Wu I-Hui, Nguyen Khanh H
Department of Cardiothoracic Surgery, Mount Sinai School of Medicine, One Gustave L. Levy Place, PO Box 1028, New York, NY 10029, USA.
Pediatr Cardiol. 2006 Jul-Aug;27(4):519-22. doi: 10.1007/s00246-006-1261-z. Epub 2006 Jun 22.
The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.
已有报道称，在采用心外管道进行全腔静脉-肺动脉连接术后的患者中出现了单侧肺动静脉畸形。理论上，肝静脉血流向肺部的分布不均是致病因素。我们报告了1例患有内脏反位综合征、单心室和下腔静脉中断的患者的肺动静脉畸形的外科治疗情况。该患者此前已接受了采用心外管道的全腔静脉-肺动脉连接术，将肝静脉血流引流至右肺动脉分支。在随后的手术中，我们将心外管道改道至无名静脉。该手术为患侧肺提供了肝静脉血，同时避免了患者遭受与体外循环相关的并发症。
