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Portopulmonary Hypertension in Nontransplanted Patients: Results of the Largest US Single-Institution Registry

Published:November 03, 2022DOI:https://doi.org/10.1016/j.mayocp.2022.08.020

      Abstract

      Objective

      To explore clinical characteristics, risk profiles, and outcomes of patients with portopulmonary hypertension (PoPH) who have contraindications to liver transplant (LT).

      Methods

      From the largest US single-institution registry of patients with PoPH, we analyzed 160 patients who did not receive LT between 1988 to 2019. Pulmonary arterial hypertension (PAH)–pertinent characteristics, hemodynamic features, treatments, and risk stratification were compared at baseline, first follow-up visit, and censor/death time.

      Results

      Median survival for the entire cohort was 27.5 months from the diagnosis of PoPH. Overall survival was 89%, 77%, 51%, and 38% at 6 months, 1 year, 3 years, and 5 years, respectively. Survival was significantly affected by the severity of liver disease (P<.001). Most patients received PAH-specific therapies (136 [85%]), predominantly monotherapy (123 [77%)]. With treatment, significant improvements were noted in World Health Organization functional class (P=.04), 6-minute walk distance (P<.001), right ventricular function (P<.001), pulmonary vascular resistance (P<.001), and Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) Lite 2 score (P=.02) univariately. Per European Society of Cardiology risk stratification, no patient met full criteria for low risk at baseline or at follow-up. In a multivariate Cox risk model, 6-minute walk distance, right atrial pressure, pulmonary capillary wedge pressure, bilirubin level, and Model for End-Stage Liver Disease–sodium score of 15 or higher were associated with increased risk of death.

      Conclusion

      Patients with PoPH who did not undergo LT had a poor prognosis. This persisted despite use of PAH-specific therapies and significant improvements in hemodynamics, echocardiography parameters of right ventricle function, 6-minute walk distance, and World Health Organization functional class.

      Abbreviations and Acronyms:

      BNP (brain natriuretic peptide), ESC (European Society of Cardiology), LT (liver transplant), MELD-Na (Model for End-Stage Liver Disease–sodium), mPAP (mean pulmonary artery pressure), PAH (pulmonary arterial hypertension), PCWP (pulmonary capillary wedge pressure), PDE-5 (phosphodiesterase type 5), PoPH (portopulmonary hypertension), PVR (pulmonary vascular resistance), RHC (right-sided heart catheterization), REVEAL (Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management), WHO (World Health Organization)
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