Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome

Abstract

CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences. Closure of portosystemic shunts may result in resolution of HPS in type II CEPS.

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

“…Patients with CEPS have been reported to develop severe pulmonary complications, such as PaHT . and, less frequently, HPS . The development of HPS and PaHT could be in relation to intestinal vasoactive mediators 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 or, on the contrary, pulmonary vasodilation in the case of HPS.…”

“…(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.…”

“…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.…”

Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome

“…It is well documented that portosystemic shunt is associated with high risk of pulmonary arteriovenous malformations 6. A large amount of the venous blood from the gut bypasses the liver and directly flows into the heart and lungs via portosystemic shunt vessels.…”

“…In addition, there is growing awareness of the association between polysplenia syndrome and congenital extrahepatic portosystemic shunt 2–4. Both Kawashima operation and extrahepatic portosystemic shunt are known as risk factors for pulmonary arteriovenous malformations, which could result in intrapulmonary right-to-left shunting and progressive cyanosis 5 6. With two risk factors for pulmonary arteriovenous malformations, a multidisciplinary approach would be required to treat progressive cyanosis in patients with polysplenia syndrome and univentricular heart disease.…”