Most common congenital heart disease in children  VSD

60% newborns  have heart murmurs

If you’re unsure of murmur type in children  systolic murmur (90% of the time you’re correct)

Most common murmur in Dr.Yao clinic  Innocent heart murmur

• AKA Still/non-significant/musical murmur
• 50-60% of kids between 3 -8yrs old
• kids can participate with normal activities
• normal heart
• Systolic/innocent murmur heard in:
Anemia
Fever
Jumping/exercising kids
Neck sound with venous hammer

1st heart sound  TIM  due to closure of tricuspid and mitral valve
 I = insufficiency
 TIM insufficiency = systolic murmur heard

2nd heart sound  SAP  due to closure to aortic and pulmonary valve
 S = stenotic aortic/pulmonary valve  murmur is always systolic

2 most common causes of systolic murmurs in newborns:

a) PDA
b) Peripheral pulmonary stenosis

6 Grades of heart murmurs

Grade 1  unsure of murmur, unable to hear
Grade 2  murmur heard, usually systolic
Grade 3  stethoscope: loud murmur heard, NO THRILL
Grade 4  hand on chest: THRILL FELT
Grade 5  stethoscope edge on chest: THRILL FELT
Grade 6  hand above chest: THRILL felt

 Murmurs louder when baby is sitting and leaned forward

 1st auscultation  Left upper sternal border  PDA, pulmonary stenosis
 Right upper sternal border  aortic valve problem, aortic stenosis, hypertrophic subaortic stenosis

 Radiate to back  pulmonary involvement

  Radiate to neck  aortic involvement
Trisomy 13, Edward’s syndrome  VSD
Fetal alcoholic syndrome  VSD
Trisomy 21, Down syndrome  AV canal, Endocardial cushion defect
Rubella syndrome  PDA
Premature baby  PDA
Ehler Danlos syndrome  Mitral valve prolapse
Marfan syndrome  Mitral valve prolapse
William’s syndrome  Supravalvular aortic stenosis
Asymmetrical Crying Facies Syndrome  Aortic stenosis
DiGeorge syndrome  Truncus arteriosus
Holt-Oram syndrome/Heart hand syndrome  ASD
Turner’s syndrome  Coarctation of aorta
Noonan syndrome  Pulmonary stenosis

Diagnosis of congenital heart disease

1) CXR  ↑sed pulmonary vascularity, ↓sed pulmonary vascularity = less blood going to lungs
-pulmonary stenosis  ↓sed pulmonary vascularity
-excessive blood to lungs = ↑sed pulmonary vascularity
-insufficient blood to lungs (TOF, Pulmonary stenosis) = ↓sed pulmonary vascularity, cyanotic,
-septal defect with left to right shunt (ASD, VSD, PDA, AV canal)  ↑sed pulmonary vascularity
**excessive blood to lungs in infants  CHF
-Mnemonic EasySTD!
Easy fatigability, sweating, tachypnea,
difficulty gaining weight/feeding, dyspnea
Descending aorta  Normal: left side orientation,
Abnormal: right side orientation (TOF, Truncus arteriosus)

Heart configuration  Boot-shaped heart = TOF

Heart/egg on a string = transposition of great vessels
Snowman/figure of 8 = Total anomalous pulmonary venous return (TAPVR)

Apex of heart  Normal: points towards left side

Abnormal: points towards right side (DEXTROCARDIA)
Scimitar syndrome
 dextrocardia
 right lung hypoplasia  asthma
 abnormal venous return to vana cava
 symptomatic

2) EKG  left atrial/ventricular enlargement, right/left ventricular hypertrophy

3) Echocardiogram (gold standard)
 Bradycardia in children  HR < 100
 Bradycardia in infant  HR < 60

 Tachycardia in children  HR > 100  seen when jumping, fever

 Most common arrhythmia treated in children  Supraventricular tachycardia (HR > 250)

 After 1 yrs old, if heart size is > 50% of thoracic ratio  cardiomegaly

 Diastolic murmur  NEVER NEVER EVER normal in kids!

 Congenital heart defects in newborns  2 signs: cyanosis, respiratory distress

 Most common cause of death in TOF  Stroke, brain abscess
 Most common mitral valve defect in rheumatic endocarditis  mitral valve insufficiency

 Most common cyanotic heart disease in newborn  Transposition of great vessels

 Most common cause of infant of diabetic mother  Transposition of great vessels

 Most common cyanotic heart disease in children  TOF

 Cyanosis  NO cognitive function impact

Causes stroke, thrombosis, brain abscess
Cyanotic baby in nursery  think about 4 conditions:
a) Cyanotic congenital heart disease
b) Respiratory problem
c) Methemoglobinemia
d) CNS Cyanosis

100% oxygen test/hyperoxia test  to differentiate cyanosis between these 4 conditions

Pink/↑ oxygen saturation upon test  Respiratory problem
Cyanotic upon test  congenital heart disease, R-L shunt
**Remember ROLEX mnemonic!
-people who wear ROLEX are cyanotic!
-R-L shunt = cyanotic
-Reduced Hb ≥ 3.5  clinically cyanotic

Factors that ↑ses cyanosis (oxygenated Hb > 3.5g)  PATRA!

