Long-term outcome after surgical correction of anomalous left coronary artery from pulmonary artery

Objectives: Early establishment of a two-coronary artery system has become the standard surgical approach in patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). After surgical repair, presentation of severely impaired ventricular function and mitral regurgitation is a common finding. Methods: We reviewed long-term outcome of 18 consecutive patients with ALCAPA undergoing surgical repair for establishment of dual coronary system operated on between 1999 and 2009. Mortality, morbidity, echocardiography assessment of left ventricular function, mitral valve regurgitation, and indications for reoperation were studied retrospectively. Results: The mean age at the time of surgery was 26±44 months, There was no in-hospital or late mortality. In 5 patients perioperative left ventricular assist device was needed. 1 patient required reoperation 6 years postoperatively because of relevant proximal stenosis of the left main coronary artery, Recovery of left ventricular function was associated with improvement in the degree of mitral valve regurgitation. At the latest follow up, mitral valve regurgitation was non or trace in 14 patients, mild to moderate in 3 patients and remained severe in 1 patient. Left ventricular function normalized in 16 patients, and remained mildly impaired in 2 patients. Conclusions: Early establishment of a two-coronary artery system artery results in complete recovery of left ventricular function and without relevant mitral valve dysfunction. Re-operation rates are acceptable. Our results support the use of LVAD in patients with refractory low cardiac output. The necessary of mechanical circulatory support was short and very effective in our patient cohort.