In patients with hepatopulmonary syndrome, supplemental oxygen and liver transplantation are the usual treatments of choice. Pharmacologic approaches have limited success in improving hypoxemia. Interventional radiology procedures may improve arterial hypoxemia in highly selected patients. In patients with portopulmonary hypertension, continuous infusion with intravenous epoprostenol (prostaglandin I(2)) can significantly improve pulmonary hemodynamics. Outcome following liver transplantation is variable; increased cardiopulmonary mortality occurs in patients with moderate to severe pulmonary hypertension.