P = Polycythemia (Hct ≥ 65%)  causes thrombosis,
blood flow sludge, blood clot,
no cognitive development impact

A = Acidosis
T = Temperature  ↑sed temperature (fever)  ↑sed cyanosis
R = Ratio of adult: fetal Hb (↑er fetal Hb = more oxygen)

If you see baby in nursery in shock, think about 4 conditions:

1) Cardiogenic shock
2) Septic shock
3) Hypovolemic shock
4) Hypoglycemic shock (blood glucose < 45)  hence, important to keep baby warm

4 most common cyanotic heart diseases: PDA, VSD, ASD, AV Canal

 Boys  TOF, Transposition of great vessels
 Girls  ASD, Mitral valve defects
ASD
 One of the mildest heart disease in children
 Don’t cause CHF
 Most common congenital heart disease in adults
 ↓sed exercise tolerance  SOB when climbing stairs, loud 1st heart sound
 Wide splitting of 2nd heart sound (longer closure of pulmonary valve)
 REMEMBER 1st heart sound is from TIM, 2nd heart sound is from SAP
 Mildest form
 No need for prophylaxis/fixation
 Fixation if 6mm or more
 CXR  ↑sed pulmonary vascularity, L-R shunt, heart not that big
 EKG  Slight atrial enlargement from blood flow in L to R atrium

3 types of ASD:
a) Mostly from ostium secundum  usually from persistent foramen ovale
foramen ovale closes at 2-3 months in 80%
foramen ovale persists  becomes ASD
b) Ostium primum 
c) Endocardial cushion defect  common in Down syndrome

Holt–Oram syndrome
 Atrial septal defect + skeletal abnormalities in upper extremities
 Autosomal dominant disorder that affects bones in the arms and hands (the upper limbs)
 May also cause heart problems

PDA
 Newborn  Systolic murmur heard in left upper sternal border
 Most common congenital heart disease in premature babies
 Murmur in left upper sternal border of newborns  PDA unless proven otherwise
 Indomethacin, Motrin  closes PDA (avoid motrin in pregnant mothers  facilitates closure of PDA)
 Prostaglandin  keeps ductus opened
 Can also occur in full term babies
  PDA: 1st systolic murmur  then becomes to and fro/continuous machinery like murmur
 Wide pulse pressure = systolic murmur = PDA
 Norwood operation to treat
 PDA life saving defect for hypoplastic heart syndrome patients
 PDA  life long risk of endocarditis (prophylaxis  amoxicillin)

VSD
 Most common congenital heart disease in children
 Eisenmenger’s syndrome  from VSD
 Holosystolic murmur + thrill  VSD unless proven otherwise
 Amoxicillin 250mg 6hrs before surgery and 6hrs after surgery  prevents endocarditis and Strep. viridans  2
kinds:
a) Membranous  75%
b) Muscular  25%, closes spontaneously (holosystolic murmur that “disappears” due to VSD closure)

Coarctation of Aorta
 More common in males
 Female + coarctation of aorta + webbed neck  Turner’s syndrome
 Normal: Leg pressure 10mmHg higher than arm (reversed in coarctation of aorta)
 Most common congential heart disease in kids with HTN
 CXR  Rib notching, figure of 3

Aortic Stenosis
 Systolic murmur heard
 Left ventricular hypertrophy
 Right upper sternal border murmur that radiates to neck
 Thrill
 Pre-systolic click

Pulmonary Stenosis
 Systolic murmur heard
 Right ventricle involved
 Right ventricular hypertrophy
 Right sided heart enlarged
 ↓sed pulmonary vascularity
 Left upper sternal border murmur that radiates to the back  Pre-systolic click  3 types:
 Mild  no treatment, just follow up in cardiology clinic 1x every 6 months,
Moderate 
Severe 

Transposition of the great vessels

 Most common cyanotic heart disease of newborn  transposition
 Most common cyanotic heart disease of infants of diabetic mothers  transposition
 CXR  heart on a string
 Treatment: Surgery

TOF
 Young baby crying  cyanotic/tet spells
 Prevent crying  Give oxygen, shot of morphine, flex hip
 Usual cause of death in TOF  Brain abscess or stroke
 Older kids  like to squat (to ↑se systemic pressure), finger clubbing, AA pts: examine oral mucosal membrane
 CXR  boot-shaped heart (from ↑sed pulmonary vascularity)
 2 conditions that give right aortic arch  TOF, truncus arteriosus
 Must have VSD to survive
 Associated with DiGeorge syndrome  4 components:
a) Right ventricular hypertrophy
b) Transposition of great vessels
c) VSD
d) Overriding of aorta

Total anomalous pulmonary venous return (TAPVR)

 CXR  figure of 8/snowman configuration, ↑sed pulmonary vascularity 
2 kinds TAPVR:
a) Obstructive pulmonary venous return  causes CHF (due to flooded lungs!), pulmonary HTN, shock
b) Non-obstructive pulmonary venous return  less severe, total mixing with large L-R shunt

