BACKGROUND: This study is a single-center experience with surgical repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with focus on the management of associated mitral regurgitation (MR). METHODS: We performed a retrospective analysis of cases presenting to a quaternary referral center between November 1990 and October 2011. RESULTS: In all, 25 patients (18 female) presented with a diagnosis of ALCAPA at a median age of 5 months (range, 1.5 to 102). Twenty-one patients (84%) had moderate to severe impairment of left ventricular function with median fractional shortening of 14% (range, 2% to 33%), and 19 patients (76%) had moderate to severe MR. Surgery was performed with direct coronary reimplantation in 16 patients (64%) and intrapulmonary tunnel (Takeuchi repair) in 9 (36%). Four patients had mitral valve repair at time of surgery, all for structural anomalies. Functional MR with a structurally normal mitral valve was not repaired. The median duration of postoperative follow-up was 93 months (range, 9 to 240). There were no early or late deaths, and no patient required mechanical support. Four patients (16%) required surgical or catheter reintervention. At last follow-up, 24 of 25 patients were asymptomatic; the left ventricular function was normal in 22 patients. Moderate MR was present in 4 patients. There was significant improvement in left ventricular function and MR (p < 0.01) during follow-up. CONCLUSIONS: Surgical repair of ALCAPA has good long-term results with low mortality and reintervention rates. The majority of MR is functional and will improve with reperfusion, but structural mitral valve abnormalities should be repaired at the time of surgery.
BACKGROUND: This study is a single-center experience with surgical repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with focus on the management of associated mitral regurgitation (MR). METHODS: We performed a retrospective analysis of cases presenting to a quaternary referral center between November 1990 and October 2011. RESULTS: In all, 25 patients (18 female) presented with a diagnosis of ALCAPA at a median age of 5 months (range, 1.5 to 102). Twenty-one patients (84%) had moderate to severe impairment of left ventricular function with median fractional shortening of 14% (range, 2% to 33%), and 19 patients (76%) had moderate to severe MR. Surgery was performed with direct coronary reimplantation in 16 patients (64%) and intrapulmonary tunnel (Takeuchi repair) in 9 (36%). Four patients had mitral valve repair at time of surgery, all for structural anomalies. Functional MR with a structurally normal mitral valve was not repaired. The median duration of postoperative follow-up was 93 months (range, 9 to 240). There were no early or late deaths, and no patient required mechanical support. Four patients (16%) required surgical or catheter reintervention. At last follow-up, 24 of 25 patients were asymptomatic; the left ventricular function was normal in 22 patients. Moderate MR was present in 4 patients. There was significant improvement in left ventricular function and MR (p < 0.01) during follow-up. CONCLUSIONS: Surgical repair of ALCAPA has good long-term results with low mortality and reintervention rates. The majority of MR is functional and will improve with reperfusion, but structural mitral valve abnormalities should be repaired at the time of surgery.