Objective: To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies. Methods: A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children's Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged (M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results: In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95%CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95%CI: 89.9% to 100%). Conclusions: Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.
目的： 探讨采用优化的肺血管单源化策略治疗肺动脉闭锁合并室间隔缺损及粗大体肺侧支患儿的早、中期结果。 方法： 回顾性分析2017年1月至2022年9月在广州医科大学附属广州市妇女儿童医疗中心心脏中心接受手术治疗的104例肺动脉闭锁合并室间隔缺损及粗大体肺侧支患儿的临床资料、手术记录及随访结果。男55例，女49例，年龄［M（IQR）］33.9（84.0）个月（范围：0.5~209.6个月）。解剖分型：B型89例，C型15例；粗大体肺侧支4.2（3.0）支（范围：1~8支）。采用Kaplan-Meier法绘制生存曲线。 结果： 一期手术中，50例接受完全根治术，12例接受部分根治术，32例接受姑息性右心室-肺动脉连接术，10例选择了体肺分流。一期完全根治率为48.1%（50/104），术后早期病死率为9.6%（10/104）。二期手术中，14例获得完全根治，4例获得部分根治，无早期死亡。总体根治率为61.5%（64/104）。两期手术间隔时间为19（10）个月（范围：9~48个月）。完全根治术后右心室/左心室收缩峰压比值为0.63±0.16（范围：0.36~1.00）。全组患儿一期手术后1年和5年生存率均为89.4%（95%CI：83.5%~95.3%），完全根治术后随访时间为28（34）个月（范围：1~67个月），1年和5年生存率均为95.2%（95%CI：89.9%~100%）。 结论： 根据肺动脉闭锁合并室间隔缺损及粗大体肺侧支患儿的特点，采用优化的肺血管完全单源化技术，可获得较高的一期完全根治率和5年累积完全根治率，以及满意的右心室/左心室收缩峰压比值和早中期生存率。.