Surgical Strategies and Results for Repair of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals: Experience of a Single Tertiary Center

Abstract Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

摘要
目的：评估伴主-肺动脉侧支动脉的肺动脉闭锁合并室间隔缺损（pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries, PA/VSD/MAPCAs）患者的外科治疗方案及临床疗效。
方法：回顾性分析2012年1月至2018年10月期间收治的连续PA/VSD/MAPCAs患者病例。将研究对象分为四组：A组为功能良好的主-肺动脉侧支动脉组；B组为发育不良的主-肺动脉侧支动脉组；C组为所有分支均严重发育不良的主-肺动脉侧支动脉组；D组为多数主-肺动脉侧支动脉分支存在远端狭窄组。
结果：本研究共纳入36例患者。患者手术时的中位年龄为5.5个月（范围2~110个月），中位体重为8kg（范围2.5~21kg），中位主-肺动脉侧支动脉数量为3支（范围1~6支）。A组14例患者接受一期完全矫治手术（total correction, TC）；B组18例患者接受侧支血管单源化联合中心分流术；C组4例患者接受主-肺动脉窗成形术及侧支血管结扎术。无患者被纳入D组。70%的患者（n=25）接受了完全矫治手术。A组无早期死亡病例，其余两组早期死亡率为13.6%。随访期间，3例患者接受了再次干预治疗，其中2例行人工管道置换术，1例行右心室流出道重建术。
结论：对PA/VSD/MAPCAs患者进行详细评估并实施分组，有助于确定个体化的外科治疗策略。采用该治疗策略可使多数患者实现完全矫治。一期完全矫治手术的早期死亡率及再次干预率均低于其他术式，因此更具临床优势。临床工作中需警惕术后早期重症监护相关并发症，并严格把握随访期间的再次干预指征。