Characteristics of Sudden Cardiac Death Late After Acute Kawasaki Disease

Abstract

When present, giant aneurysms typically occlude coronary arteries within the first year after onset of Kawasaki disease (KD) and can cause acute myocardial infarction. Bilateral giant aneurysm is major risk factor for sudden death after KD and leads to myocardial infarction, decreased left ventricular ejection fraction, multifocal premature ventricular contractions, and asymptomatic nonsustained ventricular tachycardia. Prevention of myocardial infarction and left ventricular (LV) dysfunction depends on careful follow-up, anticoagulation therapy, and coronary revascularization. LV dysfunction predisposes patients to arrhythmic sudden death; however, angiotensin-converting enzyme inhibitors and beta-blockers improve prognosis for patients with LV dysfunction. Such patients may also benefit from antiarrhythmic treatment with amiodarone or sotalol. If critical ventricular tachycardia is detected, catheter ablation or an implantable cardioverter–defibrillator should be considered. Sudden death can occur more than 20 years after acute KD in patients with LV dysfunction (left ventricular ejection fraction <40 %). Multifocal premature ventricular contractions and nonsustained ventricular tachycardia are probable risk factors in such patients.