Portopulmonary hypertension

The article is devoted to the review of literature data on portopulmonary hypertension. The data on the occurrence of this pathology among people with liver diseases, as well as among patients with pulmonary hypertension are presented. Modern concepts of the pathogenesis of the disease, including genetic disorders, are described. Treatment strategies for portopulmonary hypertension at the present stage coincide with those for pulmonary arterial hypertension, but further studies are needed to assess the effectiveness of various drugs in this pathology. Mortality in portopulmonary hypertension is higher than in pulmonary hypertension. Combination of drug therapy with liver transplantation leads to a more favorable prognosis than either of these options alone. The decision to perform a transplant requires the work of a multidisciplinary team of specialists.