PT - JOURNAL ARTICLE AU - P Herve AU - D Lebrec AU - F Brenot AU - G Simonneau AU - M Humbert AU - O Sitbon AU - P Duroux TI - Pulmonary vascular disorders in portal hypertension AID - 10.1183/09031936.98.11051153 DP - 1998 May 01 TA - European Respiratory Journal PG - 1153--1166 VI - 11 IP - 5 4099 - http://erj.ersjournals.com/content/11/5/1153.short 4100 - http://erj.ersjournals.com/content/11/5/1153.full SO - Eur Respir J1998 May 01; 11 AB - The wide spectrum of pulmonary vascular disorders in liver disease and portal hypertension ranges from the hepatopulmonary syndrome characterized by intrapulmonary vascular dilatations, to pulmonary hypertension (portopulmonary hypertension), in which pulmonary vascular resistance is elevated. Since hepatopulmonary syndrome and portopulmonary hypertension have been reported in patients with nonhepatic portal hypertension, the common factor that determines their development must be portal hypertension. The clinical presentations are very different, with gas exchange impairment in the hepatopulmonary syndrome and haemodynamic failure in portopulmonary hypertension. The severity of hepatopulmonary syndrome seems to parallel the severity of liver failure, whereas no simple relationship has been identified between hepatic impairment and the severity of portopulmonary hypertension. Resolution of hepatopulmonary syndrome is common after liver transplantation, which has an uncertain effect in portopulmonary hypertension. The pathophysiology of both syndromes may involve vasoactive mediators and angiogenic factors.