Multiple Myocardial Infarctions in a Young Adult with a History of Kawasaki’s Disease

Introduction: Kawasaki disease (KD) is one of the most common vasculitidities in children in the US with approximately 21 per 100,000 children hospitalized yearly. In children that go untreated, 20-25% develop long-term coronary artery aneurysms (CAA). Approximately 5-10% of treated children develop long-term CAA. Patients with long-term sequela have an increased risk of early onset coronary atherosclerotic disease and myocardial ischemia (MI) resulting in significant morbidity and mortality throughout adulthood that accounts for 5% of MI’s in adults under 40.

Case Description: J.B. is a 28 year old African American male with a history of KD diagnosed at age 10, treated with IVIG and steroids, complicated by coronary ectasia with multiple aneurysms and prior myocardial infarction at age 11. He presented with substernal chest pressure and numbness down his left arm and was found to have elevated cardiac biomarkers with no electrocardiogram changes concerning for a non-ST segment elevation MI. He was started on a heparin infusion and underwent cardiac catheterization. This revealed a 100% distal right coronary artery (RCA) blockage with collaterals, the 100% blockage of the first diagonal branch of the left anterior descending artery (LAD) with collaterals, and had severe aneurysmal dilatation of the left main artery, proximal LAD, proximal circumflex artery, and proximal mid RCA. A multidisciplinary risk/benefit discussion was held and it was decided to manage his heart disease medically rather than surgically. He was treated with 48 hours of heparin, his medications were optimized, and he was discharged home.

Discussion: KD is a common clinical entity in children, and these children eventually become adults. There is a growing body of evidence for increased long-term risk of heart disease in this population, even in patients without identified CAA. With the unique pathophysiology and early age this can occur the typical atherosclerotic risk management guidelines are not appropriate for this patient population. Despite an increase in this patient population, there is still a lack of evidence-based guidelines. The long-term risk of recurrent MI is also unknown, and the appropriate management following recurrent MI with CAA needs more investigation. While the American Heart Association updated their guidelines on long-term management of KD, there is still limited evidence for the treatment and monitoring of these patients.

Conclusion: KD is a common vasculitis in childhood that causes morbidity and mortality in adulthood. Adult primary care and cardiology providers will be increasingly involved in the management of these patients as the number of patients surviving into adulthood with KD continues to grow. It is important to understand the long-term needs of this young adult population and more research is needed in order to provide the appropriate management to help prevent the sequela of KD and improve patient outcomes.