Objectives: An anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, often associated with severely impaired left ventricular (LV) contractility and functional mitral valve (MV) regurgitation. Current data suggest that earlier correction of ALCAPA may result in a more complete recovery of LV function. By analysing the results of a large single-centre ALCAPA cohort, we sought to investigate whether these treatment paradigms remain valid.
Methods: A retrospective study was performed evaluating all patients undergoing repair of ALCAPA over a period of almost 40 years. All preoperative and postoperative echocardiographic reports were reviewed, focusing on the recovery of LV and MV function.
Results: The study cohort included 78 patients who underwent ALCAPA repair between 1977 and 2015, who were divided into 2 groups based on patient age at initial repair: Group A (n = 52, age <1 year) and Group B (n = 26, age >1 year). Following repair, systolic LV and MV function improved significantly (P < 0.01) in both groups. Patient age at the time of initial surgery had no significant influence on the improvement of LV function. Early mortality (within 30 days) was 10% (n = 8). No 30-day mortality was reported in the past 20 years. Survival at 20 years following ALCAPA repair was 86 ± 4%.
Conclusions: Following ALCAPA repair, LV function significantly improved, regardless of age at the time of repair. In addition, preoperative functional MV regurgitation decreased over time. Concomitant mitral valve surgery at the time of ALCAPA repair is required in patients with structural abnormalities of the MV.