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Ann Thorac Surg 2003;75:1535-1541
© 2003 The Society of Thoracic Surgeons

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Original article: cardiovascular

Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome

^a Departments of Surgery and Pediatrics, Divisions of Division ofCardiovascular Surgery, Toronto, Ontario, Canada
^b Division of Cardiology, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada
^c Department of Surgery and Pediatrics, Division of Pediatric Cardiac Surgery, University of California, San Francisco, San Francisco, California, USA

Accepted for publication November 11, 2002.

^* Address reprint requests to Dr Williams, Division of Cardiovascular Surgery, Hospital for Sick Children, 555 University Ave, Room 1525, Toronto, Ontario M5G-1X8, Canada.
e-mail: azakiet{at}surgery.ucsf.edu

BACKGROUND: To determine the early and late outcomes of patients presenting with anomalous left coronary artery from the pulmonary artery who had repair by aortic reimplantation.

METHODS: From January 1952 to July 2000, 67 patients presented with anomalous coronary artery from the pulmonary artery. Forty-seven patients who had repairs performed by aortic reimplantation are the subject of this study. The median age at repair was 7.7 months. Before repair, 10 infants (21%) presented in extremis requiring ventilatory and inotropic support, and 38 infants (80%) presented in heart failure. Autologous pericardial hood coronary arterioplasty was used in 4 patients, and concomitant mitral valve repair was used in 1 patient.

RESULTS: Hospital survival was 92%. Five children required postoperative extracorporeal membrane oxygenation for a median of 4 days (range, 2 to 8 days). Patients who had extracorporeal membrane oxygenation were significantly more likely to have presented in critical condition (40% vs 3% if no extracorporeal membrane oxygenation; p = 0.006) or with ventricular arrhythmias (67% vs 7%; p = 0.027), to have presented with significantly lower preoperative repair median ejection fraction (10%, n = 5 vs 40%, n = 38; p = 0.01) or to have presented with more severe left ventricular dilatation (p = 0.03). Within a 15-year or less follow-up (mean, 4.7 years) there were no late deaths. Kaplan-Meier survival was 91% at 5 years, and freedom from reoperation was 93% at 10 years. At late follow-up, echocardiography demonstrated significant improvements in mean ejection fraction (64% ± 9% vs 33% ± 21% preoperatively, p < 0.0001); moderate mitral regurgitation (9% vs 38% preoperatively, p < 0.02); and wall motion abnormalities (15% vs 81% preoperatively, p < 0.002). The ratio of measured left ventricular end-diastolic dimension to the 95th percentile of normal declined from 1.4 ± 0.3 to 1.0 ± 0.1 (p < 0.0006). Children who had extracorporeal membrane oxygenation had normal ejection fractions and ventricular dimensions at follow-up (n = 3). Repeated measures of mixed linear regression analysis demonstrated that normalization of ejection fraction and left ventricular function occurred within 1 year of repair. Improvements in mitral regurgitation lagged behind normalization of ejection fraction and left ventricular dilatation.

CONCLUSIONS: Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation yields excellent early survival and late functional outcomes even in critically ill infants.

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