Management of hepatopulmonary syndrome and portopulmonary hypertension

PURPOSE: Portopulmonary hypertension (PoPH) and hepatopulmonary syndrome (HPS) are two pulmonary vascular diseases, that occur in the context of liver disease, with high morbidity and mortality. The mechanistic link between liver and lung that drives disease pathogenesis in these conditions is not well understood, and although liver transplantation offers benefit for both PoPH and HPS, posttransplant consequences can be severe and result in critical illness. RECENT FINDINGS: Though the mechanisms of PoPH are still obscure, recent work has identified a deficiency of bone morphogenetic protein type 9 as a key characteristic that may drive pulmonary vascular remodeling in HPS. Although it is well established that liver transplantation is beneficial in select PoPH patients, the new ILTS guidelines specify updated pulmonary hemodynamic criteria to determine suitability for transplantation in PoPH. SUMMARY: Targeted pulmonary hypertension therapy is still the cornerstone of management in PoPH. In lieu of liver transplantation in HPS, supplemental oxygen remains the only therapy with proven clinical benefit. Posttransplant critical illness can occur in both PoPH and HPS, through mechanisms that are incompletely understood, with severe consequences for patient survival. Further work understanding PoPH and HPS is necessary to meaningfully improve patient outcomes in these devastating conditions.