"Porto-pulmonary hypertension: a comprehensive review"

Abstract

Porto-pulmonary hypertension (PoPH) is a rare but threatening vasculopathy, defined by the presence of pulmonary arterial hypertension (PAH) in the setting of portal hypertension. Although most commonly observed in cirrhotic patients, those with non-cirrhotic portal hypertension are also at risk of developing it. Little is known about the mechanisms by which PAH develop in patients with portal hypertension, but genetic factors, pulmonary vascular wall shear stress, and a dysregulation of vasoactive, proliferative and inflammatory mediators might be involved. PoPH is estimated to occur in 3 to 10% of patients with end-stage liver disease, although its frequency is not related to the severity of liver dysfunction or the degree of portal hypertension. Moderate-to-severe PoPH portends an extremely poor prognosis. Presentation is highly variable, therefore a high index of suspicion is required to establish the diagnosis. PoPH should be screened by transthoracic echocardiography (TTE) in cirrhotic patients presenting with dyspnoea as well as in all patients being evaluated for liver transplantation (LT) regardless of their symptoms. If TTE shows elevated pulmonary pressures, patients should undergo right heart catheterisation, which is required for the definitive diagnosis of PoPH. Without LT, the overall 5-year mortality in PoPH patients is 70%, but it should not be considered an indication for LT. Moderate-to-severe PoPH contraindicates LT, since it is associated with a prohibitively increased intra and postoperative mortality. However, there is now evidence supporting the use of PAH-specific therapies pre-LT in order to improve pulmonary haemodynamic measurements, so the procedure can then be performed with significantly lower risks.