The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups
The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups
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DOI：
10.1016/j.healun.2017.02.016
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发表时间：
2017-09-01
影响因子：
8.9
通讯作者：
Ghofrani, Hossein A.
中科院分区：
文献类型：
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作者：
Gall, Henning;Felix, Janine F.;Ghofrani, Hossein A.
背景：肺动脉高压（PH）是一种严重的进行性疾病。尽管已认识到 5 个 PH 亚组，但有关生存率的报告主要集中在肺动脉高压 (PAH) 上。 方法：在德国吉森的单一转诊中心的前瞻性登记中，对 PH 患者（通过右心导管插入术诊断）的长期无移植生存及其决定因素进行了调查。 结果：总共入组了 2,067 名患者（PAH，685 名患者 [33.1%]；肺静脉高压， 307 例患者 [14.9%]；546 例患者为肺疾病（LD-PH）[26.4%；主要为间质性肺病和慢性阻塞性肺疾病]；459 例患者为慢性血栓栓塞性肺动脉高压（22.2%）；由于其他/原因不明的 PH 患者为 70 例（3.4%）。中位随访时间为 37 个月。病因组之间的无移植生存率差异非常显着（p < 0.001），PAH 患者的 1 年、3 年和 5 年生存率分别为 88.2%、72.2% 和 59.4%，而 LD-PH 患者的 1 年、3 年和 5 年生存率分别为 79.5%、52.7% 和 38.1%。在多变量 Cox 回归分析中，患者的年龄、性别和 6 分钟步行距离 (6MWD)，但与纽约心脏协会 (NYHA) 功能分级无关，与所有 PH 亚型的生存率显着相关。结论：这是迄今为止描述的最大的单中心 PH 队列。临床实践中使用的一些参数不能独立预测生存率。在预测所有病因组的生存率方面，年龄、性别和 6MWD 的表现优于 NYHA 功能分级。 (C) 2017 年作者。由爱思唯尔公司出版
BACKGROUND: Pulmonary hypertension (PH) is a severe, progressive disease. Although 5 PH subgroups are recognized, reports on survival have focused mainly on pulmonary arterial hypertension (PAH).METHODS: Long-term transplant-free survival and its determinants were investigated in patients with PH (diagnosed by right heart catheterization) within a prospective registry at a single referral center in Giessen, Germany.RESULTS: In total, 2,067 patients were enrolled (PAH, 685 patients [33.1%]; pulmonary venous hypertension, 307 patients [14.9%]; PH due to lung diseases (LD-PH), 546 patients [26.4%; mainly interstitial lung disease and chronic obstructive pulmonary disease]; chronic thromboembolic PH, 459 patients [22.2%];' PH owing to miscellaneous/unknown causes, 70 patients [3.4%]). Median follow-up was 37 months. Differences in transplant-free survival between etiologic groups were highly significant (p < 0.001), with 1-, 3- and 5-year survival rates of 88.2%, 72.2% and 59.4%, respectively, for those with PAH compared with 79.5%, 52.7% and 38.1%, respectively, for patients with LD-PH. Patients' age, gender and 6-minute walk distance (6MWD), but not New York Heart Association (NYHA) functional class, associated significantly with survival across all PH subtypes in multivariate Cox regression analyses.CONCLUSIONS: This is the largest single-center PH cohort described so far. Some parameters used in clinical practice do not independently predict survival. Age, gender and 6MWD outperformed NYHA functional class in predicting survival across all etiologic groups. (C) 2017 The Authors. Published by Elsevier Inc.