Anomalous Coronary Arteries: A Multicenter Pediatric Autopsy Study

Anomalous coronary arteries (ACAs) are a potential cause of myocardial ischemia resulting in arrhythmias, angina, infarction, and sudden death. We analyzed 7857 pediatric autopsy cases from four tertiary centers to gain an overall picture of the range and significance of ACAs within this pediatric autopsy population. The incidence of ACAs was 0.5%, with ectopic origin from the aorta (43%) being most frequent, followed by ACA arising from the pulmonary trunk (40%). The mean age at death was 2.2 years (4 h-14 years). ACAs were associated with other anomalies in 57% of cases, 43% of these being cardiac defects. Growth retardation was noted in 50% of the cases. Examination of the heart showed cardiomegaly in 92% of cases, which was marked (> 95 percentile) in 63%, but histologically apparent ischemic damage was seen in only 50% of cases. ACAs arising from the pulmonary trunk were associated with earlier death (1.4 years), more frequent cardiomegaly (100%), and more frequent myocardial scarring (92%) than other types of anomalies. Although the terminal presentation was sudden death in 45% of cases, ACAs associated with other cardiac defects presented mainly with problems related to the latter anomalies. The results highlight the need to examine coronary arteries carefully during the pediatric autopsy. Not only are the positions of the coronary artery ostia important, but also the variations in the angles of arterial take-off, initial courses through the aortic adventitia, subsequent courses, and presence of ostial ridges or membranes that may result in significant compromise of blood flow.