Objectives: We reviewed our institutional experience with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) after dual coronary repair to assess preoperative variables predictive of outcome, the time course for postoperative recovery of cardiac function, the short- and long-term complications and our experience with left ventricular assist devices (LVAD) in these patients.
Background: Outcome after surgical repair of ALCAPA remains incompletely defined.
Methods: The surgical records and echocardiograms of 42 patients were reviewed. Left ventricular function was assessed by fractional shortening z-score (FSz) and stress-velocity index.
Results: The overall survival rate was 86%. All six patients who died were < 1 year old and died within 3 days of the operation. More severe preoperative mitral regurgitation (MR) was associated with increased mortality, but age, body surface area, preoperative FSz and end-diastolic dimension were not. We used an LVAD for 7 of 28 patients who underwent repair for ALCAPA since its introduction at our institution, with a survival of 5 of 7 patients. The degree of MR improved in 62% of patients and remained unchanged in 38%. Complications included supravalvar pulmonary stenosis (16 of 21 patients) and baffle leaks (11 of 21 patients) with the intrapulmonary baffling technique. Supravalvar pulmonary stenosis developed in 1 of 11 patients after direct coronary reimplantation. Left ventricular function became normalized in all 28 patients with follow-up past 1 year, regardless of preoperative FSz. Of 13 patients who underwent serial postoperative echocardiography, the average time to normalization of function was 2 to 7 months.
Conclusions: The degree of preoperative MR was predictive of outcome, whereas the severity of preoperative cardiac dysfunction and ventricular dilation were not. Mild and moderate MR tended to improve without mitral valvuloplasty. Complete recovery from myocardial dysfunction is expected after dual coronary repair of ALCAPA.