Anomalous left coronary artery from the right pulmonary artery with an intramural course

Anomalous left coronary artery from the right pulmonary artery (RPA) is a rare congenital coronary anomaly and is one of the surgically treatable causes of ventricular dysfunction in infants. The left coronary artery when it arises from the RPA or near its base tends to follow the intramural course. Careful echocardiographic evaluation of the course of the coronary artery is necessary under sedation to avoid missing this anomaly. Unroofing of this coronary artery and closing of the pulmonary artery origin are recommended for treatment. We report a case where the intramural course was retrocommissural and unroofing would have resulted in aortic incompetence. We describe how a 90° rotation is possible by augmenting the coronary button with an anterior pericardial hood. The reconstruction of the RPA should be done with adequate mobilization and redundancy to prevent compression and bowstringing of the reimplanted coronary artery.