Primary Pulmonary Hypertension: An Unusual Case Associated with Extrahepatic Portal Hypertension
Primary Pulmonary Hypertension: An Unusual Case Associated with Extrahepatic Portal Hypertension
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原发性肺动脉高压：与肝外门脉高压相关的罕见病例
DOI：
10.1002/hep.1840030419
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发表时间：
2007
期刊：
影响因子：
13.5
通讯作者：
J. Cuthbert
中科院分区：
文献类型：
--
作者：
M. Cohen;L. Rubin;W. Taylor;J. Cuthbert
报告1例肝外门脉高压合并原发性肺动脉高压患者。肺动脉高压在没有外科门体分流的情况下发生，尸检时没有大量自发性分流的证据。这种肺动脉高压和门静脉高压的相关性，既没有内在性肝病，也没有大型门体吻合术，以前还没有报道过。我们的病例支持这样的观点，即门静脉高压及其伴随的门体侧支循环可能是内在性肝病和肝外门静脉阻塞中肺动脉高压的主要诱发原因。
A patient with both extrahepatic portal hypertension and primary pulmonary hypertension is reported. The pulmonary hypertension developed without a surgical portal‐systemic shunt, and at autopsy there was no evidence of a large spontaneous shunt. This association of pulmonary arterial hypertension and portal venous hypertension without either intrinsic liver disease or a large portal‐systemic anastomosis has not been reported previously. Our case supports the concept that portal hypertension with its attendant portal‐systemic collateral circulation may be the major predisposing cause of pulmonary hypertension both in intrinsic liver disease and extrahepatic portal vein obstruction.