Surgical correction of congenital left coronary artery-main pulmonary artery fistula in association with anomalous right coronary artery.

The association of a left coronary artery-main pulmonary artery fistula and an anomalous right coronary artery originating from the main pulmonary artery is the subject of this report. This unique combination of congenital cardiac anomalies establishes a double coronary steal from the left coronary artery, which hemodynamically represents the sole source of myocardial perfusion. The left coronary artery-main pulmonary artery fistula was closed and the coexisting anomalous right coronary artery reimplanted into the anterior aspect of the ascencing aorta. A dual coronary supply was therefore established and thus eliminated the potential threat of total myocardial ischemia should the left coronary artery become critically compromised. Patency of both the left coronary artery and the transplanted right coronary artery was documented 1 year postoperatively by aortic root angiography.