Medium-term outcome after anomalous aortic origin of a coronary artery repair in a pediatric cohort.

ABSTRACT

BACKGROUND: Anomalous aortic origin of a coronary artery with an interarterial and intramural course (AAOCA) is a rare anomaly with increased risk of sudden cardiac death during or just after exercise among otherwise healthy youth. Risk stratification and management remain controversial, especially for the asymptomatic child with an anomalous right coronary artery from the left coronary sinus (ARCA). Medium-term surgical and quality-of-life (QOL) outcome data are lacking in this population. METHODS: We performed medical record review on 24 subjects who underwent AAOCA repair between 2001 and 2007 at The Children's Hospital of Philadelphia. QOL was prospectively assessed with age-appropriate questionnaires. RESULTS: Median age at follow-up was 18 (range, 11-25) years, median follow-up from surgery was 63 (range, 12-110) months, and 16 (67%) had ARCA. All were alive without exercise restriction. Thirteen (54%) complained of cardiac-type symptoms postoperatively, most commonly chest pain, none correlating with evidence of ischemia on testing. Of the 13 patients, 7 (54%) reported the same symptoms preoperatively; and of these, 5 had ARCA. Postoperative morbidity occurred in 16 (67%), including pericardial effusion (n = 11), wound infection (n = 2), and development of mild aortic insufficiency (n = 4). QOL questionnaires were sent to 21 subjects; 12 (57%) were returned. Average QOL was normal for all subjects. CONCLUSIONS: In the medium-term after AAOCA repair, cardiac-type symptoms frequently persist and morbidity is common, but these do not impair QOL. The significance of these findings in the long-term is unknown and warrants continued follow-up.