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Anomalous origin of the left coronary artery from the right pulmonary artery associated with ventricular septal defect.

A three-year-old girl with ventricular septal defect and reactive pulmonary hypertension died immediately after surgical closure of the ventricular septal defect. Necropsy revealed an associated anomalous origin of the left coronary artery from the right pulmonary artery. It is assumed that the pulmonary arterial pressure decreased from systemic levels upon closure of the ventricular septal defect. The fall in pressure, in turn, is considered to have been responsible for a fall in left coronary arterial perfusion pressure. In the absence of rich intercoronary anastomoses, myocardial ischemia and death resulted. The case seems to emphasize the point that classic features of anomalous origin of the left coronary artery from the pulmonary arterial system may be absent if pulmonary hypertension for any reason is present.