Survival in pulmonary hypertension in Spain: insights from the Spanish registry
Survival in pulmonary hypertension in Spain: insights from the Spanish registry
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DOI：
10.1183/09031936.00101211
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发表时间：
2012-09-01
影响因子：
24.3
通讯作者：
Albert Barbera, Joan
中科院分区：
文献类型：
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作者：
Escribano-Subias, Pilar;Blanco, Isabel;Albert Barbera, Joan
肺动脉高压（PH）登记（西班牙肺动脉高压登记）旨在分析西班牙肺动脉高压（PAH）和慢性血栓栓塞性肺动脉高压（CTEPH）的患病率、发病率和生存率，并评估近期生存预测方程的适用性。对先前诊断和事件的PAH或CTEPH病例进行自愿报告（2007年7月2008年6月）。对人口统计学、功能和血流动力学变量进行了评估。纳入了 866 名 PAH 患者和 162 名 CTEPH 患者。多环芳烃登记处首次报道了与毒油综合征和肺静脉闭塞性疾病相关的 PAN。估计患病率如下：PAH，16 和 CTEPH，每百万成人居民 (MAI) 3.2 例。估计发病率如下：PAH，3.7 例；CTEPH，每年每 MAI 0.9 例。 PAH 和 CTEPH 的 1 年、3 年和 5 年生存率分别为 87%、75% 和 65%。男性、右心房压和心脏指数是死亡的独立预测因素。当队列特征相似时，观察到的生存率与通过不同方程预测的生存率之间的匹配更接近。西班牙登记处的 PAH 患者的流行病学和生存率与最近的登记处相似。得出生存预测方程的群体特征会影响其对不同群体的适用性。尽管 CTEPH 的存活率与 PAN 相似，但其发病率远低于 PAN。
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. PAN 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 PAN, although has a similar survival rate.