Macalister Samuel J, Buratto Edward, Naimo Phillip S, Ye Xin Tao, Fulkoski Nicholas, Weintraub Robert G, Brizard Christian P, Konstantinov Igor E
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia.
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
Ann Thorac Surg. 2023 Feb;115(2):445-451. doi: 10.1016/j.athoracsur.2022.09.022. Epub 2022 Sep 23.
The optimal strategy for pulmonary atresia with ventricular septal defect (PA-VSD) without major aortopulmonary collateral arteries is yet to be defined. It remains unclear how primary complete repair compares with staged repair.
This study reviewed the records of 107 patients with PA-VSD who were managed with systemic-to-pulmonary shunts from February 1989 to November 2019. Tables presenting summary statistics of patient and surgical data and Kaplan-Meier curves showing repair, mortality, and reoperation were created. Logistic regression analysis was performed to identify risk factors for repair, morbidity, and mortality.
A total of 91 (85%) patients achieved complete repair at a median age of 1 year (interquartile range [IQR], 8-20 months). Survival was as follows: 97 (90%) patients at 6 months, 91 (85%) at 20 months, and 87 (81%) at 10 years. A shunt diameter of 3.5 mm or less (P = .006) and shunt after 2009 (P < .001) were associated with earlier progression to complete repair. A shunt diameter of 3.5 mm or less was an independent risk factor for interstage morbidity (hazard ratio, 4.5; IQR, 1.1-18.5; P = .039), but it was not associated with complete repair (P = .75) or mortality (P = .62). The median follow-up period was 10.5 years (IQR, 3.6-18.8 years) from birth.
The complete repair and long-term survival rates of staged PA-VSD repair are high. The use of smaller shunts has resulted in earlier complete repair and is associated with increased risk of morbidity but not mortality.
对于无主要体肺侧支动脉的室间隔缺损合并肺动脉闭锁（PA-VSD），最佳治疗策略尚未明确。目前尚不清楚一期完全修复与分期修复相比效果如何。
本研究回顾了1989年2月至2019年11月期间接受体肺分流术治疗的107例PA-VSD患者的病历。创建了呈现患者和手术数据汇总统计的表格以及显示修复、死亡率和再次手术情况的Kaplan-Meier曲线。进行逻辑回归分析以确定修复、发病和死亡的危险因素。
共有91例（85%）患者在1岁时（四分位间距[IQR]，8 - 20个月）实现了完全修复。生存率如下：6个月时97例（90%），20个月时91例（85%），10年时87例（81%）。分流直径3.5毫米及以下（P = 0.006）以及2009年后进行的分流（P < 0.001）与更早进展至完全修复相关。分流直径3.5毫米及以下是分期手术期间发病的独立危险因素（风险比，4.5；IQR，1.1 - 18.5；P = 0.039），但与完全修复（P = 0.75）或死亡率（P = 0.62）无关。从出生起的中位随访期为10.5年（IQR，�.6 - 18.8年）。
分期PA-VSD修复的完全修复率和长期生存率较高。使用较小的分流管可使完全修复时间提前，且与发病风险增加相关，但与死亡率无关。
