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. 2017 Jul;36(7):770-779.
doi: 10.1016/j.healun.2016.12.014. Epub 2016 Dec 31.

Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry

Sasiharan Sithamparanathan  1 , Arun Nair  2 , Logan Thirugnanasothy  1 , J Gerry Coghlan  3 , Robin Condliffe  4 , Konstantinos Dimopoulos  5 , Charlie A Elliot  4 , Andrew J Fisher  1 , Sean Gaine  6 , J Simon R Gibbs  7 , Michael A Gatzoulis  5 , Clive E Handler  3 , Luke S Howard  7 , Martin Johnson  8 , David G Kiely  4 , James L Lordan  1 , Andrew J Peacock  8 , Joanna Pepke-Zaba  9 , Benjamin E Schreiber  3 , Karen K K Sheares  9 , Stephen J Wort  5 , Paul A Corris  10 ; National Pulmonary Hypertension Service Research Collaboration of the United Kingdom and Ireland
Affiliations

Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry

Sasiharan Sithamparanathan et al. J Heart Lung Transplant. 2017 Jul.

Abstract

Background: Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival, and the effect of modern therapies that target pulmonary arterial hypertension (PAH) on long-term outcome is unknown. This study investigated the baseline characteristics and survival in the cohort of patients diagnosed with PoPH in the United Kingdom National Pulmonary Hypertension Service.

Methods: A retrospective review was conducted of all incident treatment-naïve patients with PoPH within the United Kingdom national registry diagnosed between January 2001 and December 2010.

Results: Patients with PoPH (n = 110) had survival rates of 85%, 60%, and 35% at 1, 3, and 5 years. The prevalence of PoPH was 0.85 cases/1 million. Mean age at diagnosis was 53 ± 12 years, 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) of patients, endothelin receptor antagonists were used in 10% (n = 11) and prostacyclin analogs in 12.7% (n = 14). Univariate and multivariate analysis of baseline characteristics did not demonstrate a significant influence of severity of portal hypertension or liver cirrhosis, World Health Organization Functional Class, cardiopulmonary hemodynamics, 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.

Keywords: PAH-targeted treatment; portal hypertension; portopulmonary hypertension; pulmonary arterial hypertension; survival.

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