Department of Cardiovascular Surgery, Guangdong Provincial People's Hospital, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong Academy of Medical Sciences, Guangzhou, China.
Department of Cardiovascular Intensive Care Unit, Guangdong Provincial People's Hospital, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong Academy of Medical Sciences, Guangzhou, China.
J Thorac Cardiovasc Surg. 2023 Jul;166(1):193-200.e1. doi: 10.1016/j.jtcvs.2022.10.008. Epub 2022 Oct 14.
Supracardiac total anomalous pulmonary venous connection is the most common subtype of total anomalous pulmonary venous connection. We aimed to describe the morphological spectrum of supracardiac total anomalous pulmonary venous connection and to identify risk factors for death and postoperative pulmonary venous obstruction.
From February 2009 to June 2019, 241 patients diagnosed with supracardiac-Ia (left-sided vertical vein, n = 185) or supracardiac-Ib (right-sided connection directly to superior vena cava, n = 56) total anomalous pulmonary venous connection underwent initial surgical repair at our institute. Cases with functionally univentricular circulations or atrial isomerism were excluded. Patients' postoperative survival was described by Kaplan-Meier curves. Cox proportional hazards models and competing risk regression models were used to identify clinical risk factors for death and postoperative pulmonary venous obstruction.
There were 8 early deaths and 4 late deaths. The overall survivals at 30 days, 1 year, and 10 years were 97.1%, 94.8%, and 94.8%, respectively, in the supracardiac-Ia group (2.7%, 5/185) (hazard ratio, 4.8; P = .003). Five patients required reoperation for pulmonary venous obstruction, including 2 patients who required reintervention for superior vena cava syndromes (all in the supracardiac-Ib group). One patient required superior vena cava balloon dilation for superior vena cava syndromes. Multivariable analysis showed that the supracardiac-Ib group (12.5%, 7/56) had a significantly higher mortality rate than the supracardiac-Ia group (adjusted hazard ratio, 8.5, P = .008). Surgical weight less than 2.5 kg (adjusted hazard ratio, 10.8, P = .023), longer duration of cardiopulmonary bypass (adjusted hazard ratio, 1.15 per 10 minutes, P = .012), and supracardiac-Ib subtype (adjusted hazard ratio, 4.7, P = .037) were independent risk factors associated with death. The supracardiac-Ib subtype (adjusted hazard ratio, 4.8, P = .003) was an incremental risk factor associated with postoperative pulmonary venous obstruction.
Morphological features of supracardiac total anomalous pulmonary venous connection, especially the supracardiac-Ib subtype, were risk factors associated with postoperative pulmonary venous obstruction and survival. Patients with unique anatomic subtypes might require more individualized surgical planning.
心上型完全性肺静脉异位连接是完全性肺静脉异位连接中最常见的亚型。本研究旨在描述心上型完全性肺静脉异位连接的形态谱，并确定死亡和术后肺静脉阻塞的危险因素。
从 2009 年 2 月至 2019 年 6 月，在我院接受心上型-Ia（左侧垂直静脉，n=185）或心上型-Ib（直接连接至上腔静脉的右侧连接，n=56）完全性肺静脉异位连接初始手术修复的 241 例患者。排除功能性单心室循环或心房异构的病例。通过 Kaplan-Meier 曲线描述患者术后生存情况。Cox 比例风险模型和竞争风险回归模型用于确定死亡和术后肺静脉阻塞的临床危险因素。
共有 8 例早期死亡和 4 例晚期死亡。心上型-Ia 组术后 30 天、1 年和 10 年的总生存率分别为 97.1%、94.8%和 94.8%（2.7%，5/185）（风险比，4.8；P=0.003）。5 例患者因肺静脉阻塞需再次手术，其中 2 例因上腔静脉综合征需再次干预（均为心上型-Ib 组）。1 例患者因上腔静脉综合征行上腔静脉球囊扩张。多变量分析显示，心上型-Ib 组（12.5%，7/56）的死亡率明显高于心上型-Ia 组（校正风险比，8.5，P=0.008）。体重小于 2.5kg（校正风险比，10.8，P=0.023）、体外循环时间较长（校正风险比，每 10 分钟增加 1.15，P=0.012）和心上型-Ib 亚型（校正风险比，4.7，P=0.037）是与死亡相关的独立危险因素。心上型-Ib 亚型（校正风险比，4.8，P=0.003）是术后肺静脉阻塞的一个附加危险因素。
心上型完全性肺静脉异位连接的形态特征，尤其是心上型-Ib 亚型，是与术后肺静脉阻塞和生存相关的危险因素。具有独特解剖亚型的患者可能需要更个体化的手术计划。
