Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation
Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation
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DOI：
10.1016/j.jtcvs.2010.08.088
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发表时间：
2011-06-01
影响因子：
6
通讯作者：
del Nido, Pedro J.
中科院分区：
文献类型：
--
作者：
McElhinney, Doff B.;Marx, Gerald R.;del Nido, Pedro J.
目的：肺动静脉畸形是一种重要但不常见的腔静脉连接并发症，特别是在异位症患者中。肺动脉血中无肝静脉流出物似乎是一个诱发因素。肺动静脉畸形在上级腔静脉吻合术后最常见，但如果肝静脉血流完全或主要流向对侧肺，则Fontan完成后可能在1个肺中发展、进展或持续。自1985年至2005年，对53例异位症和下腔静脉阻断患者进行了改良Fontan手术，8例肝静脉包容后出现肝静脉血流单侧分流和临床显著的肺动静脉畸形，并接受了腔静脉通路重构。在所有8例患者中，肝静脉-肺动脉通路均位于单根或优势上级腔静脉的肺动脉吻合口的对侧和偏移处。重建途径包括肺动脉支架置入术2例，上级静脉-肺动脉连接改良术1例，肝静脉-肺动脉分支通道重建术2例，肝静脉-奇静脉直接通道重建术5例。结论：单侧肺动静脉畸形和Fontan完成后肝静脉血流分流患者的腔静脉通路重构后低氧血症的解决支持肝静脉流出物在肺动静脉畸形发病机制中的重要性，以及此类患者的腔静脉通路修正的实践。完成或重构Fontan循环，直接肝静脉-奇静脉连接，可以为该患者人群提供最可靠的肝静脉血液混合和双侧分布。（《胸血管外科杂志》2011; 141：1362-70）
Objective: Pulmonary arteriovenous malformations are an important but uncommon complication of cavopulmonary connection, particularly in patients with heterotaxy. Absence of hepatic venous effluent in pulmonary arterial blood seems to be a predisposing factor. Pulmonary arteriovenous malformations are most common after superior cavopulmonary anastomosis, but may develop, progress, or persist in 1 lung after Fontan completion if hepatic venous blood streams completely or primarily to the contralateral lung.Methods: Among 53 patients with heterotaxy and inferior vena cava interruption who underwent a modified Fontan procedure from 1985 to 2005, 8 had unilateral streaming of hepatic venous flow and clinically significant pulmonary arteriovenous malformations after hepatic venous inclusion and underwent reconfiguration of the cavopulmonary pathway. In all 8 patients, the hepatic vein-pulmonary artery pathway was contralateral to and offset from the pulmonary artery anastomosis of the single or dominant superior vena cava. Pathway reconfiguration included pulmonary arterial stenting (n = 2), revision of the superior vena cava-pulmonary artery connection (n = 1), construction of a branched hepatic vein-pulmonary artery conduit (n = 2), and surgical or transcatheter construction of a direct hepatic vein-azygous vein pathway (n = 5).Results: Hepatic vein-azygous vein connection led to improvement in 4 of 5 patients; other approaches typically did not lead to improvement.Conclusions: Resolution of hypoxemia after cavopulmonary pathway reconfiguration in patients with unilateral pulmonary arteriovenous malformations and hepatic venous flow-streaming after Fontan completion supports the importance of hepatic venous effluent in the pathogenesis of pulmonary arteriovenous malformations and the practice of cavopulmonary pathway revision in such patients. Completion or reconfiguration of the Fontan circulation with direct hepatic vein-azygous vein connection may provide the most reliable mixing and bilateral distribution of hepatic venous blood in this population of patients. (J Thorac Cardiovasc Surg 2011; 141: 1362-70)