Clinical Outcomes After Liver Transplantation in Patients With Portopulmonary Hypertension

Abstract

Background: Portopulmonary hypertension (POPH) is the presence of pulmonary arterial (PA) hypertension in patients with portal hypertension and is associated with significant morbidity and mortality. In a cohort of POPH patients, we describe the clinical outcomes of POPH patients who underwent liver transplantation (LT). Methods: Retrospectively collected data from a prospectively assembled cohort of all consecutive POPH adults evaluated in three transplant centers from 1996 to 2019. Results: From a cohort of 228 POPH patients, 50 patients underwent LT. Significant hemodynamic improvement after PA-targeted therapy was observed, with 58% receiving only monotherapy pre-transplant. After LT, 21 (42%) patients were able to discontinue and remained off PA-targeted therapy. The 1, 3, and 5 year unadjusted survival rates after LT were 72%, 63% and 60%, respectively. An elevated pulmonary vascular resistance (PVR) before LT was associated with worse survival rate (HR 1.91, 95% CI 1.07-3.74, p=0.04). No survival difference was observed in those granted MELD exception or transplants performed before or after the year 2010. Conclusion: Significant number of POPH patients discontinued PA-targeted therapy after LT. Higher PVR before LT was associated with worse survival, as was monotherapy use. Despite effective PA-targeted therapies, POPH survival outcomes after LT in our cohort were modest and may reflect the need for more aggressive therapy.