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coronary arteries may be detected by echocardiography as early as 6 days after the
onset of fever and usually peaks 3 or 4 weeks into the course of the illness.
Coronary aneurysms in early KD usually occur in the proximal segments of the
major coronary vessels; abnormalities that occur distally are almost always associated
with proximal coronary dilatation. Aneurysms may also occur in arteries outside the
coronary system, most commonly the subclavian, brachial, axillary, iliac, or femoral
vessels, and occasionally in the abdominal aorta and renal arteries. It is reasonable to
obtain advanced imaging studies in select patients, but they are not routinely indicated
for diagnosis and management of acute illness. Transesophageal echocardiography,
computed tomographic angiography (CTA), and cardiac MRI can be useful in older
patients if visualization of the coronary arteries with echocardiogram is not adequate.
Patients with CAA may require additional antithrombotic therapy beyond aspirin, and
this is determined by the size of the coronary artery abnormality. Patients with
large/giant coronary aneurysms may require multiple antithrombotic medications, and
should receive anticoagulation treatment in consultation with a cardiologist.
Myocardial Disease. Myocardial infarction caused by thrombotic occlusion of an
aneurysmal and/or stenotic coronary artery is the principal cause of death in KD.
Patients with giant CAA (Z score ≥10 or absolute dimension ≥8 mm) have the most
serious long-term consequences. Rarely, dilated and weakened coronary arteries may
rupture. Mortality due to KD has decreased from almost 2% to <0.1% as a result of
improved treatment. Nonetheless, most deaths continue to occur during the first 6
months after disease onset, when myocardial and coronary artery inflammation are
greatest.
If an acute ST-elevation myocardial infarction (STEMI) occurs in a KD patient
beyond the acute phase of the illness, immediate angiography with restoration of blood
flow is indicated in patients who are large enough for adult catheters. If patients are not
candidates for mechanical restoration of flow (in the acute phase of disease or if the
child is not large enough in size), then “triple therapy” with systemic recombinant
tissue plasminogen activator (alteplase), low-dose aspirin, and heparin are indicated.
Maintenance of reperfusion then requires chronic antithrombotic therapy (e.g.,
warfarin, low–molecular-weight heparin), although the ideal regimen has not been

established. For patients with a large thrombus, treatment with reduced dose
thrombolytic therapy and abciximab (glycoprotein IIb/IIIa inhibitor) may be used.
After an acute myocardial infarction, the following therapies may be indicated: beta
blockers, (ACE) inhibitors, or angiotensin II receptor blockers, and aldosterone
blockers. These medications are used in addition to an antithrombotic regimen. In
select patients with signs of coronary ischemia, the following procedures may be
indicated: percutaneous coronary intervention or coronary artery bypass grafting.
Rarely, cardiac transplantation may be indicated for the patient with end-stage ischemic
cardiomyopathy.