Department of Cardiothoracic Surgery, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China.
Chin Med J (Engl). 2010 Feb 5;123(3):259-64.
Pulmonary atresia with ventricular septal defect (PA-VSD) and major aortopulmonary collateral arteries (MAPCAs) remains a challenging complex congenital heart disease nowadays. In the present study, we aimed to develop a two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and MAPCAs.
Between December 2003 and December 2008, 7 female and 4 male patients between the age of 5 and 10 years who were suffering from PA-VSD and MAPCAs were selected and recruited. The native pulmonary artery trunks were absent in all patients; the lungs were solely supplied by major aortopulmonary collaterals, and the numbers of supplied lung segments ranged from 15 to 20 (17.9 +/- 1.6). There were a total of 43 MAPCAs in all the patients (3 - 5 (3.9 +/- 0.7) MAPCAs per patient). The accumulated Nakata index was (222.9 +/- 29.9) mm(2)/m(2) (ranged from 182 to 272). All the patients underwent two sequential operations. Stage one included left major aortopulmonary collateral unifocalization and modified Blalock-Taussig shunt from left posterior lateral thoracotomy; stage two comprised right unifocalization, ligation of the shunt, followed by ventricular septal defect closure and right ventricular outflow tract reconstruction assisted with cardiopulmonary bypass from midline sternotomy.
All the patients survived the initial surgery, but one of them died of low cardiac output syndrome on the third day after the second operation. Postoperative complications included pneumonia in one case and capillary leak syndrome in another. Postoperative oxygen saturation maintained about 95% - 100%, which was significantly higher than pre-operation (P < 0.01). During the follow-up period of 3 - 51 (25.4 +/- 15.2) months, there were no late death and no need for re-intervention. All the patients enjoyed their lives with good conditions.
This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative surgical approach in the treatment of PA-VSD and MAPCAs.
肺动脉瓣闭锁伴室间隔缺损（PA-VSD）合并主-肺动脉侧支循环（MAPCAs）仍然是当今具有挑战性的复杂先天性心脏病。本研究旨在提出一种两阶段的外科治疗方法，并评价其治疗 PA-VSD 和 MAPCAs 的效果。
2003 年 12 月至 2008 年 12 月，7 例女性和 4 例男性患儿，年龄 5-10 岁，均患有 PA-VSD 和 MAPCAs。所有患儿的主肺动脉干均缺如，仅由 MAPCAs 供血，供应肺段数 15-20 个（17.9+/-1.6）。共 43 支 MAPCAs（每例 3-5 支，3.9+/-0.7）。全组患儿的心脏指数（CI）为 222.9+/-29.9mm2/m2（182-272）。所有患儿均接受两期手术治疗。一期手术：经左后外侧开胸行左主-肺动脉侧支共干化+改良 Blalock-Taussig 分流术；二期手术：经正中开胸行右主-肺动脉侧支共干化+分流结扎+室间隔缺损修补+右心室流出道重建，术中体外循环辅助。
所有患儿均存活至一期手术，其中 1 例患儿二期手术后第 3 天死于低心排血量综合征。术后并发症：肺炎 1 例，毛细血管渗漏综合征 1 例。术后氧饱和度较术前明显升高（P<0.01），维持在 95%-100%。随访 3-51 个月（25.4+/-15.2），无晚期死亡及再次干预。所有患儿生活质量良好。
两期完全纠治策略安全、有效，可获得良好的近中期效果，是治疗 PA-VSD 和 MAPCAs 的有效方法。
