Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
目的：回顾了过去13年在我们中心接受修复的完全性肺静脉异位连接患者的临床资料。在这项研究中,我们系统地回顾了我们在完全性肺静脉异位连接患者的最佳手术策略方面的经验，旨在为临床决策提供依据。
方法：2009年1月1日至2021年12月31日在我院接受手术治疗的122例完全性肺静脉异位引流患者。其中,研究中排除了18例单心室修复患者。多因素分析用于确定早期和晚期死亡的危险因素以及肺静脉阻塞的危险因素。
结果：男性64例，女性40例。手术年龄中位数为107天（范围，25天-788天），手术时的中位体重为4.8kg（范围，3kg-22kg),中位随访时间为59个月(范围，0-150个月）。7例患者术后早期死亡，6例患者出院后晚期死亡。多因素分析显示体外循环时间延长是术后早期死亡的独立危险因素。多因素分析未发现晚期死亡的危险因素。急诊手术,术前中度和重度肺动脉高压，体外循环时间延长是术后肺静脉阻塞的独立危险因素。
结论：完全性肺静脉异位连接患者的早期和长期晚期修复结果令人鼓舞。术后肺静脉阻塞仍然是全世界专家的主要问题。术前急诊手术患儿应考虑肺静脉阻塞，中度至重度肺动脉高压和延长的体外循环时间，定期跟进是必要的。
方法：2009年1月1日至2021年12月31日在我院接受手术治疗的122例完全性肺静脉异位引流患者。其中,研究中排除了18例单心室修复患者。多因素分析用于确定早期和晚期死亡的危险因素以及肺静脉阻塞的危险因素。
结果：男性64例，女性40例。手术年龄中位数为107天（范围，25天-788天），手术时的中位体重为4.8kg（范围，3kg-22kg),中位随访时间为59个月(范围，0-150个月）。7例患者术后早期死亡，6例患者出院后晚期死亡。多因素分析显示体外循环时间延长是术后早期死亡的独立危险因素。多因素分析未发现晚期死亡的危险因素。急诊手术,术前中度和重度肺动脉高压，体外循环时间延长是术后肺静脉阻塞的独立危险因素。
结论：完全性肺静脉异位连接患者的早期和长期晚期修复结果令人鼓舞。术后肺静脉阻塞仍然是全世界专家的主要问题。术前急诊手术患儿应考虑肺静脉阻塞，中度至重度肺动脉高压和延长的体外循环时间，定期跟进是必要的。
