Portopulmonary Hypertension: An Effect of Vasomodulation and Mortality Post-Transplant

Abstract

Introduction: Portopulmonary hypertension (PPH) complicates the perioperative conditions in patients who anticipate orthotopic liver transplant (OLT). PPH is rare-occurring in only 6% of those referred for OLT. There is decrease in survival with the moderate to severe PPH subgroup with OLT. Overall only a third live to 5 years time after transplant. Treatment of PPH with vasomodulative therapies before transplantation has increased survival both pre- and post-transplant. The purpose of this study is to report the mortality, prevalence of vasomodulator therapy in PPH before and after OLT depending on group severity. 

Methods: This is a retrospective cohort study conducted from our liver transplant database over the course of 15 years. Patients were selected if they were diagnosed with PPH and received OLT. There pulmonary pressures were recorded preoperatively thereby categorizing patients into severity stages. Patient age, ethnicity, gender, BMI, and concurrent comorbidities were also included. The outcomes were overall and group-specific survival, and vasomodulator therapy types used. 

Results: The most common cause of liver failure in both groups was Hepatitis C and alcohol-induced cirrhosis. Overall mortality was 50%. There was an increase in use of sildenafil and epoprostenol post-transplant in the PPH group. Forty-percent of transplanted patients lived to more than 1200 day end-point. Patients in moderate to severe had a sustained survival rate from years 1-5 post-transplant. 

Conclusions: Patients with pulmonary vascular disease have a complicated liver transplant course specifically with moderate to severe PPH. This retrospective study suggests that in the setting of PPH and liver transplant, mortality is greatly improved after OLT with long-term treatment with vasomodulators, specifically those with moderate-severe disease.