Liver Transplantation in Hepatopulmonary Syndrome
Keywords:
Liver Transplantation, Hepatopulmonary Syndrome, LiverAbstract
Introduction: Hepatopulmonary syndrome (HPS) results from the association between liver disease, intrapulmonary vascular dilations, and hypoxemia, representing a relevant complication of cirrhosis. Liver transplantation (LT) is the only curative treatment; however, patients with HPS present a higher risk of mortality while on the waiting list, which is why they receive exception points in the Model for End-Stage Liver Disease (MELD) score. Objectives: To evaluate whether LT improves prognosis and survival in patients with HPS. Methods: This is an integrative literature review. The search was conducted in PubMed, LILACS, and Cochrane databases using the descriptors “Liver transplantation,” “Hepatopulmonary syndrome,” and “Liver.” Of the 272 articles identified, 13 met the eligibility criteria and were included in the final analysis. Data extraction included demographic and clinical characteristics, such as mean age, sex distribution, main etiologies of liver disease, mean MELD/Pediatric End‐Stage Liver Disease (PELD) score, and mean PaO₂ and PaCO₂ values. Results: Patients with PaO₂ < 45 mmHg were more likely to be prioritized on the transplant waiting list (hazard ratio [HR] 1.51; p = 0.007), although they also showed lower post-transplant survival compared with those with PaO₂ ≥ 45 mmHg. In the pediatric context, data from an observational study conducted in the United States indicated that children with HPS had a higher risk of mortality after LT compared with those without HPS. In a prospective cohort study, transplanted patients with HPS remained on mechanical ventilation for an average of 19.5 ± 4.3 hours, significantly longer than the 12.5 ± 3.3 hours observed in patients without HPS. Conclusion: LT remains the only curative therapy for HPS. The severity of hypoxemia is associated with worse post-transplant outcomes, highlighting the need for proper stratification of HPS. However, although useful for estimating liver-related mortality, the MELD/PELD score does not accurately reflect the severity of this syndrome.
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Copyright (c) 2025 Maria Clara Santini, Olival Lucena, Bruna Schwambach, Laura Barbosa, Letícia Cardoso, Luana Ribeiro, Sofia Andrade
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