Portopulmonary Hypertension (Literature Review)

Abstract

Portopulmonary hypertension is a form of pulmonary arterial hypertension that develops as a complication of portal hypertension. The specific gravity of liver cirrhosis as a cause of portal hypertension is about 75%, which means that a significant proportion of patients with portopulmonary hypertension have liver cirrhosis, therefore, these patients are potentially candidates for liver transplantation. Although this complication is not common, it is extremely important to distinguish portopulmonary hypertension from other causes of pulmonary arterial hypertension, since an increase in mean pulmonary arterial pressure > 35 mmHg is associated with a 50-100% mortality rate during waiting and after liver transplantation. There are no clear recommendations for the treatment of this complication in patients with portal hypertension, since there are not enough clinical trials in this group of patients. A significant number of obstacles can limit the adequate treatment of patients with portopulmonary hypertension and explain the lower survival rate of this group of patients compared with other types of pulmonary arterial hypertension. Until recently, only one randomized controlled trial included patients with portopulmonary hypertension, and most of the treatment data came from relatively small observational studies. Currently, the treatment of portopulmonary hypertension includes therapy specific for pulmonary arterial hypertension regardless of its cause, and in some cases such therapy is necessary to facilitate successful liver transplantation.