A 38-year-old male presented to the hospital with fatigue and progressive exertional dyspnea. He had a childhood history of Kawasaki disease (KD) with no treatment or subsequent follow up. Physical exam revealed marked volume overload, JVD, bibasilar crackles, and an S3 at the left ventricular apex. EKG was unremarkable. Chest x-ray showed pulmonary congestion and an enlarged cardiac silhouette. Transthoracic echocardiography revealed severe global hypokinesis, ejection fraction of 20-25%, and dilated cardiomyopathy. He was admitted for medical management of acute systolic heart failure.
Overnight, the patient developed substernal chest pain with uptrending troponins. EKG demonstrated ST elevations in precordial leads, and reciprocal depressions in the inferior leads. Left heart catheterization revealed large coronary artery aneurysms (CAA) with variable stenosis.
Following cardiac catheterization, the patient was started on anticoagulation therapy and aggressive diuresis with marked clinical improvement. He was discharged with long-term warfarin, carvedilol, lisinopril, atorvastatin, and aspirin. In addition, he was encouraged to undergo serial echocardiographic screening to prevent future cardiac events. He was also evaluated for insertion of an implantable cardioverter defibrillator for primary prevention of sudden cardiac death.
Discussion:
KD is an acute, self-limited viral illness and vasculitis of childhood, which is the most common cause of acquired heart disease of children in the developing world. It is a rare but important cause of premature ischemic heart disease in adults. The vasculitis leads to the development of CAA, thrombosis, and early myocardial dysfunction—all of which can cause morbidity and premature mortality. About 20-25% of survivors of untreated KD will develop CAA, with half of these at increased risk for future ischemic events. Even if our patient was treated with intravenous immunoglobulin and aspirin as a child, there still remained a 5% chance of developing CAA and cardiovascular sequlae.
The pathophysiology of CAA formation is mediated by endothelial cell dysfunction, intimal weakening, and smooth muscle cell reconstruction followed by fibrosis and calcification. Turbulent flow through the aneurysmal arteries increases the risk of thrombus formation and stenosis. Treatment of CAA includes early revascularization and advanced imaging to delineate coronary artery anatomy. The mainstay therapy to prevent acute coronary syndrome is antithrombotic agents.
Conclusions:
Kawasaki disease can have a myriad of cardiac complications in adults, most commonly coronary artery aneurysms. Though most patients present with acute coronary syndrome, acute systolic heart failure as seen in our patient is also possible, and clinicians need to be cognizant of it. A high index of suspicion for CAA in patients with a history of KD is crucial, as anticoagulation therapy and early intervention can improve clinical outcomes.