● CXR may show aortic indentation at the CoA site “3 sign,” (see Figure)
and notching on the underside of the ribs from collateral vessels
● TTE views from the suprasternal notch can demonstrate a narrowed
aortic lumen as well as measure pressure gradient across the CoA
segment
● CT/MRI preferred for screening for late complications (aneurysm, focal
dissection, re-stenosis) and for helping guide interventional approach
Management1
● Class I indication for surgical repair:
o Adults with native or recurrent CoA who have hypertension and significant native/recurrent
coarctation
● Type of repair varies based on anatomy
o Percutaneous stent implantation or surgical repair is procedure of choice, though balloon
angioplasty can be considered if stent/surgical repair not feasible
References:
1. Stout KK et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J am Coll
Cardiol. 2019;73(12):1494-1563
2. Baumgartner H et al. ESC guidelines for the management of grown-up congenital heart disease.
Eur Heart J 2010;31:2915–57
172
Max Quinn, Chris Marnell
TOC
Tetralogy of Fallot
Tetralogy of Fallot (TOF) is a constellation of four
abnormalities: pulmonic stenosis, VSD, overriding
aorta, and right ventricular hypertrophy
Epidemiology/Presentation
● Initial diagnosis and management
typically occurs by early childhood
o Most patients in the current era will
have undergone surgical repair in
early infancy
o Some older adult patients may
have undergone palliative
shunting prior to surgical repair
● It is the most common palliated complex
CHD in adults
Exam:
● Findings in adults are variable and will
depend on prior management (e.g. repair
versus palliative shunt).
● Scar anatomy is important (thoracotomy
and/or sternotomy) Figure 1. Native anatomy in
● Many patients will have systolic RVOT murmur and patients with PR will have a TOF (Adult Congenital Heart short
diastolic murmur. Disease in Clinic Practice
● Diminished radial pulse may indicate prior BT shunt. 2018)
● Right heart failure exam is critical (JVP, ascites, leg edema, right heart gallops)
Imaging:
● TTE is first line imaging and used to assess the degree of pulmonary regurgitation and other associated residual defects
(VSD, pulmonic stenosis, aortic dilation)
● Cardiac MRI is the gold standard for following RV size and function and pulmonic regurgitation quantification and to assess for
indications for pulmonary valve replacement (performed once RV volumes reach a certain size).
Management1
● Management in adulthood is primarily aimed at addressing late complications after childhood surgical repair including
pulmonic insufficiency, recurrent pulmonary stenosis, complications of prior shunts, and arrhythmia
o Many patients require repeat pulmonary valve intervention during adulthood (either surgical or transcatheter). Note:
the origins and proximal courses of coronary arteries must be mapped prior.
o Even after repair, adult pts with TOF are at increased risk of heart failure and sudden cardiac death (~2% per decade
of life), which may require ICD for primary or secondary prevention as well as targeted anti-arrhythmics and/or
ablation
173
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
There are 2 types of Transposition of the Great Arteries (TGA): D-TGA and L-TGA
● D-TGA (“dextra-TGA”): aorta arises from anterior of the RV and the PA arises from the LV such that the RV is the systemic
ventricle (deoxygenated blood is in systemic circulation, oxygenated blood is in the pulmonary circulation). Thus, systemic and
pulmonary circulations are in parallel
● L-TGA (“levo-TGA”): in this “congenitally corrected” type of TGA, the morphologic RV is on the left and the morphologic LV is
on the right, the pulmonary artery arises from the right sided LV and the aorta from the left sided RV, such that pulmonary and
systemic circulation still occur in series
D-TGA
174
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
From Adult Congenital Heart Disease in Clinic Practice (DeFaria Yeh & Bhatt)
Figure 3: Jatene
Procedure for D-TGA
correction
175
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
L-TGA
● Because this is a “congenitally corrected” anomaly (ie systemic and
pulmonary circulation are in series), patients may present in adulthood
o Some adult patients will have undergone surgical correction in
childhood
o May be picked up earlier in life if associated PS/VSD where
murmurs may lead to TTE
● L-TGA can be associated with other congenital anomalies such as VSD,
pulmonary stenosis, apical displacement of systemic tricuspid valve, and
displacement of the AV node with accessory AV node pathways (this often
leads to complete heart block and/or re-entrant SVTs).
