Wang Zhangwei, Ma Kai, Li Shoujun
Department of Cardiovascular Surgery, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Fuwai Hospital, Beijing, China.
Department of Cardiac Surgery, Beijing Children's Hospital, Capital Medical University, National Children's Medical Center, Beijing, China.
Cardiol Young. 2024 May 27:1-10. doi: 10.1017/S1047951124025204.
The clinical data of patients with total anomalous pulmonary venous connection who underwent repair in our centre in the past 13 years were reviewed. In this study, we systemically reviewed our experience in the optimal surgical strategy for patients with total anomalous pulmonary venous connection, aiming to provide evidence for clinical decision-making.
From January 1, 2009, to December 31, 2021, 122 patients undergoing surgical treatment for total anomalous pulmonary venous connection in our hospital were enrolled. Among them, 18 patients with single ventricle repair were excluded from the study. Multivariate analysis was used to determine the risk factors for early and late death and the risk factors for pulmonary vein obstruction.
There were 64 males and 40 females. The median age at surgery was 107 days (range, 25 days-788 days), the median weight at surgery was 4.8 kg (range, 3 kg-22 kg), and the median follow-up was 59 months (range, 0-150 months). Seven patients died early after surgery and six died late after discharge. Multivariable analysis indicated that prolonged cardiopulmonary bypass time was the only independent risk factor for early postoperative mortality. Multivariate analysis did not identify risk factors for late death. Emergency surgery, preoperative moderate and severe pulmonary hypertension, and prolonged cardiopulmonary bypass time were independent risk factors for postoperative pulmonary vein obstruction.
Early and long-term late outcomes of repair in patients with total anomalous pulmonary venous connection have been encouraging. Postoperative pulmonary vein obstruction remains a major problem for specialists worldwide. Pulmonary vein obstruction should be considered in children with preoperative emergency surgery, moderate to severe pulmonary hypertension and prolonged cardiopulmonary bypass time, and regular follow-up is necessary.
回顾过去13年在我院中心接受矫治的完全性肺静脉异位连接患者的临床资料。本研究系统回顾了我们在完全性肺静脉异位连接患者最佳手术策略方面的经验，旨在为临床决策提供依据。
纳入2009年1月1日至2021年12月31日在我院接受完全性肺静脉异位连接手术治疗的122例患者。其中，18例单心室修复患者被排除在研究之外。采用多因素分析确定早期和晚期死亡的危险因素以及肺静脉梗阻的危险因素。
男性64例，女性40例。手术时的中位年龄为107天（范围25天至788天），手术时的中位体重为4.8 kg（范围3 kg至22 kg），中位随访时间为59个月（范围0至150个月）。7例患者术后早期死亡，6例患者出院后晚期死亡。多变量分析表明，体外循环时间延长是术后早期死亡的唯一独立危险因素。多因素分析未确定晚期死亡的危险因素。急诊手术、术前中重度肺动脉高压和体外循环时间延长是术后肺静脉梗阻的独立危险因素。
完全性肺静脉异位连接患者矫治的早期和长期远期结果令人鼓舞。术后肺静脉梗阻仍然是全球专家面临的一个主要问题。对于术前急诊手术、中重度肺动脉高压和体外循环时间延长的患儿，应考虑肺静脉梗阻的可能，且有必要进行定期随访。
