Background. Anomalous aortic origin of a coronary artery (AAOCA) from an incorrect sinus of Valsalva is a relatively rare congenital defect and is associated with sudden death. Several surgical techniques have been described to address this defect, but functional outcome has never been addressed. In this report we evaluate a consecutive series of patients treated with unroofing techniques using transthoracic echocardiography, graded exercise testing, and stress echocardiography to assess functional repair. Methods. Nine consecutive patients (range 7 to 65 years) underwent surgical repair of AAOCA from 1995 to 2001. In all patients the intramural segment was at or below the level of the commissure. All patients underwent a modified unroofing procedure to move the coronary artery orifice to the appropriate sinus. In 2 patients, a new orifice was created without significant unroofing and disruption of the commissure. Patients were evaluated prospectively with exercise electrocardiography testing and by resting and stress echocardiography. Results. Of the 9 patients, 8 presented with symptoms suggestive of ischemia (chest pain, dyspnea on exertion, or syncope). Six patients had anomalous left main coronary artery arising from the right sinus of Valsalva, and 3 patients had anomalous right coronary artery from the left sinus of Valsalva. Transthoracic echocardiography and graded exercise testing was performed in all 9 patients (mean 29 months, range 4 to 85 months) after repair. Of the 9 patients, 8 also underwent stress echocardiography. In 8 of 9 patients the newly created coronary artery ostium was visualized by either two-dimensional echocardiography or color flow Doppler. All patients were symptom free at the time of follow-up. Exercise stress echocardiography was negative in all patients. Of the 8 patients, 7 had normal left ventricular shortening. No patients had regional wall motion abnormalities suggestive of ischemia. All patients were intervention free except 1 patient who developed severe aortic insufficiency and underwent a subsequent Ross procedure 44 months after his initial procedure. Conclusions. Anomalous origin of a coronary artery from an incorrect sinus of Valsalva is known to be associated with increased risk of sudden death. Surgical correction can be carried out with minimal risk and good anatomic and functional results. Manipulation of the commissure can be avoided by creation of a neo-ostia without extensive unroofing of the intramural segment or manipulation of the intercoronary commissure. This may avoid aortic valve malfunction. © 2003 by The Society of Thoracic Surgeons.