Toggle Main Menu Toggle Search

Open Access padlockePrints

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

Lookup NU author(s): Sasiharan Sithamparanathan, Dr Logan Thirugnanasothy, Professor Andrew Fisher, Dr James Lordan, Emeritus Professor Paul Corris


Full text for this publication is not currently held within this repository. Alternative links are provided below where available.


© 2017 International Society for Heart and Lung Transplantation. 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.

Publication metadata

Author(s): Sithamparanathan S, Nair A, Thirugnanasothy L, Coghlan JG, Condliffe R, Dimopoulos K, Elliot CA, Fisher AJ, Gaine S, Gibbs JSR, Gatzoulis MA, E Handler C, Howard LS, Johnson M, Kiely DG, Lordan JL, Peacock AJ, Pepke-Zaba J, Schreiber BE, Sheares KKK, Wort SJ, Corris PA

Publication type: Article

Publication status: Published

Journal: Journal of Heart and Lung Transplantation

Year: 2017

Volume: 36

Issue: 7

Pages: 770-779

Print publication date: 01/07/2017

Online publication date: 31/12/2016

Acceptance date: 02/04/2016

ISSN (print): 1053-2498

ISSN (electronic): 1557-3117

Publisher: Elsevier USA


DOI: 10.1016/j.healun.2016.12.014


Altmetrics provided by Altmetric


Find at Newcastle University icon    Link to this publication