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Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension-A Case Study Series.

Medicina (Kaunas)

October 2022

Department of Cardiology and Angiology, Adult Congenital Heart Disease Department, Medical Faculty, Slovak Medical University and The National Institute of Cardiovascular Diseases, Pod Krasnou Horkou 1, 833 48 Bratislava, Slovakia.

. Congenital absence of the portal vein (CAPV) is an extremely rare malformation that is caused by aberrant venous development during embryogenesis and is usually associated with congenital portosystemic shunts (CPSS). This hemodynamic allows mesenteric blood to bypass the liver metabolism and causes an imbalance between vasodilators and vasoconstrictors in the pulmonary circulation, which, again, might lead to the development of secondary portopulmonary hypertension (PoPH).

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