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Portopulmonary hypertension associated with congenital absence of the portal vein treated with bosentan.

Portopulmonary hypertension (PPHTN) is pulmonary arterial hypertension (PAH) associated with portal hypertension. It is a common condition among liver transplantation candidates; however, its association with congenital absence of the portal vein (CAPV) has not yet been established. CAPV is a very rare developmental anomaly, which is usually accompanied by abnormal mesenteric drainage that bypasses the liver. Here, we report a rare case of severe PPHTN secondary to CAPV.