[Porto-pulmonary hypertension]

Abstract

Introduction: Porto-pulmonary hypertension (PoPH) is the association of pulmonary artery hypertension and portal hypertension. The diagnosis of PoPH is based on pulmonary haemodynamic criteria, obtained via right heart catheterisation, including an increase in mean pulmonary arterial pressure (> 25 mmHg) and in pulmonary vascular resistance (> 240 dyn.s.cm-5).

State of the art: The exact pathophysiological mechanisms of PoPH are unknown. However, since PoPH has been reported in patients with non-hepatic portal hypertension, the factor that determines the development must be portal hypertension rather than liver disease per se. Moreover, no simple relationship has been identified between the degree of hepatic impairment and the severity of PoPH. The clinical presentation is non-specific with haemodynamic failure occurring at the end stage. As a consequence, screening by annual transthoracic echocardiography is highly recommended in potential liver transplant candidates. Therapy with prostacyclin analogues may partially relieve pulmonary arterial hypertension (PAH). Liver transplantation has an uncertain effect in PoPH and because PoPH is associated with a high perioperative mortality, moderate to severe PoPH remains a contraindication for liver transplantation.

Perspectives and conclusions: Recent advances in the management of PoPH have improved the prognosis. The safety and efficacy of oral endothelin receptor antagonists and oral phosphodiesterase inhibitors is currently under evaluation. A therapeutic approach utilising combinations of drugs should provide better long-term results.