Michiaki Imamura1, Amy M Dossey, Robert D B Jaquiss. 1. Division of Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital, Little Rock, Arkansas 72202, USA. imamuramichiaki@uams.edu
Abstract
BACKGROUND: Although outcomes for repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) have improved, early postoperative mechanical circulatory support is occasionally still required. This study was undertaken to determine whether long-term outcomes for children supported with extracorporeal membrane oxygenation (ECMO) after ALCAPA repair differ from those in children who did not require ECMO. METHODS: Between 1989 (when our ECMO program began) and 2010, 26 consecutive patients (median age of 0.26 years) underwent surgical repair of ALCAPA mainly with a strategy to produce a dual coronary system. Among the 26 patients, 21 did not require ECMO postoperatively (non-ECMO group) and 5 were supported by ECMO (ECMO group). Hospital and clinic records were reviewed to determine endpoints of early or late death, cardiac transplantation, and late reoperation. RESULTS: There were no early or late deaths in either study group, at a mean follow-up of 6.5±6.5 years. Mean duration of ECMO support was 10.7±6.7 days. There was no difference in age or weight between the two groups. Two patients, one in each group, required cardiac transplantation at 6 days and 21 months, respectively. Four other patients required 6 reoperations (5 for mitral regurgitation and 1 for an atrial septal defect with pulmonary stenosis). Actuarial freedom from cardiac transplantation or reoperation at 5 years was 0% in the ECMO group and 92% in the non-ECMO group (p<0.001; log-rank test). CONCLUSIONS: Overall survival is excellent after ALCAPA repair. However, those patients who require mechanical support after repair appear to be at higher risk for transplantation or reoperation, typically for mitral regurgitation.
BACKGROUND: Although outcomes for repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) have improved, early postoperative mechanical circulatory support is occasionally still required. This study was undertaken to determine whether long-term outcomes for children supported with extracorporeal membrane oxygenation (ECMO) after ALCAPA repair differ from those in children who did not require ECMO. METHODS: Between 1989 (when our ECMO program began) and 2010, 26 consecutive patients (median age of 0.26 years) underwent surgical repair of ALCAPA mainly with a strategy to produce a dual coronary system. Among the 26 patients, 21 did not require ECMO postoperatively (non-ECMO group) and 5 were supported by ECMO (ECMO group). Hospital and clinic records were reviewed to determine endpoints of early or late death, cardiac transplantation, and late reoperation. RESULTS: There were no early or late deaths in either study group, at a mean follow-up of 6.5±6.5 years. Mean duration of ECMO support was 10.7±6.7 days. There was no difference in age or weight between the two groups. Two patients, one in each group, required cardiac transplantation at 6 days and 21 months, respectively. Four other patients required 6 reoperations (5 for mitral regurgitation and 1 for an atrial septal defect with pulmonary stenosis). Actuarial freedom from cardiac transplantation or reoperation at 5 years was 0% in the ECMO group and 92% in the non-ECMO group (p<0.001; log-rank test). CONCLUSIONS: Overall survival is excellent after ALCAPA repair. However, those patients who require mechanical support after repair appear to be at higher risk for transplantation or reoperation, typically for mitral regurgitation.