A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently as...

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Detalles Bibliográficos
Autores principales: Yi, Jeong-Eun, Jung, Hae-Ok, Youn, Ho-Joong, Choi, Jong Young, Chun, Ho Jong, Lee, Jae Young
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Academy of Medical Sciences 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3991808/
https://www.ncbi.nlm.nih.gov/pubmed/24753712
http://dx.doi.org/10.3346/jkms.2014.29.4.604
Descripción
Sumario:Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 µg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension. GRAPHICAL ABSTRACT: [Image: see text]

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