PT  - JOURNAL ARTICLE
AU  - P. Escribano-Subias
AU  - I. Blanco
AU  - M. López-Meseguer
AU  - C. Jimenez Lopez-Guarch
AU  - A. Roman
AU  - P. Morales
AU  - M.J. Castillo-Palma
AU  - J. Segovia
AU  - M.A. Gómez-Sanchez
AU  - J.A. Barberà
AU  - on behalf of the REHAP investigators
TI  - Survival in pulmonary hypertension in spain insights from the spanish registry
AID  - 10.1183/09031936.00101211
DP  - 2012 Jan 01
TA  - European Respiratory Journal
PG  - erj01012-2011
4099  - http://erj.ersjournals.com/content/early/2012/02/22/09031936.00101211.short
4100  - http://erj.ersjournals.com/content/early/2012/02/22/09031936.00101211.full
AB  - A pulmonary hypertension (PH) registry (REHAP) was undertaken to analyse prevalence, incidence, and survival of pulmonary arterial hypertension (PAH) and chronic-thromboembolic PH (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations.Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (Jul/2007–Jun/2008). Demographic, functional and hemodynamic variables were evaluated.866 patients with PAH and 162 with CTEPH were included. PAH associated with toxic oil syndrome and pulmonary veno-occlusive disease were reported for the first time in a PAH registry. Estimated prevalences: PAH, 16 and CTEPH, 3.2 cases/million adult inhabitants (MAI); estimated incidences: PAH, 3.7 and CTEPH, 0.9 cases/MAI·yr−1. One-, 3- and 5 yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male gender, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and predicted by different equations was closer when characteristics of the cohorts were similar.Epidemiology and survival of PAH in the Spanish registry are similar to recent registries. Characteristics of the population from which survival prediction equations are derived influence their applicability to a different cohort. CTEPH is much less prevalent than PAH, although with similar survival rates.