Ten-year experience with surgical unroofing of anomalous aortic origin of a coronary artery from the opposite sinus with an interarterial course

Background Anomalous aortic origin of a coronary artery from the opposite sinus with an intramural course between the great arteries (AAOCA) is associated with ischemia and sudden cardiac death in children, and surgical unroofing has been used to alleviate that risk. Methods The cardiology database was reviewed to identify all patients with AAOCA who underwent surgical unroofing 10 years. Results From March 1999 to September 2009, 27 patients with a mean age of 12.6 ± 3.5 years (range, 4–20 years) had surgical unroofing of AAOCA of the left coronary from the right sinus (left AAOCA, 7/27, 26%) or of the right coronary from the left sinus (right AAOCA, 20/27, 74%). Of these, 26 had diagnoses made by transthoracic echocardiography. Symptoms included resuscitated sudden cardiac death in 3, syncope in 8, and chest pain in 4. No symptoms were present in 12 cases of serendipitous diagnosis. Unroofing of the intramural portion was successfully performed in all cases. A slitlike coronary orifice was described at surgical inspection in 12 patients, 7 of whom had right AAOCA and no symptoms. All patients after unroofing have patent coronary flow by Doppler and normal echocardiography and exercise treadmill testing at mean follow-up of 1.8 years. None have activity restrictions. Conclusions AAOCA is frequently characterized by an intramural course, which can be reliably identified by echocardiography. This form can be safely repaired by unroofing the intramural segment without early morbidity. Symptoms of possible ischemia are common but not always correlated with coronary ostial findings at surgery.