Treatment of Portopulmonary Hypertension (PoPH): A Review

• All classes of PAH targeted therapy are currently used in PoPH • Targeted therapy in PoPH is effective in improving hemodynamic disease severity • Targeted therapy in PoPH can sometimes unmask HPS • Liver transplantation improves hemodynamic disease severity in most PoPH patients • Liver transplantation can resolve PoPH in some patients • PAH can develop de-novo following liver transplantation in some patients • The precise predictors of treatment and transplant response in PoPH remain unknown • The best combination of targeted therapy and liver transplant in PoPH is unknown Portopulmonary Hypertension (PoPH) is a type of pulmonary arterial hypertension that uniquely afflicting patients with underlying liver disease and portal hypertension. The mainstay of treatment centers around the use of targeted pulmonary vasodilator therapy and evaluation for liver transplantation. This review article focuses on the role of targeted pulmonary vasodilator therapy and liver transplantation in the management of PoPH, with special emphasis on the expected hemodynamic and clinical effects of treatment. The relationship between PoPH and Hepatopulmonary Syndrome (HPS), another type of pulmonary vascular disease due to liver disease, is also reviewed.