Marcela Tavačová, Monika Kaldarárová, Iveta Šimková
Congenital agenesis of the portal vein (CAPV) as a result of abnormal early embryogenesis is very rare. This condition leads to an abnormal blood flow directly from the portal to systemic circulation via persistent ductus venosus or other congenital portosystemic shunt. Portopulmonary hypertension (PPH) is a relatively isolated complication of portal hypertension with or without associated liver disease. Most articles deal with PPH related to liver cirhosis; cases of PPH due to congenital agenesis of the portal vein are published less frequently. Pathophysiology, clinical presentation, diagnosis and treatment of PPH due to CAPV is to a great extend modified not only by the degree of pulmonary hypertension but also by the severity of liver affection; as well as by the type of portosystemic shunt and other possible congenital anomalies. The management of this complex disease is therefore multidisciplinary, in the hands of experts from several disciplines. A case of a 31-year old male patient with congenital agenesis of the portal vein with portosystemic shunts leading to severe portopulmonary hypertension, is described.