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

Sithamparanathan, S. et al. (2017) Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry. Journal of Heart and Lung Transplantation, 36(7), pp. 770-779. (doi: 10.1016/j.healun.2016.12.014) (PMID:28190786)

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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.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Peacock, Professor Andrew and Johnson, Dr Martin
Authors: Sithamparanathan, S., Nair, A., Thirugnanasothy, L., Coghlan, J. G., Condliffe, R., Dimopoulos, K., Elliot, C. A., Fisher, A. J., Gaine, S., Gibbs, J. S. R., Gatzoulis, M. A., Handler, C. E., Howard, L. S., Johnson, M., Kiely, D. G., Lordan, J. L., Peacock, A. J., Pepke-Zaba, J., Schreiber, B. E., Sheares, K. K.K., Wort, S. J., and Corris, P. A.
College/School:College of Medical Veterinary and Life Sciences > School of Cardiovascular & Metabolic Health
Journal Name:Journal of Heart and Lung Transplantation
Publisher:Elsevier
ISSN:1053-2498
ISSN (Online):1557-3117
Published Online:31 December 2016

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