PT  - JOURNAL ARTICLE
AU  - Pilar Escribano-Subias
AU  - Isabel Blanco
AU  - Manuel López-Meseguer
AU  - Carmen Jimenez Lopez-Guarch
AU  - Antonio Roman
AU  - Pilar Morales
AU  - María Jesús Castillo-Palma
AU  - Javier Segovia
AU  - Miguel A. Gómez-Sanchez
AU  - Joan Albert Barberà
TI  - Survival in pulmonary hypertension in Spain: insights from the Spanish registry
AID  - 10.1183/09031936.00101211
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - 596--603
VI  - 40
IP  - 3
4099  - http://erj.ersjournals.com/content/40/3/596.short
4100  - http://erj.ersjournals.com/content/40/3/596.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - 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.