Clinical Outcomes After Liver Transplantation in Patients With Portopulmonary Hypertension

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.