Background/Purpose: Coronary artery aneurysms (CAA) may remain silent after Kawasaki disease (KD) until adulthood when myocardial ischemia can lead to sudden death. CAA attributed to antecedent KD were present in 5% of young adults (<40 years (yrs)) evaluated for symptoms of myocardial ischemia by coronary angiography (Daniels et al. 2012). We postulated that there would be young adults with sudden, unexpected death due to CAA from KD who would have a state-mandated autopsy performed by the Medical Examiner (ME).

Methods: We systematically reviewed all autopsy cases < 35 yrs of age from 1997-2012 at the San Diego County ME Office with a cardiovascular cause of death. Cases were excluded if another medical condition was listed as a contributing factor to death including trauma, substance abuse, suicide, cancer, congenital heart disease, morbid obesity, diabetes, hypertension, or hyperlipidemia. We reviewed the ME records for demographic data, clinical history, and gross and histologic autopsy findings. For the two cases adjudicated as KD, the parents of the decedents were interviewed following written informed consent.

Results: There were 154 cases that met inclusion criteria and122 cases (80%) met exclusion criteria. Of the 32 cases reviewed, two (6.25%) had CAA described in the autopsy report. Case 1 was a 30-yr old Hispanic male with no significant medical history or cardiovascular risk factors who collapsed after a boxing work out. Cause of death was myocardial infarction due to thrombosis of the left anterior descending (LAD) aneurysm. Histologic findings included myocardial fibrosis a re-canalized aneurysm in right coronary artery (RCA) with multiple lumens, and calcification of the LAD aneurysm wall with thinned media and organized and acute thrombus. Interview of the parents revealed a possible KD-compatible illness in childhood diagnosed as scarlet fever. Case 2 was a 22-yr old Korean male who collapsed during vigorous exercise. Sudden cardiac death was associated with chronic ischemic changes due to a partially occluded 15mm aneurysm at the bifurcation of the LMCA and LAD with diffuse calcification. There were two 6mm aneurysms of the RCA. There was patchy fibrosis throughout the myocardium. Interview of the mother revealed that this patient had been diagnosed with KD complicated by giant aneurysms at age 2.75 yrs. He had been maintained on aspirin therapy under the care of a cardiologist until he left home at age 20 yrs. Detailed immunohistochemical studies of these two cases are pending.

Conclusion: In an ME office serving a population of approximately 3 million people, 2/154 (1.3%) of cardiovascular deaths in persons <35 yrs could be attributed to CV complications of KD in childhood. Pathologic findings that support a diagnosis of antecedent KD include aneurysms with or without thrombosis,  recanalization, or stenosis, luminal myofibroblastic proliferation, and CA wall calcification. Antecedent KD leading to myocardial ischemia or thrombosed aneurysms should be considered in the evaluation of all cases of sudden, unexpected death in young adults.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/systematic-analysis-and-pathologic-findings-in-young-adults-with-sudden-death-attributed-to-kawasaki-disease-in-childhood/