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Portopulmonary hypertension (POPH) affects 5% to 6% of patients with advanced liver disease and accounts for 5% to 15% of pulmonary arterial hypertension (PAH) cases. Compared with idiopathic PAH, POPH is associated with significantly worse survival. Recent studies have improved our understanding of the role of both PAH therapy and liver transplantation (LT) in the management of POPH and their impact on overall prognosis. ⋯ LT can result in improvement or resolution of POPH in one-half of patients and has been associated with improved survival in highly selected patients. In summary, the prognosis in POPH is poor and is impacted by the severity of both pulmonary hypertension and liver disease. Management with a combination of PAH therapy and LT in selected patients has been associated with improved pulmonary hemodynamics and survival.