Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands.
Pulm Circ. 2013 Apr;3(2):440-3. doi: 10.4103/2045-8932.113185.
A congenital extrahepatic portosystemic venous shunt (CEPVS), also known as an Abernethy malformation, is a rare cause of pulmonary arterial hypertension (PAH). In this case series, we describe three male patients of 30, 23, and 27 years of age with PAH due to a CEPVS. In all three patients, a right heart catheterization revealed a high cardiac output. The aim of this case series is to make pulmonary hypertension physicians aware of the possibility of a CEPVS when PAH is accompanied with a high cardiac output state.
先天性肝外门体静脉分流（CEPVS），也称为 Abernethy 畸形，是肺动脉高压（PAH）的罕见病因。在本病例系列中，我们描述了 3 名男性患者，年龄分别为 30、23 和 27 岁，因 CEPVS 导致 PAH。在所有 3 名患者中，右心导管检查均显示心输出量高。本病例系列的目的是使肺高血压医师在 PAH 伴有高心输出量状态时意识到 CEPVS 的可能性。
