Sudden death in an infant revealing atypical Kawasaki disease

We present a case of a 4-month-old girl referred to the emergency department with a provisional diagnosis of acute life-threatening event with a recent episode of heart block and a history of long-lasting fever. Soon after admission, the child suddenly deteriorated rapidly; she became pulseless with complete heart block and died despite intensive resuscitation efforts. Postmortem examination showed coronary arteritis with aneurysmal dilatation and coronary thrombosis, revealing atypical Kawasaki disease. With this case presentation, we discuss the importance of early recognition and treatment of atypical and/or incomplete forms of Kawasaki disease, which are most common in young infants and may lead, if untreated, to coronary artery abnormalities with a potential for myocardial infarctions, aneurysm formation, and sudden death. In addition, the relevance of postmortem examination in a case of sudden and undiagnosed infant death is underlined.