TY - JOUR T1 - Survival in pulmonary hypertension in spain insights from the spanish registry JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/09031936.00101211 SP - erj01012-2011 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 Y1 - 2012/01/01 UR - http://erj.ersjournals.com/content/early/2012/02/22/09031936.00101211.abstract N2 - 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. ER -