Kawasaki Disease: A Rare Cause of Sudden Cardiac Death

Background Kawasaki disease is an extremely rare cause of sudden cardiac death (SCD). Case A healthy 17-year-old male suffered ventricular fibrillation cardiac arrest while playing football. He underwent successful defibrillation. ECG showed sinus rhythm with left posterior fascicular block. Echocardiogram and cardiac MRI demonstrated normal biventricular function and no structural anomaly. Invasive coronary angiography after coronary CTA revealed severe focal stenosis of the proximal right coronary artery (RCA) proximal to a 9 mm aneurysm, as well as left main coronary artery calcification and an 8 mm aneurysm. Decision-making He underwent coronary artery bypass grafting (CABG) of the RCA via right internal mammary artery graft. A tortuous thready left anterior descending artery was managed medically. Young patients presenting with exertional SCD raise concern for multiple etiologies such as hypertrophic cardiomyopathy, catecholaminergic polymorphic ventricular tachycardia, anomalous coronary artery, and commotio cordis, among others. Considering the bilateral coronary artery aneurysms and severe RCA stenosis, a presumptive diagnosis of Kawasaki disease was made. Given this, CABG was performed and patient additionally underwent implantation of an automated defibrillator for secondary prevention of SCD. Conclusion Kawasaki disease is a rare cause of SCD. Assessment of coronary anatomy remains a cornerstone in evaluation of young individuals with SCD.