● Heart block is common: 2% development per year
Exam: In both D-TGA and L-TGA the exams will be highly variable based on prior
surgical corrections and/or associated congenital anomalies
Imaging: In both D-TGA and L-TGA, cardiac MRI generally provides the best
assessment of structural and functional complications. Cardiopulmonary exercise
testing is important to assess peak VO2 which is highly prognostic. From Adult Congenital Heart Disease
in Clinic Practice (DeFaria Yeh &
Management Bhatt)
● Management of the adult patient with D-TGA involves managing
complications of prior intervention
o Arrhythmia: Ambulatory monitoring for bradycardia or sinus node dysfunction (especially true of pts treated with beta
blockers/other rate-slowing medications).1 Otherwise, standard targeted anti-arrhythmics and ablation procedures
advised.
o Baffle obstruction/leak: May present as edema of the face and upper extremities with superior baffle limb obstruction.
If the inferior baffle limb is obstructed, cirrhosis and ascites may develop. These patients may also exhibit
desaturation or paradoxical embolization if baffle leaks occur. Leaks/obstruction can be corrected percutaneously or
surgically. Cardiac MRI provides the best assessment of structural and functional complications.
o Progressive systemic RV dysfunction: Manage as per any other pt with RV dysfunction
● Management of the adult patient with L-TGA involves management of complications including arrhythmia and hemodynamic
effects
o Arrhythmia: As a result of AV nodal displacement, patients are at high risk for CHB. Many will require pacemaker
implantation (in subpulmonary LV). Ablation may also be necessary for accessory pathways contributing to AVRTs
o Progressive systemic right ventricular dysfunction: Pts are at risk of severe systemic TR due to anterior leaflet
dislocation, and TV repair can be considered in rare situations. In many cases, if systemic RV failure develops then
transplantation must be entertained.
References:
1. Stout KK et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J am Coll Cardiol.
2019;73(12):1494-1563
176
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
177
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
Fontan Procedure: (a) RA to PA connection (b) lateral tunnel, intracardial (c) extracardiac Fontan
Management
● Patients with
Fontan circulation
must be managed in
conjunction with
specialists in ACHD
● Fenestrations
(between the Fontan
lateral tunnel and
systemic circuit) can
be created which
allow a small right-
to-left shunt to
From Adult Congenital Heart Disease in Clinic Practice (DeFaria Yeh & Bhatt) persist
o Benefits include
increased cardiac output due to increased systemic preload and decreased central venous pressures
o Downside is that fenestration is associated with increased risk of paradoxical thromboembolism
o Hypoxemia/cyanosis may also occur in cases of excessive right greater than left shunting
● Common complications:
o Arrhythmia: Atrial dysrhythmias are common and poorly tolerated. Targeted antiarrhythmics, ablation procedures, and
ultimately conversion to extra-cardiac conduit may be indicated
o Cyanosis: Cross sectional imaging (CTA or MRI) plus RHC should be performed to look for systemic to pulmonary
venous connections, which may be treated by transcatheter occlusion
o Thrombosis: Fontan physiology represents a low-flow state, and the degree of right-to-left shunting in these patients
makes systemic thromboembolism a very high risk. Anticoagulation is indicated if there is history of atrial arrhythmia,
fenestration, or EF <40% (even in patients without history of thromboembolism)
o Protein-losing enteropathy: Though the pathophysiology is poorly understood, this is a complication occurring in 3-18% of
patients. Mortality is very high with only ~50% 5-year survival. Therapy is empiric and includes optimizing Fontan
hemodynamics, considering fenestration, optimizing cardiac output, and reducing pulmonary vascular resistance
o Spontaneous fenestration closure: This occurs frequently and may be a silent event. However, in patients with poor
cardiac output who were dependent on fenestration to augment ventricular preload, cardiac output may drop, systemic
venous pressures may rise, and protein-losing enteropathy may ensue
o Heart failure: Symptoms may progress such that transplantation may be the only therapeutic option. Of note, early post
procedure transplant outcomes within this population are poor relative to those with biventricular circulation due to
anastomotic challenges and bleeding. However, if patients survive, early post procedure window survival is on par with
other age-matched patients
o NAFLD: regular screening is imperative with liver ultrasound or MRI, HCC screening
There are several congenital abnormalities that cause blood to flow from the systemic to pulmonary circulation (left-to-right shunt).
