Clinical management of portopulmonary hypertension

Background: Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH. Data sources: An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension. Results: Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH. Conclusions: After an accurate diagnosis of PPH, treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.