Portopulmonary hypertension: peculiarities of diagnosis and treatment

Portopulmonary hypertension is defined as the development of pulmonary hypertension secondary to portal one. Its exact prevalence is difficult to determine due to the lack of routine screening in patients with portal hypertension. Hemodynamic changes associated with portal hypertension, including the hyperdynamic state, portosystemic shunts, and splanchnic vasodilation, cause significant disturbances in the pulmonary vasculature and play a key role in the pathogenesis of the disease. Without treatment, portopulmonary hypertension leads to progressive right ventricular failure with a poor prognosis. Although Doppler echocardiography is the best initial screening tool for symptomatic patients and candidates for liver transplant, right heart catheterization remains the gold standard for disease diagnosis. Treatment of patients with portopulmonary hypertension is aimed at improving cardiac function, reducing pulmonary vascular resistance, and optimizing functional capacity. Pulmonary hypertension-specific therapy, which includes prostacyclin and its receptor agonists, endothelin receptor antagonists, phosphodiesterase inhibitors, and guanylate cyclase stimulators, plays a key role in the treatment of patients with portopulmonary hypertension. Small uncontrolled and recent single randomized controlled trials have reported promising results of vasodilator therapy in terms of clinical and hemodynamic improvement in patients, allowing certain patients to undergo liver transplantation. This review discusses the epidemiology, approach to diagnosis and treatment of patients with portopulmonary hypertension. We used MEDLINE database on the PubMed platform and the Cochrane library to search for literature sources using the keywords: portopulmonary hypertension, portal hypertension, pulmonary hypertension, liver cirrhosis, pulmonary complications.