PDF(Pubmed)
Abstract：
Congenital extrahepatic portocaval shunt (CEPS) is a rare condition in which a rare congenital vascular anomaly of the portal system is present. CEPS may manifest as pulmonary arterial hypertension (PAH). When diagnosed and treated early, PAH can be reversible. We report a case of a previously asymptomatic woman, who manifested with severe pulmonary hypertension during pregnancy and was consequently diagnosed with CEPS. After unsuccessful medical treatment, urgent lung transplantation was done.
摘要：
先天性肝外门腔分流术（CEPS）是一种罕见的情况，其中存在罕见的先天性门静脉系统血管异常。CEPS可表现为肺动脉高压(PAH)。早期诊断和治疗时，PAH可以是可逆的。我们报告了一个以前无症状的女性，在怀孕期间表现出严重的肺动脉高压，因此被诊断为CEPS。在治疗不成功之后,进行了紧急肺移植。
