2014
DOI: 10.3346/jkms.2014.29.4.604
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A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt

Abstract: 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… Show more

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Cited by 10 publications
(15 citation statements)
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“…(19,33) and, less frequently, HPS. (14,27) The development of HPS and PaHT could be in relation to intestinal vasoactive mediators (14,15,34) that, having bypassed the liver and not being properly metabolized, reached the pulmonary vascular bed, inducing a long-standing pulmonary vasoconstriction in the case of PaHT (15,16) or, on the contrary, pulmonary vasodilation in the case of HPS. Our study confirms that PaHT is a significant problem in patients with CEPS, causing symptomatic dyspnea in 80% of cases.…”
Section: Discussionmentioning
confidence: 99%
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“…(19,33) and, less frequently, HPS. (14,27) The development of HPS and PaHT could be in relation to intestinal vasoactive mediators (14,15,34) that, having bypassed the liver and not being properly metabolized, reached the pulmonary vascular bed, inducing a long-standing pulmonary vasoconstriction in the case of PaHT (15,16) or, on the contrary, pulmonary vasodilation in the case of HPS. Our study confirms that PaHT is a significant problem in patients with CEPS, causing symptomatic dyspnea in 80% of cases.…”
Section: Discussionmentioning
confidence: 99%
“…The spectrum of clinical variants of CEPS ranges from completely asymptomatic forms to severe forms of hepatic encephalopathy (HE), (12,13) hepatopulmonary syndrome (HPS), and pulmonary arterial hypertension (PaHT). (14)(15)(16) Nodular liver lesions are frequent in patients with CEPS and although most of these nodules are benign, hepatocellular carcinomas (HCCs) and adenomas, among other neoplastic lesions, have been reported. (17,18) However, the vast majority of these publications are single case reports providing only a cross-sectional description without follow-up.…”
mentioning
confidence: 99%
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“…Abernethy malformation can be in combination with other congenital malformations such as cardiovascular malformations, biliary atresia, choledochus cysts, polysplenia or musculoskeletal abnormalities [3,4]. Besides, patients may also suffer from some complications, including hepatic encephalopathy [5], hepatopulmonary syndrome [6], pulmonary arterial hypertension [7], even hepatocellular carcinomas [8]. For the reason that there have been only about 300 patients reported in the literature since the first patient was reported by Abernethy in 1797 [9] and the vast majority of these publications are single case reports, it is difficult to calculate the accurate prevalence of this disease.…”
Section: Introductionmentioning
confidence: 99%
“…Therefore, structurally, type 2 shunt is not characterized by true absence of the portal vein. A type 2 shunt is more common in males and is rarely associated with other malformations [4].…”
Section: Introductionmentioning
confidence: 99%