Rarely, large uncorrected shunts may progress to Eisenmenger physiology in which the excess flow across the pulmonary arteries from
the shunt leads to pulmonary vascular remodeling and increased pulmonary vascular resistance (PVR), which results in shunt reversal
(right-to-left shunting) and cyanosis.
178
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
● In progression to Eisenmenger Syndrome, R-sided pressure overload results from both direct transmission of pressure from the
higher-pressure LV as well as increased afterload due to pulmonary arterial remodeling
o Overtime, increased PVR causes elevated right-sided pressures as the RV aims to maintain cardiac output. If right-sided
pressures approximate and then exceed left-sided pressures, the shunt direction can reverse, resulting in systemic
desaturation
● Rate of progression of pulmonary hypertension is dependent on the type and size of the anatomic defect, as well as the magnitude
of shunt flow
o Patients with ventricular (i.e. VSD, AVSD) or arterial shunts (i.e. PDA, truncus arteriosus) are at higher risk of progressing
rapidly to Eisenmenger syndrome compared to patients with pre-tricuspid shunt lesions
Exam: Examination may reveal signs of PH and RV failure (RV heave, loud P2 that extends to apex, prominent venous a wave);
chronic hypoxemia (cyanosis, clubbing); and/or TR/PR and murmur of the causative lesion (will become progressively more quiet as
pulmonary and systemic pressures equalize).
Imaging: TTE should be initial diagnostic imaging (as well as CT or cardiac MR in certain cases)
Management
Eisenmenger syndrome patients must be managed in conjunction with an ACHD specialist
● Care of patients with Eisenmenger syndrome includes avoidance of the following (Class I recommendations)1:
o Significant volume shifts (dehydration, or volume overload)
o Chronic exposure to high altitude
o Iron deficiency
o Strenuous activity (in particular, isometric exercise)
o Excessive heat (i.e. sauna, hot tub) due to risks associated with dehydration as well as worsening of right-to-left
shunting through systemic vasodilation
o Unnecessary surgery or anesthesia
o Pregnancy (high maternal mortality)
● These pts require prompt workup and treatment for infection, as they may not have the cardiac reserve to support septic
physiology
o Systemic vasodilation will worsen right-to left shunt flow
179
Max Quinn, Chris Marnell
Congenital Heart Disease
TOC
o Hyperuricemia: Increased uric acid levels are common in patients with cyanotic congenital heart disease and are due to
increased production and decreased renal clearance. Serum uric acid increases in proportion to hemodynamic severity in
adults with Eisenmenger syndrome and is associated with long-term mortality. Uric acid levels should be assessed
annually, and treatment of hyperuricemia should be initiated in patients who develop gout.
References:
1. Stout KK et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J am Coll Cardiol.
2019;73(12):1494-1563
2. DeFaria Yeh et al. 2018. Adult Congenital Heart Disease in Clinic Practice
180
Max Quinn, Chris Marnell
Cardio-Obstetrics
TOC
CARDIO-OBSTETRICS
Basic Physiology of Pregnancy
ANTEPARTUM
Hemodynamic changes in normal pregnancy (source: AHA)
SVR: ↓ (nadir 2T)
HR: ↑ (peaks in late 3T)
Total Blood Volume: ↑ “physiologic anemia
and ↑EPO”
CO: ↑ to 30-50% above baseline (peaks in
early 3T; increases due to (1) increased
preload, (2) reduced afterload and (3)
increased HR)
MAP: Mild ↓ in 1+2T, returns to pre-pregnancy
baseline in 3T (balanced ↓ SVR reduction with
concomitant ↑ in CO)
INTRAPARTUM
CO ↑ by ~15% in early labor, ~25% in active
phase of labor, ~50% during pushing in second stage. Note: Epidural anesthesia attenuates some of this increase.
Uterine contraction contributes 300-500cc blood/contraction into maternal circulation.
POSTPARTUM
Immediately post-partum CO ↑ to 80% above pre-labor value due to autotransfusion from aortocaval
decompression/uterine involution (blood auto-transfused usually > blood lost during labor). BP should remain constant
during this phase, hypotension is abnormal. Returns to baseline over ~ 3 months.
