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目的探讨伴室间隔缺损的肺动脉闭锁(VSD-PA)的外科治疗的手术时机、适应证和方法。方法 1984年6月至2005年3月对32例 VSD-PA 进行外科手术治疗33例次,32例中男15例、女17例,年龄6个月～9岁,其中合并主动脉到肺的侧支循环动脉(APCA)的9例。3例周围肺动脉发育差、肺动脉指数(PAI)<150 mm~2/m~2,行升主动脉-肺动脉分流术;2例全部新的肺动脉指数(TNPAI)<150 mm~2/m~2或肺循环完全由分散、细小的 MAPCA 供应者,行一期单源化手术和室间隔缺损开放;27例(28例次,包括1例分流术后13个月)肺动脉发育达到 PAI>150 mm~2/m~2或 TNPAI>200 mm~2m~2,行中度低温体外循环下一期矫治手术。结果术后早期死亡5例,其中一期矫治手术后死亡4例,分别为严重低心排血量综合征2例,灌注肺1例,多脏器功能衰竭1例;升主动脉肺动脉分流术后死亡1例,为切口感染、心内膜炎。21例随访3个月～15.5年,NYHA 心功能Ⅰ和Ⅱ级19例,Ⅲ和Ⅳ级各1例。结论 VSD-PA 的外科治疗包括姑息或矫治手术,对于肺动脉发育较好的 VSD-PA,尽可能早期进行一期矫治手术可以得到满意的效果。
Objective To investigate the surgical timing, indications and methods of surgical treatment of pulmonary artery atresia with ventricular septal defect (VSD-PA). Methods Thirty-two cases of VSD-PA were treated surgically from June 1984 to March 2005. There were 15 males and 15 females in 32 cases, 17 females, ranging in age from 6 months to 9 years. Among them, Nine cases of collateral circulation artery (APCA). 3 cases had poor peripheral pulmonary artery development, pulmonary artery index (PAI) <150 mm ~ 2 / m ~ 2 and ascending aorta-pulmonary artery shunt. All 2 new pulmonary artery index (TNPAI) Or pulmonary circulation was completely open with scattered, small MAPCA providers, single-source single-source surgery and ventricular septal defect. The pulmonary development in 27 patients (28 sessions, including 13 months after shunting) reached PAI> 150 mm 2 / m ~ 2 or TNPAI> 200 mm ~ 2m ~ 2, underwent moderate hypothermia and cardiopulmonary bypass. Results In the early postoperative period, 5 patients died. One patient died after the first operation, 4 patients were severe low cardiac output syndrome, 1 perfusion lung and 1 multiple organ failure. Ascending aorta pulmonary artery shunt After death in 1 case, incision infection, endocarditis. Twenty-one patients were followed up for 3 months to 15.5 years. There were 19 patients with NYHA class I and II and 1 patient with grade III and IV respectively. Conclusion Surgical treatment of VSD-PA includes palliative or orthodontic treatment. For VSD-PA with good pulmonary artery development, satisfactory results can be achieved by performing an orthodontic treatment as early as possible.