Abstract Rationale Portopulmonary hypertension (PoPH) affects approximately 2-10% of liver transplant (LT) candidates and represents a severe subtype of pulmonary vascular disease. Historically, outcomes after LT were poor, with five-year survival rates of only 4-14%. The advent of targeted pulmonary arterial hypertension (PAH) therapies and advances in perioperative management have transformed PoPH from a contraindication to a potential indication for transplantation in selected patients. We conducted a PRISMA-based systematic review to evaluate post-transplant survival, hemodynamic predictors, and the role of continued vasodilator therapy in adults with PoPH. Methods Following PRISMA 2020 guidelines, a systematic search of PubMed, Scopus, and the Cochrane Library was performed through 2000-2025. Eligible studies included adult PoPH patients who underwent LT and reported post-transplant outcomes. Extracted data included survival rates, pre-LT pulmonary vascular resistance (PVR) and mean pulmonary arterial pressure (mPAP), discontinuation of PAH-specific therapy, and perioperative complications. Studies with overlapping cohorts, insufficient endpoints, or high risk of bias were excluded. Quality appraisal was performed using standardized risk-of-bias tools. Results Twenty studies met inclusion criteria. Post-transplant outcomes demonstrated progressive improvement over time. In optimized single-center cohorts treated with PAH-targeted therapy, one-year and five-year survival reached 95.8% and 90.9%, respectively. Larger registry and multicenter analyses reported one-year survival of 72-86% and five-year survival of 60-75%. Elevated pre-LT PVR consistently predicted higher mortality; one multicenter study reported a hazard ratio of 1.91 (95% CI 1.07-3.74). Among survivors, 40-62% discontinued all PAH-specific therapy within 7-14 months post-LT, suggesting durable hemodynamic improvement.Despite these advances, perioperative morbidity remains significant. Prolonged mechanical ventilation (48 h) was associated with a 30-day mortality of 26.5%, compared with 1.4% among non-ventilated patients. The mean hospital stay averaged 42 ± 39 days, reflecting high resource utilization. Heterogeneity in study design and reporting precluded formal meta-analysis. Conclusions Once an absolute contraindication, PoPH is now a potential indication for LT in therapy-responsive patients following hemodynamic optimization. Lower pre-LT PVR and favorable response to PAH therapy predict improved survival. Long-term outcomes now approach those of non-PoPH LT recipients, though early mortality remains driven by perioperative respiratory failure and critical care burden. Multidisciplinary preoperative optimization and meticulous perioperative management are essential to maximize post-transplant survival This abstract is funded by: n/a
Ansari et al. (Fri,) studied this question.
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