NOTE: hypotension in third trimester may be due to positioning or volume depletion
Supine Positioning 🡪 hypotension 2/2 compression of aorta and vena cava due to gravid uterus (reduced CO and SV,
increases HR) 🡪 place in left lateral decubitus shifts uterus off major vessels 🡪 restoration of venous return, CO 🡪
improved BP
Risk stratification of women with pre-existing cardiovascular disease interested in pregnancy
1. Modified WHO classification (most commonly used; 2018 ESC guidelines). Population specific: CARPREG, ZAHARA,
ROPAC (Regitz-Zagrosek et al. EHJ 2018, 39(34): 2165-3241)
181
Adi Achanta and Nelson Hsieh
Cardio-Obstetrics
TOC
ACLS in Pregnancy
See 2015 AHA Statement on cardiac arrest in pregnancy for more details (Circulation 2015;132:1747-1773)
o At onset of arrest PAGE MATERNAL CARDIAC ARREST TEAM (see ACLS algorithm below)
o MATERNAL interventions and OBSTETRIC interventions should be performed IN TANDEM
Maternal interventions
o Place IV above diaphragm
o If receiving IV mag, STOP mag and give calcium chloride or gluconate
o Defibrillation and epinephrine should be given when indicated according to the standard ACLS algorithm
o If you suspect PE as a cause of arrest, treat it and get PERT involved; Alteplase is not strictly contraindicated in
pregnancy; heparin and LMWH are safe
Obstetric Interventions
o While performing compressions relieve aortocaval compression with lateral uterine displacement
o If no ROSC within 4-5 minutes, consider immediate perimortem cesarean delivery. Goal is to improve both
maternal and fetal outcomes
Potential Etiologies of Maternal Cardiac Arrest: ABCDEFGH
▪ Anesthetic ▪ Embolic
complications ▪ Fever
▪ Bleeding ▪ General non-OB causes (Hs
▪ Cardiovascular & Ts)
▪ Drugs ▪ Hypertension
182
Adi Achanta and Nelson Hsieh
Cardio-Obstetrics
TOC
183
Adi Achanta and Nelson Hsieh
Cardio-Obstetrics
TOC
Pregnancy and Lactation Labeling Rule (PLLR): In 2015, PLLR replaced previous FDA scoring system of medications
(used to be ABCDX). Incorporates consideration that baseline rate of major birth defects and miscarriage, and includes
recommendations for pregnancy, labor, delivery and lactation all on the same label. New system more descriptive than
prior ABCDX approach.
Guiding principles: Consider necessity, urgency, timing during gestation, fetal adverse effect of drug. Use lowest
effective dose. Counsel re: limited availability of data, when pertinent. MFM should help guide intrapartum and post-
partum medications.
Summary of safety during pregnancy for the most commonly used cardiac medications (adaptation of central
illustration from Halperin et al, see original paper for further discussion as well as FDA categorization and lactation safety)
(Jeejeebhoy et al. Circulation 2015; 132(18); 1747-73)
● Arrythmias Heart Failure/Shock HTN/PHTN Heme
Aldosterone
Atenolol
antagonists Statins
Contraindicated Amiodarone ACEi, ARB
Atenolol DOAC
Endothelin-R antag
ACEi, ARB
Metoprolol
Adenosine
Carvedilol Labetalol
Digoxin
Furosemide Nifedipine UFH
Considered Safe Lidocaine
Dopamine Alpha-methyldopa (PO) Enoxaparin
Metoprolol
Dobutamine
Propranolol
Norepinephrine
Verapamil Hydralazine
Amlodipine Epoprostenol Alteplase Bivalirudin
Diltiazem Isosorbide dinitrate
Limited data/ Hydralazine Nitroglycerin ASA 81mg Fondaparinux
Procainamide Nitroglycerin
to be used with Iso dinitrate Nitroprusside Clopidogrel Prasugrel
Sotalol Metolazone
caution Iloprost Sildenafil Warfarin Streptokinase
Flecainide Torsemide
Argatroban Ticagrelor
Propafenone
Incidence: PPCM seen in ~103/100k births in the United States with marked regional variation across the world, maternal
mortality rate ~ 14/100k of live births (Kolte et al. JAHA 2014; 3(3):e001056)
Risk factors: Age > 30 y.o, African descent, pregnancy with multiple fetuses, hx of PEC, eclampsia or postpartum HTN,
longterm tocolysis (Lee et al. Circ Heart Fail 2018; 11(4):e004134)
Pathophysiology: dysregulated metabolic pathways and angiogenic imbalance. Prolactin regulation (via STAT pathway)
and VEGF (in PGC-1a KO murine models and via sFlt1 antagonism) regulation implicated (Hoes et al. Nat Rev Cardiol
2022; doi: 10.1038/s41569-021-00664-8).
