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 dea th. We analyzed 7857 pediatric autopsy cases from four tertiary center s to gain an overall picture of the range and significance of ACAs wit hin this pediatric autopsy population. The incidence of ACAs was 0.5%, with ectopic origin from the aorta (43%) being most frequent, followe d 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 anomali es in 57% of cases, 43% of these being cardiac defects. Growth retarda tion was noted in 50% of the cases. Examination of the heart showed ca rdiomegaly in 92% of cases, which was marked (>95 percentile) in 63%, but histologically apparent ischemic damage was seen in only 50% of ca ses. ACAs arising from the pulmonary trunk were associated with earlie r death (1.4 years), more frequent cardiomegaly (100%), and more frequ ent myocardial scarring (92%) than other types of anomalies. Although the terminal presentation was sudden death in 45% of cases, ACAs assoc iated with other cardiac defects presented mainly with problems relate d to the latter anomalies. The results highlight the need to examine c oronary arteries carefully during the pediatric autopsy. Not only are the positions of the coronary artery ostia important, but also the var iations in the angles of arterial take-off, initial courses through th e aortic adventitia, subsequent courses, and presence of ostial-ridges or membranes that may result in significant compromise of blood flow.