Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University School of Medicine, Stanford, CA 94305, USA.
Eur J Cardiothorac Surg. 2012 Aug;42(2):235-40; discussion 240-1. doi: 10.1093/ejcts/ezr310. Epub 2012 Mar 7.
Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.
This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).
There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.
The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.
主-肺动脉侧支循环（PA/MAPCAs）合并肺动脉瓣闭锁是一种复杂的先天性心脏病，在过去 15 年中，其治疗方法取得了重大进展。一小部分 PA/MAPCAs 患者合并有 Alagille 综合征，这会对许多其他器官系统产生不良影响。本研究旨在回顾我们机构内 PA/MAPCAs 合并 Alagille 综合征患者的手术治疗结果。
这是一项回顾性研究，纳入了 2001 年 11 月至 2011 年 8 月期间接受手术重建的合并有 PA/MAPCA 和 Alagille 综合征的患者。我们的数据库中确定了 15 例患者。其中 13 例为伴有室间隔缺损的肺动脉瓣闭锁（PA/VSD），2 例为伴有完整室间隔的肺动脉瓣闭锁（PA-IVS）。
在这 15 例合并有 PA/MAPCA 和 Alagille 综合征的患者中，没有出现早期或晚期死亡。这些患者共接受了 38 次心脏外科手术。13 例伴有 PA/VSD 的患者中，有 10 例实现了完全修复，包括单心室化、右心室到肺动脉的管道以及所有心内分流的关闭。未修复的 3 例伴有 PA/VSD 的患者仍有可能最终进行完全修复，而 2 例伴有 PA-IVS 的患者仍适合单心室途径。该系列患者还接受了 12 次主要的非心脏手术。
数据表明，在 Alagille 综合征患者中，PA/MAPCAs 的手术重建是可以成功实现的。基于 Alagille 综合征的多器官系统受累，其长期预后仍不容乐观。
