Outcomes after surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: a series of 104 cases
10.3760/cma.j.cn112139-20230108-00012
- VernacularTitle:肺动脉闭锁合并室间隔缺损及粗大体肺侧支外科治疗104例临床分析
- Author:
Minghui ZOU
1
;
Fan CAO
;
Li MA
;
Yuansheng XIA
;
Shengchun YANG
;
Weidan CHEN
;
Wenlei LI
;
Xinxin CHEN
Author Information
1. 广州医科大学附属广州市妇女儿童医疗中心心脏中心，广州 510623
- Keywords:
Pulmonary atresia;
Heart septal defects, ventricular;
Major aortopulmonary collateral arteries;
Unifocalization;
Rehabilitation
- From:
Chinese Journal of Surgery
2023;61(12):1093-1098
- CountryChina
- Language:Chinese
-
Abstract:
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.