Diagnosis: Most often first month postpartum. S/Sx: dyspnea, pedal edema, orthopnea, PND, chest tightness. Note: do
not dismiss pedal edema and SOB symptom of pregnancy, especially post-partum (Arany et al. Circulation 2016;
133(14):1397-409). Work-up: NTproBNP, TTE, can obtain cMR but avoid gadolinium during pregnancy, no EMB unless
high suspicion for other etiology.
Management: Similar to other forms of HFrEF (see position statement from HF Association of the ESC for details) i.e.
GDMT; continue if EF remains depressed, however no data to support duration of tx after EF recovery (see figure below).
Important points: Anticoagulation: Consider when LVEF<30-35% during late pregnancy and 6-8 weeks postpartum.
Favor LMWH over warfarin (unless mechanical heart valves), both safe with lactation. No data for DOACs, avoid (Davis et
al. JACC 2020; 75(2) 207-221). Experimental: Bromocriptine in addition to standard therapy is associated with a higher
184
Adi Achanta and Nelson Hsieh
Cardio-Obstetrics
TOC
rate of EF improvement, although no difference in overall rates of recovery in several small trials. (Silwa et al. Circulation
2010; 121:1465-1473; Yaméogo et al. J Cardiol Clin Res 2017; 5:1098-1106; Hilfiker-Kleiner et al. Eur Heart J 2017;
38:2671-2679). Ongoing REBIRTH trial of 200 women with PPCM to further evaluate bromocriptine in PPCM on
myocardial recovery. ESC 2018 guidelines include Class IIb recommendation for bromocriptine; if used, must also
consider therapeutic AC in conjunction due to elevated thrombotic complication risk. Advanced therapies: 60% of
cardiogenic shock during or after pregnancy are related to PPCM (Banavan et al. Hypertens Pregnancy 2017; 36:117-
123). tMCS and ECMO should be considered early. Lactation: Current data suggest breastfeeding safe in PPCM, in
spite of stimulation of prolactin secretion (Koczo et al. JACC Basic Trans Science 2019; 4:291-300). Primary PPX for
sudden cardiac death: Because PPCM has higher likelihood of recovery of EF, important to avoid premature ICD;
consider wearable vest in severely reduced LVEF until recovery or ICD placement. Subsequent pregnancy: as
illustrated below, risk is stratified by recovery of EF; persistent systolic dysfunction portends high risk pregnancy.
185
Adi Achanta and Nelson Hsieh
Cardio-Obstetrics
TOC
(illustrations from JACC State-of-the-Art Review - Davis et al. JACC 2020; 75(2) 207-221)
Prognosis: 46% of patients have LVEF recovery within 6 months of diagnosis (Hoes et al. Nat Rev Cardiol 2022; doi:
10.1038/s41569-021-00664-8). If LVEF normalizes post-partum, women still carry an increased risk of clinical HF, further
deterioration in LVEF and persistent LV dysfunction with a subsequent pregnancy. If LVEF does not normalize post-
partum, risks of HF, further deterioration of LVEF, persistent LV dysfunction are further increased, and importantly
maternal mortality is increased with subsequent pregnancy (Elkayam JACC 2011; 58 (7) 659–670). Women should be
carefully counseled about very high risks of further pregnancies (Davis et al. JACC 2020; 75(2) 207-221)
186
Adi Achanta and Nelson Hsieh
Cardio-Obstetrics
TOC
Chronic (pre- ● HTN diagnosed or present before 20w gestation (also if first diagnosed during
existing) HTN pregnancy and present for >12w post-partum)
Chronic HTN + ● Chronic HTN with sudden increase in BP that was previously well controlled, or
superimposed requiring escalation of anti-HTN regiment to control BP
PEC +/- severe ● New onset proteinuria or sudden increase in proteinuria if had prior to pregnancy
features ● See severe features above
Adapted from UTD Hypertensive Disorders of Pregnancy
Risk factors: personal history of PEC, chronic HTN, age, multiple gestation, obesity, race, diabetes, renal disease,
autoimmune disease.
Management:
Acute severe hypertension: reduce MAP by no more than 25% over 2h using IV labetalol 20 mg over 2 minutes
(hydralazine also OK but labetalol preferred), followed at 10 min interval doses of 20 to 80 mg up to max total cumulative
dose of 300 mg if BP remains above target, constant infusion can be used instead of intermittent therapy. If labetalol
alone ineffective ACOG recommends switching to hydralazine (and vice versa). Alternative options include nicardipine
and nifedipine. If PEC or Eclampsia 🡪 give IV mag sulfate in addition to anti-hypertensive medication. Call OB.
