Cardiovascular pathology in 2 young adults with sudden, unexpected death due to coronary aneurysms from Kawasaki disease in childhood

• Published in: Cardiovascular pathology Vol. 24; no. 5; pp. 310 - 316
• Main Authors: Shimizu, Chisato, Sood, Alka, Lau, Hubert D, Oharaseki, Toshiaki, Takahashi, Kei, Krous, Henry F, Campman, Steven, Burns, Jane C
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-09-2015
• Subjects: Adult; Autopsy; Coronary Aneurysm - etiology; Coronary Aneurysm - pathology; Coronary artery aneurysm; Death, Sudden, Cardiac - etiology; Death, Sudden, Cardiac - pathology; Humans; Kawasaki disease; Male; Mucocutaneous Lymph Node Syndrome - complications; Myocardial fibrosis; Pathology; Sudden death; Transforming growth factor beta; Young Adult; CAA; Myocardial fibrosis; SDCMEO; Sudden death; IVIG; Transforming growth factor beta; coronary artery aneurysm; Kawasaki disease; transforming growth factor-β; Autopsy; San Diego County Medical Examiner’s Office; KD; TGF-β; intravenous immunoglobulin; Coronary artery aneurysm
• DOI: 10.1016/j.carpath.2015.02.006
• EISSN: 1879-1336
• ISSN: 1054-8807

Abstract Purpose Coronary artery aneurysms (CAA) may remain silent after Kawasaki disease (KD) until adulthood when myocardial ischemia can lead to sudden death. 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 San Diego County Medical Examiner’s Office (SDCMEO). Methods We reviewed all autopsy cases < 35 years of age from 1997 to 2012 at the SDCMEO with a cardiovascular cause of death (n = 154). Results We found 2 cases meeting inclusion criteria. Case 1 was a 22-year-old Korean male with chronic ischemic changes due to a partially occluded and diffusely calcified 15 mm aneurysm at the bifurcation of the left main coronary artery. Interview of the mother revealed that this patient had been diagnosed with KD complicated by giant aneurysms at age two years. Case 2 was a 30-year-old Hispanic male with myocardial infarction due to thrombosis of a calcified left anterior descending artery aneurysm. Histologic findings included diffuse myocardial fibrosis and a recanalized aneurysm in the right coronary artery. Interview of the family revealed a KD-compatible illness in childhood. Immunohistochemical staining showed expression of transforming growth factor β pathway molecules in the aneurysmal arterial wall. Conclusions In a medical examiner’s office serving a population of approximately 3 million people, 2 of 154 (1.3%) cardiovascular deaths in persons < 35 years were attributed to cardiovascular complications of KD in childhood. Antecedent KD should be considered in the evaluation of all cases of sudden, unexpected death in young adults.