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.

Long-term data from the Swiss pulmonary hypertension registry

“…The overall survival in nonoperated CTEPH patients was lower compared with cohorts reported from Sheffield or Spain, where half of the patients were operated [7,10] , but comparable to nonoperated patients [7,24] . This clearly indicates that Switzerland has to make a clear effort to get more patients operated.…”

“…Both the mPAP and the cardiac output were significantly higher in PAH compared with CTEPH, resulting in a similar PVR. A higher cardiac output in PAH than in CTEPH was also found in the ASPIRE and Spanish registries [7,10] . The arterial oxygen saturation was significantly lower in CTEPH than PAH and lowest in PH due to lung diseases.…”

“…This should be analyzed in the future. The percentage of nonoperated CTEPH patients is herewith strikingly higher compared with other registries and warrants medical action [7,10,11] . One reason might be the absence of a dedicated pulmonary endarterectomy center during that time in Switzerland, a small country with 8 million inhabitants potentially below the critical yearly case number.…”

“…The first registry providing data on primary PH, today named idiopathic PAH, was the NIH registry including 194 patients from 1981 to 1988 in 32 US centers [15] . Other nationwide registries followed, mainly including patients with PAH or CTEPH, with follow-up periods up to 8 years [8,10,16,17] . The first long-term registry on different PH groups was the ASPIRE registry, a singlecenter registry from Sheffield with a follow-up of up to 10 years [7] .…”

“…Registries have given information on some aspects of the disease in different parts of the world, but only few registries included patients with different PH classifications [7][8][9][10][11][12][13][14] . The first registry providing data on primary PH, today named idiopathic PAH, was the NIH registry including 194 patients from 1981 to 1988 in 32 US centers [15] .…”