Iyer K S, Mee R B
Victorian Pediatric Cardiac Surgical Unit, Royal Children's Hospital, Parkville, Australia.
Ann Thorac Surg. 1991 Jan;51(1):65-72. doi: 10.1016/0003-4975(91)90452-v.
Fifty-eight consecutive patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries with arborization defects, and major aortopulmonary collaterals were entered into a program for staged surgical repair between January 1979 and July 1989. Prerepair preparatory procedures were designed to (1) encourage native pulmonary artery growth by increasing blood flow and (2) unifocalize pulmonary blood supply by transplanting or ligating major collaterals. A total of 121 staging procedures were performed with an overall mortality of 10.3%. One hundred thirty-four major collaterals were either ligated or transplanted. Thirty patients eventually underwent hemodynamic repair with an early mortality of 3.3% and late mortality of 10.0%. Twenty-six current survivors of repair remain clinically well after a mean follow-up of 3.6 years. Ten patients are currently in various stages of preparation. Twelve patients (20.7%) failed to achieve minimum requirements for repair after staging and await further palliation or heart-lung transplantation. The principles of management have evolved over the years and are discussed.
1979年1月至1989年7月期间，58例患有肺动脉闭锁、室间隔缺损、伴有分支缺陷的发育不良肺动脉以及主要主肺动脉侧支的连续患者被纳入分期手术修复计划。修复前的准备程序旨在：（1）通过增加血流量促进原生肺动脉生长；（2）通过移植或结扎主要侧支使肺供血单源化。共进行了121次分期手术，总死亡率为10.3%。134条主要侧支被结扎或移植。30例患者最终接受了血流动力学修复，早期死亡率为3.3%，晚期死亡率为10.0%。26例目前的修复幸存者在平均随访3.6年后临床状况良好。10例患者目前处于不同的准备阶段。12例患者（20.7%）在分期后未达到修复的最低要求，等待进一步的姑息治疗或心肺移植。多年来管理原则不断演变，本文对此进行了讨论。
