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一种罕见的发绀原因：先天性肝外门体分流所致的肝肺综合征。

A rare cause of cyanosis: hepatopulmonary syndrome caused by congenital extrahepatic portosystemic shunt.

作者信息

Ding Xue-Yan, Chen Feng, Zhao Xian-Xian, Wu Hong, Chen Shao-Ping, Qin Yong-Wen

机构信息

Department of Cardiology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China.

出版信息

Case Rep Vasc Med. 2011;2011:508171. doi: 10.1155/2011/508171. Epub 2011 Dec 20.

DOI:10.1155/2011/508171
PMID:22937464
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3420761/
Abstract

A 19-year-old male patient presented cyanosis and dyspnoea because of the presence of multiple pulmonary arteriovenous fistulas resulting in oxygen desaturation. The CTA revealed that intestinal and splenic venous blood bypasses the liver and drains into the inferior vena cava. This is the first reported case of hepatopulmonary syndrome caused by congenital extrahepatic portosystemic shunt in which intestinal and splenic venous blood bypasses the liver and drains into the inferior vena cava.

摘要

一名19岁男性患者因存在多个肺动静脉瘘导致氧饱和度降低而出现发绀和呼吸困难。CT血管造影显示肠静脉和脾静脉血绕过肝脏，直接汇入下腔静脉。这是首例因先天性肝外门体分流导致肠静脉和脾静脉血绕过肝脏直接汇入下腔静脉而引起肝肺综合征的报告病例。

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引用本文的文献

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Congenital portocaval fistula associated with hepatopulmonary syndrome: ligation vs liver transplantation.
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Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies.先天性门静脉缺如：两例报告及门静脉系统血管异常的分类系统建议
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