Portopulmonary Hypertension

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. We performed a review of the published literature to summarize the available evidence and guidelines regarding the diagnosis and management of POPH. POPH is defined by the presence of precapillary pulmonary hypertension in the context of portal hypertension. POPH is associated with increased perioperative risk at the time of LT, which can be stratified by mean pulmonary arterial pressure and pulmonary vascular resistance. Screening with echocardiography is recommended in all LT candidates to facilitate detection and treatment of POPH. Despite a paucity of evidence, POPH is treated similarly to idiopathic PAH with PAH therapy. These therapies are associated with improved pulmonary hemodynamics and facilitation of safe LT. 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. 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. We performed a review of the published literature to summarize the available evidence and guidelines regarding the diagnosis and management of POPH. POPH is defined by the presence of precapillary pulmonary hypertension in the context of portal hypertension. POPH is associated with increased perioperative risk at the time of LT, which can be stratified by mean pulmonary arterial pressure and pulmonary vascular resistance. Screening with echocardiography is recommended in all LT candidates to facilitate detection and treatment of POPH. Despite a paucity of evidence, POPH is treated similarly to idiopathic PAH with PAH therapy. These therapies are associated with improved pulmonary hemodynamics and facilitation of safe LT. 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.