Sudden Death Due to Ruptured Three-Vessel Giant Coronary Artery Aneurysm in a 28 Year Old Woman with Trisomy 21

We report a case of sudden death due to rupture of a giant right coronary artery aneurysm, likely a sequela of remote Kawasaki disease, in a young woman with Down syndrome. The decedent was a 28 year old female with trisomy 21 who was found deceased in the backyard pool. An autopsy disclosed an enlarged heart with 400 ml of clotted blood in the pericardial sac and undiagnosed giant coronary artery aneurysms involving the left anterior descending, left circumflex, and right coronary artery, with rupture of the latter artery. The histologic findings were consistent with remote Kawasaki disease. Giant coronary artery aneurysms are a rare complication of Kawasaki disease, infectious vasculitis, and atherosclerosis, among other causes. While Kawasaki disease is typically considered a disease of children, late-onset complications should be considered in the differential diagnosis of young adults with myocardial infarction, coronary aneurysm, or sudden death.