75 - SUDDEN UNEXPECTED DEATH IN CHILDREN AND YOUNG ADULTS DUE TO KAWASAKI DISEASE IN CHILDHOOD

Background: In California, unexpected deaths outside a medical setting require an autopsy. We collaborated with the San Diego County Medical Examiner’s Office (SDCMEO) to determine if children and young adults ( < 35 yrs) with known or missed Kawasaki disease (KD) were among those with sudden, unexpected death from cardiovascular causes.

Methods: From 2013 to 2022, there were 219 cases age 0-35 yrs with a cardiovascular (CV) cause of death who were autopsied at the SDCMEO. We excluded 76 cases due to the following diagnoses: suicide, homicide, cancer, drug/alcohol abuse, congenital heart disease, morbid obesity, spontaneous coronary artery dissection, collagen vascular disease, and diabetes. The remaining causes of CV death were: arrhythmia, atherosclerosis, cardiomyopathy, hypertensive CV disease, myocardial infarction, and myocarditis. We further excluded 30 cases attributed to arrhythmia who had no pathologic findings in the heart. Autopsy reports will be screened for gross and histologic findings suggestive of post-KD vascular damage including calcification, recanalized thrombus, and thrombosed aneurysms.

Results: We identified 113 cases who met the inclusion and exclusion criteria. 20 cases reviewed, 2 cases in progress of gross and histologic review for suspicion of missed KD. Review of 93 autopsy reports is in progress. Although the case was outside the age range for our study but met inclusion criteria, the SDCMEO contacted us regarding a 42 yr old woman whose cause of death was myocardial infarction. We reviewed the autopsy report and histology that revealed thrombosed giant coronary artery aneurysms that are pathognomonic of KD. We interviewed the decedent’s mother who described a prolonged febrile illness requiring hospitalization at age 19 yrs. for which there was no final diagnosis. The clinical description met criteria for KD.

Conclusions: The diagnosis of KD continues to be missed and individuals suffering from the complications of coronary artery aneurysms may only be diagnosed at autopsy.