Kawasaki Disease
Kawasaki Disease
Annual incidence
EARLY LATE
Leukocytosis Thrombocytosis
Left shift
Elevated CRP
Mild anemia
Thrombocytopenia/
Thrombocytosis
Elevated ESR
Elevated CRP
Hypoalbuminemia
Elevated transaminases
Sterile pyuria
ATYPICAL OR INCOMPLETE
KAWASAKI DISEASE
ST-T–wave changes.
Suggestive of myocarditis (50%) EMERGENCY
Tachycardia, murmur, gallop rhythms
Valvar insufficiency
IVIG
Most commonly proximal, can be distal
Left main > LAD > Right
CORONARY ARTERIAL CONT...
Vary in severity from echogenicity due to
thickening and edema or asymptomatic
coronary artery ectasia to giant
aneurysms
May lead to myocardial infarction (MI),
sudden death, or ischemic heart disease
CORONARY ANEURYSMS
25 % become smaller
25 % do not regress
7-20 % develop stenosis or myocardial infarction
attributed to their aneurysms
Giant aneurysms (>8mm) worst prognosis
CARDIOVASCULAR SEQUELAE
0.3-2% mortality rate due to cardiac disease
10% from early myocarditis
Aneurysms may thrombose, cause MI/death
Rarely, may rupture.
MI is principal cause of death in KD
32% mortality
Most often in the first year
High dose
80-100 mg/kg/day (4 times a day)
until 14 days after onset or 2-3 days after afebrile phase
Decrease to low dose
3-10 mg/kg/day (once a day)
for 6-8 weeks or until platelet levels normalize
Being stop if the result of echocardiography is normal
Long term usage
Beingsuggested for a patient with coronary aneurysm
2-5 mg/kg
Dueto potential risk of Reye syndrome instruct
parents about symptoms of influenza or varicella.
OTHER TREATMENTS
Pharmacologic Therapy
None beyond 6-8 weeks
Physical Activity
No restrictions beyond 6-8 weeks
Follow-up and diagnostic testing
CV risk assessment, counseling @ 5 yr intervals
Invasive testing
None recommended
II. TRANSIENT CORONARY ARTERY ECTASIA,
RESOLVED WITHIN 6-8 WEEKS
Pharmacologic Therapy
None beyond 6-8 weeks
Physical Activity
No restrictions beyond 6-8 weeks
Follow-up and diagnostic testing
CV risk assessment, counseling @ 3-5 yr intervals
Invasive testing
None recommended
III. SINGLE SMALL OR MEDIUM SIZE
ANEURYSM
Pharmacologic Therapy
Low dose aspirin until regression documented
Physical Activity
None beyond 1st 6-8 weeks in patients <11 y.o.
11-20 y.o.: Restrictions based on biennial stress
test/myocardial perfusion scan
Contact/high-impact discouraged if taking anti-plt drugs
Invasive testing
Angiography if suggestion of ischemia
IV. ANEURYSMS WITHOUT STENOSIS
Pharmacologic Therapy
Long term aspirin
Long-term antiplatelet tx & warfarin or low molecular heparin (LMWH).
Physical Activity
Restrictions based on stress test/myocardial perfusion scan
Contact/high-impact avoided due to risk of bleeding
Follow-up and diagnostic testing
Biannual exam, echo, EKG
Annual stress test/myocardial perfusion scan
Invasive testing
Angiography @ 6-12 months, sooner/repeated if clinically indicated
Elective repeat in certain circumstances
V. OBSTRUCTION
Pharmacologic Therapy
Long-term low-dose aspirin, ± warfarin or LMWH if giant aneurysm
persists
Consider ß-blockade to reduce myocardial O2 consumption
Physical Activity
No contact or high impact sports
Other activity guided by stress testing or perfusion scan
Follow-up and diagnostic testing
Biannual exam, echo and EKG
Annual stress test/myocardial perfusion scan
Invasive testing
Angiography indicated to assess lesions and guide therapy. Repeat
angiography with change in symptoms.
THANK YOU