伴室间隔缺损的肺动脉闭锁的外科治疗

Surgical treatments and results of pulmonary atresia with ventricular septal defect

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 evaluate the indication and operative program of ventricular septal defect with pulmonary atresia（VSD-PA）. Methods From June 1984 to March 2005, there were 32 patients with VSD-PA, which underwent 33 operations. Among them, 15 were males and 17 were females, The ages ranged from 6 months to 9 years. There were 9 patients with aortopulmonary collateral arteries, The operations included central aorta-pulmonary shunts 3 cases, one stage complete repair 27 cases and one stage unifocalization with VSD open 2 cases. Results There were early 5 death including one death after aortapulmonary shunt and 4 deaths after one stage complete repair, The causes of death were severe low cardiac output （2 patients）, respiratory function failure （ 1 patient）, multiorgan function failure （ 1 patient） and severe infect （ 1 patient）. Twenty-one patients were followed up from 3 months to 15.5 years. Heart function （NYHA） was class Ⅰ or Ⅱin 19 cases and class Ⅲor Ⅳ in 2 cases. Conclusions The operative indication and methods mainly depend on the developing of pulmonary arteries and aortopulmonary collateral arteries. Completely surgical repair of patients with VSD-PA can be achieved with acceptable mortality.