PULMONARY VASCULAR DISORDERS IN PORTAL-HYPERTENSION

The wide spectrum of pulmonary vascular disorders in liver disease and portal hypertension ranges from the hepatopulmonary syndrome characte rized by intrapulmonary vascular dilatations, to pulmonary hypertensio n (portopulmonary hypertension), in which pulmonary vascular resistanc e is elevated, Since hepatopulmonary syndrome and portopulmonary hyper tension have been reported in patients with nonhepatic portal hyperten sion,the common factor that determines their development must be porta l hypertension. The clinical presentations are very different, with ga s exchange impairment in the hepatopulmonary syndrome and haemodynamic failure in portopulmonary hypertension. The severity of hepatopulmona ry syndrome stems to parallel the severity of liver failure, whereas n o simple relationship has been identified between hepatic impairment a nd the severity of portopulmonary hypertension. Resolution of hepatopu lmonary syndrome is common after liver transplantation, which has an u ncertain effect in portopulmonary hypertension. The pathophysiology of both syndromes may involve vasoactive mediators and angiogenic factor s.