Department of Cardiology, Children's Hospital, Boston, MA 02115, USA.
J Thorac Cardiovasc Surg. 2011 Jun;141(6):1362-70.e1. doi: 10.1016/j.jtcvs.2010.08.088. Epub 2010 Dec 13.
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.
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).
Hepatic vein-azygous vein connection led to improvement in 4 of 5 patients; other approaches typically did not lead to improvement.
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.
肺动静脉畸形是腔静脉-肺动脉连接的一种重要但罕见的并发症，特别是在异构患者中。肺动脉中缺乏肝静脉流出似乎是一个诱发因素。肺动静脉畸形在上腔静脉-肺动脉吻合术后最为常见，但如果肝静脉血流完全或主要流向对侧肺，Fontan 完成后 1 肺中可能会发展、进展或持续存在。
在 1985 年至 2005 年间接受改良 Fontan 手术的 53 例异构伴下腔静脉中断患者中，8 例在肝静脉包含后出现单侧肝静脉血流分流和临床显著的肺动静脉畸形，并进行了腔静脉-肺循环途径的重建。在所有 8 例患者中，肝静脉-肺动脉途径位于单或优势上腔静脉的肺动脉吻合口的对侧并与之错开。途径重建包括肺动脉支架置入术（n=2）、上腔静脉-肺动脉连接修正术（n=1）、构建分支肝静脉-肺动脉导管（n=2），以及经导管或手术构建直接肝静脉-奇静脉途径（n=5）。
肝静脉-奇静脉连接使 5 例中的 4 例缺氧得到改善；其他方法通常不会导致改善。
在 Fontan 完成后单侧肺动静脉畸形和肝静脉血流分流的腔静脉途径重建后，低氧血症的改善支持肝静脉流出在肺动静脉畸形发病机制中的重要性，并支持此类患者进行腔静脉途径修正。直接肝静脉-奇静脉连接完成或重建 Fontan 循环可能为该患者群体提供最可靠的肝静脉血液混合和双侧分布。
