Vanderlaan Rachel D, Caldarone Christopher A
University of Toronto, Division of Cardiac Surgery, Toronto, Ontario, Canada.
Hospital for Sick Children, Division of Cardiovascular Surgery, Toronto, Ontario, Canada.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2018 Mar;21:83-91. doi: 10.1053/j.pcsu.2017.11.010.
Total anomalous pulmonary venous connection (TAPVC) constitutes a spectrum of congenital lesions whereby the pulmonary veins remain connected to systemic venous vessels or aberrantly connect to the right atrium. Definitive management requires surgical intervention and, in patients with obstruction to pulmonary venous flow, urgent operation is required. Use of temporizing catheter-based interventions allow for optimization in hemodynamically unstable neonates. Overall, survival has significantly improved over the past decades through better perioperative management and evolution of surgical approaches to minimize post-repair pulmonary vein stenosis, which persists as a major determinant of long-term outcomes.
完全性肺静脉异位连接（TAPVC）是一系列先天性病变，在此病变中肺静脉仍与体静脉血管相连或异常连接至右心房。确定性治疗需要手术干预，对于肺静脉血流受阻的患者，需要紧急手术。使用基于导管的临时干预措施可优化血流动力学不稳定新生儿的状况。总体而言，在过去几十年中，通过更好的围手术期管理和手术方法的改进，以尽量减少修复后肺静脉狭窄，生存率已显著提高，而肺静脉狭窄仍然是长期预后的主要决定因素。