Chronic HTN 🡪 target is less than 140/90 mmHg (CHAP Trial Consortium, NEJM 2022; prior CHIPS 2015 trial suggested
strict versus lax BP control were equal, but underpowered for clinical outcomes). Meds: First line: labetalol and nifedipine.
Second line: hydralazine. Methyldopa less effective. If already on amlodipine or HCTZ, ok to continue. STOP ACEi, ARBs,
MRA for teratogenicity.
Risk reduction: ASA 81mg daily during 2T and 3T reduces PEC risk. USPTF Grade B recommendation for ASA after 12
weeks gestation (similar to ACOG recommendation for ASA).
Arrhythmias in Pregnancy
Incidence: Significant ectopy (PVC, paroxysmal SVT) common. In addition, variation of SVT most common, followed by
AF/AFL, VF/VT (overall 70 per 100,000) (Vaidya et al. Circulation 2017; 135(6):619-621).
Pathophysiology: hypothesized to be related to atrial stretch leading to irritated tissue and re-entrant arrhythmias.
Important to control arrhythmias due to risk of impaired fetal perfusion. Sustained tachycardia not well tolerated, especially
AFL.
Diagnosis and Management: diagnosis and treatment for arrhythmias in pregnancy follows same principles as in non-
pregnant patients. Pregnancy may reveal index case of underlying structural heart disease or inborn arrhythmia (e.g.
WPW, ARVC, HCM, etc.) Notable points: adenosine and cardioversion are safe. Catheter ablation is safe, better to delay
until late pregnancy or postpartum. Metoprolol, propranolol, verapamil, digoxin, lidocaine (fetal bradycardia), procainamide
are preferred agents; AVOID atenolol (fetal bradycardia, IUGR, highest risk among BB although risk is shared),
amiodarone (consider after 1T), dronedarone. Lactation: All passed into breast milk. Anticoagulation: Use CHAD2S2-
VASc. Unclear risk of stroke with limited data (ESC Guidelines On Management of Cardiovascular Diseases During
Pregnancy Eur Heart J 2011; 32(34):3147-97).
Key principle: The fundamentals of diagnosing ACS in pregnant patient are the same as in non-pregnant patient
There are some baseline EKG changes in pregnancy (ex: LAD 2/2 diaphragm elevation, TWI III and aVF, small Q in III)
but ST changes are never normal (Kealey et al. Can J Cardiology 2010; 26(6): 185-9)
Management of acute MI in pregnancy: See diagram below. Adapted from: Ismail et al Clinical Cardiology 2017;40:
399-406
In addition, low-dose ASA (81mg), Beta Blocker (Metoprolol preferred) is safe. NB: Despite the appropriate role of PCI in
early mgmt. of MI in pregnancy, coronary catheterization rates during pregnancy are lower than expected in ACS
(Cauldwell et al Heart 2019;105(3):189-195).
Special case: Spontaneous coronary artery dissection (SCAD): See 2018 2018 AHA Scientific Statement on
management of SCAD for further details
Most common cause of acute MI in pregnancy and most commonly occurs first week after delivery. Dx: Coronary
angiogram is recommended. Management: medical management may be preferred over invasive given risk of
propagating the dissection22 especially if there is only minimal distal coronary ischemia or preserved coronary flow. PCI v
187
Adi Achanta and Nelson Hsieh
Cardio-Obstetrics
TOC
CABG should be considered in patients with ongoing ischemia, hemodynamic instability, or LM disease (Hayes et al,
Circulation 2018; 137: e523-e557). Obtain arterial imaging from head to pelvis as there is an association between SCAD
and fibromuscular dysplasia. Prognosis: SCAD treated conservatively 95% of the time will heal in one month (JACC
State-of-the-Art Review. J Am Coll Cardiol 2020;76:961-984).
Clopidogrel
Anti-Thrombin Low dose ASA (continue throughout delivery)
Therapy UFH (Argatroban for patients with history of HIT)
Peri-procedural GP IIb/IIa inhibitors in patients with prior MI,
high thrombus burden, complex PCI
188
Adi Achanta and Nelson Hsieh
TOC
189