Portopulmonary hypertension in the current era of pulmonary hypertension management

Abstract Background & Aims Long-term outcomes in portopulmonary hypertension (PoPH) are poorly studied in the current management era of pulmonary hypertension. We analysed the effect of pulmonary arterial hypertension (PAH)-targeted therapies, the survival and the predictors of death in a large contemporary cohort of patients with PoPH. Methods Data from patients with PoPH consecutively enrolled in the French Pulmonary Hypertension Registry between 2007 and 2017 were collected. Effect of initial treatment strategies on functional class, exercise capacity and cardiopulmonary haemodynamics were analysed. Survival and its association with PAH- and hepatic-related characteristics were also examined. Results Six hundred and thirty-seven patients (mean age 55±10 years; 58% male) were included. Fifty seven percent had mild cirrhosis, i.e. Child-Pugh (CP) stage A. The median MELD score was 11 (Q1-Q3; 9-15). Most patients (n=474; 74%) were initiated on monotherapy, either with a phosphodiesterase-5 inhibitor (n=336) or with an endothelin-receptor antagonist (n=128); 95 (15%) were initiated on double oral combination therapy and 5 (1%) on triple therapy. After a median treatment time of 4.5 months, there were significant improvements in functional class (p Conclusion Survival of patients with PoPH is strongly associated with the severity of liver disease. Patients who underwent liver transplantation had the best long-term outcomes.