Tricuspid Atresia
 Absent tricuspid valve
 Right ventricular hypoplasia
  Right atrium enlargement
 Associated with Ebstein anomaly
• Mothers on depression meds/lithium  tricuspid atresia
• -Parkinson-White syndrome
• Tricuspid valve moves downward towards right ventricular apex  tricuspid valve
regurgitation (holsystolic murmur)

Hypoplastic Left Heart Syndrome

 Most serious congenital heart disease
 Most common cause of death in first months of life
 Stenotic aorta/most vessels

Blalock–Taussig shunt  for first 6 months of severe cyanosis

 connect aorta to pulmonary artery
One branch of subclavian artery or carotid artery is separated and connected
with the pulmonary artery

Endocarditis
 Risk factors: valve defect/replacement, history of endocarditis,
untreated cyanotic heart disease, heart transplant
 Most common organism  Strep.viridans (for pts with congenital heart
disease)  Staph.aureus (for pts WITHOUT congenital heart disease)  2
criteria to diagnose endocarditis:
Blood culture  organisms
Echocardiogram  valve lesions

Acquired Heart Disease in Children  Rheumatic endocarditis, Kawasaki

Rheumatic Endocarditis  Most common acquired heart disease in children ABOVE AGE 5
 Sore throat
 Tonsilitis
 Hemolytic ß-Strep Group A
 Antibiotic to treat
 Heart murmur from mitral insufficiency
 Mitral valve involvement
  Treatment: oral penicillin, erythromycin for penicillin allergic pts
 Lifelong prophylaxis
 CHF  death
 Untreated Strep. Throat in kids  rheumatic endocarditis
 ACESS
A = Arthritis
C = Carditis
E = Erythema marginatum
S = Subcutaneous nodules
S = Sydenham chorea

Kawasaki/Mucocutaneous lymph node syndrome  FACES

F = Fever (104/105 for 5-6 days)
A = Adenopathy – unilateral (≥ 1.5cm)
C = Conjunctivits
E = Edema of feet and hands
S = Skin rashes

 Aspirin 100mg per Kg per day + Immunoglobulins to treat  ↓ses fever

 Sterile pyuria
 Normal platelets  after 2 weeks develops thrombocytosis  die from coronary aneurysm
 Abdominal pain from hydrops gallbladder
 Cloudy urine with no bacteremia + negative urine culture  Coronary bypass procedure in
children  ONLY IN KAWASAKI!
 Echocardiogram to check for coronary aneurysm (usually develops in 2-3 weeks)
 Most common acquire heart disease BELOW AGE 5

 Inotropic agent ↑ses heart contraction. Eg.Digoxin

 Chronotropic agent  ↑ses heart rate

 African americans  Diuretics  ACE-I  ß-Blockers

 Potassium in arrhythmias
 Remember the mnemonic KID  K+ Increase, heart stops at Diastole
 K+ too low, heart stops at Systole
 Mitral valve open and closure = Diastole Aortic valve open and close =
Systole
Supraventricular Tachycardia  HR > 230

Sinus Bradycardia
 HR < 60 in children
 HR < 20 in infants

Sinus Tachycardia  HR > 100 < 230

1st Degree AV Block  Prolongation of PR interval

2nd Degree AV Block  2 types: Mobitz Type I  PR interval prolongation, dropped beat
Mobitz Type II  no PR prolongation, dropped beat

3rd Degree/Complete Heart Block  HR < 50, pacemaker needed

Atrial Fibrillation
 Older patients
 Complications: Emboli

Sudden Infant Death Syndrome/Crib Death

 Sudden death BELOW 1YR OLD (usually 2 – 4 months old)
 Male > Female
 Common in African American babies
 Mothers  drug users
 Lung petechiae
 2 theories: Inhaling own carbon dioxide, Inhaling carbon dioxide triggering heart arrhythmias
 Normal heart and brain autopsy
 More common during winter
 Risk factors  Smoking during pregnancy/after delivery, premature baby,
 heater set too high (high heat), blankets/pillow overload
 Breast fed babies  ↓sed incidence
 Associated with Long QT Interval Syndrome
 Prone to develop ventricular tachycardia/fibrillation
Thickened heart septum  causes sudden death
Myocarditis
 Good prognosis
 Coxsackie virus

Pericarditis/Water Bottle Syndrome

 Children complain of chest pain
 Virus
 Pain alleviates with leaning forward

Hypertrophic Cardiomyopathy  Most common cause of sudden death in a normal person/athlete

Apparent life-threatening events (ALTE)

 AKA brief resolved unexplained event (BRUE)  baby for less than a minute has one or more of bluish skin,
poor breathing, weakness, or poor responsiveness

Munchausen syndrome by proxy

 Mothers purposely hurt babies to get pity from others
 Laxatives usually given to baby
 Lie about baby having cancer
 Usually from intelligent parents due to overwhelming stress from babysitting

Wolff–Parkinson–White syndrome (WPW)

 Short PR interval
 Delta wave
 Seen in  Ebstein anomaly
 Supraventricular tachycardia