title: Survival in pulmonary hypertension in Spain: insights from the Spanish registry.
abstract: A pulmonary hypertension (PH) registry (Spanish Registry of Pulmonary Arterial Hypertension) was undertaken to analyse prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations. Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (July 2007-June 2008) was performed. Demographic, functional and haemodynamic 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 were as follows: PAH, 16 and CTEPH, 3.2 cases per million adult inhabitants (MAI). Estimated incidences were as follows: PAH, 3.7 and CTEPH, 0.9 cases per MAI per yr. 1-, 3- and 5-yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male sex, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and that predicted by different equations was closer when the characteristics of the cohorts were similar. Epidemiology and survival of PAH patients 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 has a similar survival rate.
authors.affiliation: Pulmonary Hypertension Unit, Cardiology Department, Hospital Universitario 12 de Octubre, Complutense University, Madrid, Crtra Andalucia km 5, 400, 28041 Madrid, Spain. pilar.escribano@telefonica.net
authors.forename: Pilar
authors.initials: P
authors.lastname: Escribano-Subias
authors.forename: Isabel
authors.initials: I
authors.lastname: Blanco
authors.forename: Manuel
authors.initials: M
authors.lastname: López-Meseguer
authors.forename: Carmen Jimenez
authors.initials: CJ
authors.lastname: Lopez-Guarch
authors.forename: Antonio
authors.initials: A
authors.lastname: Roman
authors.forename: Pilar
authors.initials: P
authors.lastname: Morales
authors.forename: María Jesús
authors.initials: MJ
authors.lastname: Castillo-Palma
authors.forename: Javier
authors.initials: J
authors.lastname: Segovia
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Gómez-Sanchez
authors.forename: Joan Albert
authors.initials: JA
authors.lastname: Barberà
mesh_terms: D000328:Adult; D000368:Aged; D002908:Chronic Disease; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D008954:Models, Biological; D015995:Prevalence; D011668:Pulmonary Veno-Occlusive Disease / Q000453:epidemiology; D012042:Registries / Q000706:statistics & numerical data; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology; D013923:Thromboembolism / Q000453:epidemiology