Impact of medical therapy on liver transplant eligibility in patients with portopulmonary hypertension: a systematic review.

Introduction: Portopulmonary hypertension (PoPH) occurs in roughly 5% of patients evaluated for liver transplant and increases perioperative morbidity and mortality. PoPH patients often succumb to complications of the underlying liver disease, therefore liver transplantation can be a lifesaving intervention. Medical treatment of PoPH optimizes patients for transplantation, but the knowledge on the effect of PH specific therapy on transplant eligibility is limited.

Evidence acquisition: We searched Medline, Embase, and Cochrane Library from inception to August 19, 2024. We aimed to include studies of adult patients with PoPH treated with pharmacologic therapies with comparison to placebo, baseline condition of the patient, or another medication. We only included studies where liver transplantation outcomes were reported.

Evidence synthesis: Out of 606 studies retrieved, 17, all observational cohorts, met inclusion criteria. None of the included studies had suitable control or comparison groups to assess medication efficacy in a pooled fashion. As a result, this systematic review aimed to describe the effect of PoPH treatments on transplant eligibility across studies and medication classes. A total of 812 patients received medical treatment, with 52% receiving liver transplant. Of studies reporting transplant eligibility, 65% of treated PoPH patients became transplant eligible.

Conclusions: Within the limitations of the available data, PoPH therapies improve transplant eligibility. Prospective studies, especially RCTs, are needed to better define the best treatment strategy in this population.