Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry.
Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry.
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DOI：
10.1378/chest.13-3014
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发表时间：
2014-12
期刊：
影响因子：
9.6
通讯作者：
Zamanian RT
中科院分区：
文献类型：
--
作者：
Chung L;Farber HW;Benza R;Miller DP;Parsons L;Hassoun PM;McGoon M;Nicolls MR;Zamanian RT
与系统性硬化症（SSc-APAH）相关的肺动脉高压（PAH）患者的死亡率高于特发性疾病和其他结缔组织疾病（CTD-APAH）患者。我们试图在CTD-APAH人群中确定与SSc-APAH相关的死亡率的独特预测因素。评价早期和长期PAH管理的登记研究（REVEAL登记研究）是一项多中心、前瞻性、基于美国的既往和新诊断（诊断性右侧心脏导管插入术后90天内入组）PAH患者登记研究。考克斯回归模型评价了整个REVEAL登记研究人群中所有先前确定的死亡率候选预测因子，以确定SSc-APAH（n = 500）与非SSc-CTD-APAH（n = 304）人群中死亡率的显著预测因子。既往诊断和新诊断SSc-APAH组的3年生存率分别为61.4% ± 2.7%和51.2% ± 4.0%，而非SSc-CTD-APAH组分别为80.9% ± 2.7%和76.4% ± 4.6%（P <0.001）。在多变量分析中，年龄> 60岁、收缩压（SBP）≤ 110 mm Hg、6分钟步行距离（6 MWD）< 165 m、1年内平均右房压（mRAP）> 20 mm Hg和肺血管阻力（PVR）> 32 Wood单位仍然是SSc-APAH组死亡率的唯一预测因素; 6 MWD ≥ 440 m在non-SSc-CTD-APAH组中具有保护作用，而在SSc-APAH组中无保护作用。SSc-APAH患者的死亡率高于非SSc-CTD-APAH患者。识别死亡风险特别高的SSc-APAH患者，包括老年男性和基线SBP或6 MWD较低或mRAP或PVR显著升高的患者，将使医生能够识别可能受益于更密切监测和更积极治疗的患者。ClinicalTrials.gov;编号：NCT00370214; URL：www.clinicaltrials.gov
Patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-APAH) experience higher mortality rates than patients with idiopathic disease and those with other connective tissue diseases (CTD-APAH). We sought to identify unique predictors of mortality associated with SSc-APAH in the CTD-APAH population. The Registry to Evaluate Early and Long-Term PAH Management (REVEAL Registry) is a multicenter, prospective US-based registry of patients with previously and newly diagnosed (enrollment within 90 days of diagnostic right-sided heart catheterization) PAH. Cox regression models evaluated all previously identified candidate predictors of mortality in the overall REVEAL Registry population to identify significant predictors of mortality in the SSc-APAH (n = 500) vs non-SSc-CTD-APAH (n = 304) populations. Three-year survival rates in the previously diagnosed and newly diagnosed SSc-APAH group were 61.4% ± 2.7% and 51.2% ± 4.0%, respectively, compared with 80.9% ± 2.7% and 76.4% ± 4.6%, respectively, in the non-SSc-CTD-APAH group (P < .001). In multivariate analyses, men aged > 60 years, systolic BP (SBP) ≤ 110 mm Hg, 6-min walk distance (6MWD) < 165 m, mean right atrial pressure (mRAP) > 20 mm Hg within 1 year, and pulmonary vascular resistance (PVR) > 32 Wood units remained unique predictors of mortality in the SSc-APAH group; 6MWD ≥ 440 m was protective in the non-SSc-CTD-APAH group, but not the SSc-APAH group. Patients with SSc-APAH have higher mortality rates than patients with non-SSc-CTD-APAH. Identifying patients with SSc-APAH who are at a particularly high risk of death, including elderly men and patients with low baseline SBP or 6MWD, or markedly elevated mRAP or PVR, will enable physicians to identify patients who may benefit from closer monitoring and more aggressive treatment. ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov
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影响因子：
27.4
作者：
Launay D;Sitbon O;Hachulla E;Mouthon L;Gressin V;Rottat L;Clerson P;Cordier JF;Simonneau G;Humbert M
通讯作者：
Humbert M
影响因子：
24.3
作者：
Campo, A.;Mathai, S. C.;Hassoun, P. M.
通讯作者：
Hassoun, P. M.
影响因子：
4.7
作者：
Chung, Lorinda;Domsic, Robyn T.;Steen, Virginia D.
通讯作者：
Steen, Virginia D.
影响因子：
5.5
作者：
Hachulla, Eric;Launay, David;Humbert, Marc
通讯作者：
Humbert, Marc
影响因子：
--
作者：
Lefevre, Guillaume;Dauchet, Luc;Launay, David
通讯作者：
Launay, David