Division of Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital, Little Rock, Arkansas 72202, USA.
Ann Thorac Surg. 2011 Jul;92(1):167-72; discussion 172-3. doi: 10.1016/j.athoracsur.2011.02.074. Epub 2011 May 18.
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.
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.
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).
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.
尽管异常起源左冠状动脉来自肺动脉(ALCAPA)修复的结果已经改善,但术后早期机械循环支持偶尔仍需提供。本研究旨在确定在 ALCAPA 修复后接受体外膜氧合(ECMO)支持的儿童与不需要 ECMO 的儿童的长期结果是否不同。
1989 年(我们的 ECMO 计划开始时)至 2010 年期间,26 例连续患者(中位年龄为 0.26 岁)接受了 ALCAPA 的手术修复,主要采用产生双冠状动脉系统的策略。在 26 例患者中,21 例术后不需要 ECMO(非 ECMO 组),5 例接受 ECMO 支持(ECMO 组)。回顾医院和诊所记录以确定早期或晚期死亡、心脏移植和晚期再次手术的终点。
两组均无早期或晚期死亡,平均随访 6.5±6.5 年。ECMO 支持的平均持续时间为 10.7±6.7 天。两组之间在年龄或体重方面没有差异。两组各有 1 例患者分别在第 6 天和第 21 个月需要心脏移植。其他 4 例患者需要 6 次手术(5 例为二尖瓣反流,1 例为房间隔缺损伴肺动脉狭窄)。ECMO 组和非 ECMO 组的 5 年心脏移植或再次手术的累积无事件生存率分别为 0%和 92%(p<0.001;对数秩检验)。
ALCAPA 修复后总体生存率良好。然而,那些在修复后需要机械支持的患者似乎移植或再次手术的风险更高,通常是二尖瓣反流。