Portopulmonary hypertension (PPH) is characterized by the development of pulmonary arterial hypertension (PAH) associated with portal hypertension, with or without liver disease. It is defined as a mean pulmonary artery pressure (MPAP) greater than 25 mmHg, pulmonary vascular resistance (PVR) above 240 dynes.s.cm-5, pulmonary artery occlusion pressure (PAOP) normal when less than 15 mmHg or transpulmonary gradient (TPG) > 10 mmHg. In the pulmonary hypertension classification PPH is classified in Group I. Pulmonary arterial hypertension in association with cirrhosis and portal hypertension is underdiagnosed. Epidemiological studies estimated that about 2–6% of patients with portal hypertension develop PPH. Mortality is directly proportional to measured MPAP and PVR. Mean pulmonary artery pressure is an independent predictor of mortality, and many centers consider that values greater than 50 mmHg is an absolute contraindication to liver transplantation (LT). The aim of the review is to explore the current aspects of PPH relative to concept, diagnosis, and treatment.
Part of the book: Frontiers in Transplantology