Associated with a mean pulmonary artery pressure of 25 mm Hg on rest with a pulmonary capillary wedge pressure less than 15 mm Hg, a pulmonary vascular resistance greater than 120 dynes/second/cm-5 and the presence of portal hypertension without any other secondary cause of pulmonary hypertension.
Clinically resembles primary pulmonary hypertension.
Treatments include calcium channel blockers and bosentan.
Liver transplantation may reverse minor or moderate pulmonary hypertension, but is contraindicated with severe disease with pulmonary artery pressures above 40-45 mm Hg because of high mortality rates.