The pulmonary involvement in portal hypertension: portopulmonary hypertension and hepatopulmonary syndrome.

Pulmonary abnormalities are common in patients with advanced chronic liver disease. Two distinct syndromes strictly related to the presence of portal hypertension, but clearly different from a pathophysiologic point of view, have been identified. Portopulmonary hypertension, characterized by an increased pulmonary arterial pressure, is due to a progressive arteriolar vasoconstriction induced by excess local production of vasoconstrictor substances. Hepatopulmonary syndrome results from intrapulmonary microvascular dilation caused by an inadequate synthesis or metabolism of putative pulmonary vasoactive substances leading to a functional vasodilation of the pulmonary vasculature, ultimately leading to hypoxemia. Controversies on pathogenesis imply different tentative therapeutic approaches for the medical management of these conditions. The development of portopulmonary hypertension or the hepatopulmonary syndrome has important clinical and prognostic implications facing the impact of new therapeutic strategies for the management of the main complications of advanced liver diseases on cardiopulmonary function.