A critical analysis of survival in idiopathic pulmonary arterial hypertension
A critical analysis of survival in idiopathic pulmonary arterial hypertension
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DOI：
10.1016/s0755-4982(10)70006-3
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发表时间：
2010-06-01
期刊：
影响因子：
2.7
通讯作者：
Humbert, Marc
中科院分区：
文献类型：
--
作者：
Humbert, Marc
特发性肺动脉高压(PAH)是一种罕见的疾病，其特征是肺血管阻力的进行性增加导致右心衰竭和死亡。最近在PAH队列中的生存数据被纳入的流行患者(“幸存者”)所偏颇，这些患者的预后好于发病病例。然而，对最近的多中心事件队列的批判性分析表明，由于更好的管理，在当前的管理时代，存活率有所改善。然而，特发性PAH仍然是一种严重的疾病，预后很差。目前的建议强调了对症状性PAH进行积极的以目标为导向的管理的必要性。现代的存活率数字也强调了肺移植与符合条件的PAH患者的相关性，这些患者无法达到目前最好的治疗标准。
Idiopathic pulmonary arterial hypertension (PAH) is a rare disease characterized by a progressive increase in pulmonary vascular resistance leading to right heart failure and death.Recent survival data in PAH cohorts have been biased by the inclusion of prevalent patients ("survivors") who have a better prognosis than incident cases. Nevertheless, a critical analysis of recent multicenter incident cohorts has shown survival improvements in the current management era, thanks to better management.However idiopathic PAH remains a severe disease with a poor prognosis. Current recommendations emphasize the necessity of an aggressive goal-oriented management of symptomatic PAH. Modern survival figures also highlight the relevance of lung transplantation in eligible PAH patients refractory to current best standard of care.