Congenital Portosystemic Venous Connections and Other Abdominal Venous Abnormalities in Patients with Polysplenia and Functionally Univentricular Heart Disease: A Case Series and Literature Review
Congenital Portosystemic Venous Connections and Other Abdominal Venous Abnormalities in Patients with Polysplenia and Functionally Univentricular Heart Disease: A Case Series and Literature Review
复制标题
DOI：
10.1111/j.1747-0803.2010.00478.x
复制
发表时间：
2011-01-01
影响因子：
0.3
通讯作者：
Newburger, Jane W.
中科院分区：
文献类型：
--
作者：
McElhinney, Doff B.;Marx, Gerald R.;Newburger, Jane W.
目标。已发表的病例报告表明，先天性门体静脉连接(PSVC)和其他腹部静脉畸形在多脾综合征患者中可能相对常见，并且可能是重要的。我们的目的是调查在本中心行腔静脉-肺吻合术的多脾和下腔静脉(IVC)阻断患者门静脉和其他腹部系统静脉异常的频率和范围，并回顾已发表的关于这一主题的文献和此类异常的潜在临床意义。采用回顾性队列研究和文献综述相结合的方法。在77例接受双向Glenn和/或改良Fontan手术的异位畸形、单室性心脏病和下腔静脉阻断的患者中，33例(43%)被诊断为肺动静脉畸形。42例(55%)患者存在双侧上腔静脉。尽管许多患者成像不充分，但仍有15例患者(19%)检测到部分PSVC、双腔静脉和/或肾静脉异常。6例(8%)发现由一条曲折的血管从体静脉系统至肝外门静脉形成的PSVC。除PSVC外，13例(16%)患者存在腹内静脉异常，其中9例(12%)存在某种形式的重复下腔静脉系统，其中大的奇静脉延续至上腔静脉，对侧平行的下腔静脉大小相似或更小，7例合并肾静脉异常。在部分PSVC或重复的下腔静脉连接到心房的患者中，异常连接允许从右向左分流。PSVC和其他腹部静脉异常在临床上可能很重要，但在下腔静脉阻断和单室性心脏病患者中被忽视。对于此类患者，术前评估腹部全身静脉系统可能是有价值的。需要更多的数据来确定异位的多脾变异型PSVC和腔静脉吻合相关的肺动静脉畸形之间是否存在病理生理联系。
Objective. Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemic venous anomalies in patients with polysplenia and inferior vena cava (IVC) interruption who underwent a cavopulmonary anastomosis procedure at our center, and to review the published literature on this topic and the potential clinical importance of such anomalies.Design. Retrospective cohort study and literature review were used.Results. Among 77 patients with heterotaxy, univentricular heart disease, and IVC interruption who underwent a bidirectional Glenn and/or modified Fontan procedure, pulmonary arteriovenous malformations were diagnosed in 33 (43%). Bilateral superior vena cavas were present in 42 patients (55%). Despite inadequate imaging in many patients, a partial PSVC, dual IVCs, and/or renal vein anomalies were detected in 15 patients (19%). A PSVC formed by a tortuous vessel running from the systemic venous system to the extrahepatic portal vein was found in six patients (8%). Abdominal venous anomalies other than PSVC were documented in 13 patients (16%), including nine (12%) with some form of duplicated IVC system, with a large azygous vein continuing to the superior vena cava and a parallel, contralateral IVC of similar or smaller size, and seven with renal vein anomalies. In patients with a partial PSVC or a duplicate IVC that connected to the atrium, the abnormal connection allowed right-to-left shunting.Conclusions. PSVC and other abdominal venous anomalies may be clinically important but under-recognized in patients with IVC interruption and univentricular heart disease. In such patients, preoperative evaluation of the abdominal systemic venous system may be valuable. More data are necessary to determine whether there is a pathophysiologic connection between the polysplenia variant of heterotaxy, PSVC, and cavopulmonary anastomosis-associated pulmonary arteriovenous malformations.