Open AccessPortopulmonary hypertension
Author(s) -
Sarfraz Saleemi
Publication year - 2010
Publication title -
annals of thoracic medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.639
H-Index - 33
eISSN - 1817-1737
pISSN - 1998-3557
DOI - 10.4103/1817-1737.58953
Subject(s) - portopulmonary hypertension , medicine , portal hypertension , pulmonary hypertension , cardiology , disease , liver disease , pulmonary artery , pathological , liver transplantation , cirrhosis , transplantation
Portopulmoanry hypertension (POPH) is a form of pulmonary arterial hypertension (PAH) associated with portal hypertension with or without underlying chronic liver disease. POPH is increasingly recognized and recent evidence suggests that it is one of the leading causes of PAH. The pathophysiology of POPH is poorly understood although the pathological changes in pulmonary vasculature in advanced POPH are similar to those seen in idiopathic pulmonary hypertension. The prognosis in patients with liver disease who also suffer from significant POPH is considered to be poor. Higher degree of pulmonary artery pressure (PAP) may preclude a patient from liver transplant as mortality in these patients is high. The treatment with vasodilator therapy has shown to improve both hemodynamics and clinical outcome in POPH in retrospective studies and in some case series. The aim of medical management is to bring PAP <35 mmHg that may make a patient with POPH and advanced liver disease eligible for liver transplant, which otherwise would have been denied because of high PAP.