Survival in portopulmonary hypertension: Outcomes of the united kingdom national pulmonary arterial hypertension registry

Rationale Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival and the effect of modern pulmonary arterial hypertension (PAH) targeted therapies on long term outcome is unknown. Objectives To investigate the baseline characteristics and survival in a cohort of all patients diagnosed with PoPH in the United Kingdom Pulmonary Hypertension service. Methods Retrospective review of all incident treatment naïve cases of PoPH within the UK national registry diagnosed between January 2001 and December 2010. Measurements and Main Results Patients with PoPH (n =110) had 1, 3 and 5 year survival rate of 85, 60, and 35%. The prevalence of PoPH was 0.85 cases per 1 million. Mean age at diagnosis was 53 ± 12 yrs, with a balanced distribution in gender. Alcohol (n=57) and hepatitis C (n=10) were the most common causes of portal hypertension. Phosphodiesterase V-inhibitors were the most frequently used targeted therapy in 63.6% (n=70) patients, with endothelin receptor antagonists in 10% (n=11) and prostacyclin analogues in 12.7% (n=14). Univariate and multivariate analysis of baseline characteristics did not demonstrate significant influence of severity of portal hypertension or liver cirrhosis, WHO functional class, cardiopulmonary haemodynamics or year of diagnosis on survival. Conclusions Survival of patients with PoPH remains poor despite targeted therapy and worse than patients with idiopathic PAH. The benefit of PAH therapies in PoPH on long-term morbidity and mortality outcomes needs further consideration and study.