Long-Term Results of Repair of Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery

Background Upon inital repair, most patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) present with severely impaired ventricular function and mitral regurgitation. In this study, both parameters were investigated at long-term in patients in whom either coronary transfer or subclavian artery anastomosis was applied. Methods Records of 56 patients with ALCAPA, operated between 1977 and 2002, were reviewed retrospectively. Patients were divided into two groups: subclavian artery anastomosis group (n = 25), and coronary transfer group (n = 31). Results Concomitant mitral valve repair was performed in one patient. Thirty-day mortality was 14.3% (subclavian artery anastomosis group, n = 3; coronary transfer group, n = 5). Preoperative age (p = 0.007) and left ventricular ejection fraction (LVEF) less than 0.35 (p = 0.020) were predictive for 30-day mortality, whereas preoperative mitral regurgitation, surgical technique, and cardiopulmonary bypass time were not. For both groups, LVEF (p = 0.006) and mitral regurgitation (p = 0.013) were improved at the time of hospital discharge. Mean follow-up was 11.0 ± 7.2 years. Survival of hospital survivors at 20 years was 94.8 ± 3.6%. At final follow-up, 95.5% of the patients presented with a LVEF greater than 0.50, and 84.1% with a mitral regurgitation less than grade 2. Mitral valve replacement was performed in three patients. Conclusions In the majority of patients with ALCAPA, both ventricular function and mitral valve regurgitation normalize over time. Concomitant mitral reconstruction may not be required upon initial repair. Coronary transfer is the technique of choice today. However, subclavian artery anastomosis may remain an option in selected cases.