Functional Class Improvement and 3-Year Survival Outcomes in Patients With Pulmonary Arterial Hypertension in the REVEAL Registry
Functional Class Improvement and 3-Year Survival Outcomes in Patients With Pulmonary Arterial Hypertension in the REVEAL Registry
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DOI：
10.1378/chest.12-2417
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发表时间：
2013-07-01
期刊：
影响因子：
9.6
通讯作者：
McGoon, Michael D.
中科院分区：
文献类型：
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作者：
Barst, Robyn J.;Chung, Lorinda;McGoon, Michael D.
目的：纽约心脏协会/世界卫生组织功能分级(FC)与肺动脉高压(PAH)的预后相关。我们评估了从FC III改善到FC I/III的PAH患者与仍在FC HI或恶化到FC IV的患者相比，是否提高了生存率。方法：从注册中心收集年龄=19岁的FC III PAH患者，以评估早期和长期PAH疾病管理(揭示注册)(N=982)，根据他们在登记后1年内从FC登记到首次随访评估的FC变化，将其分为改善、不变或恶化。Kaplan-Meier估计从首次随访到首次随访的3年存活率，以及从登记到首次随访的6分钟步行距离(6MWD)的变化。亚组分析按病因(如特发性/家族性、结缔组织病[CTD]、先天性心脏病)和诊断时间(即新诊断或先前诊断分别在入选后3个月内诊断为右心导管术)进行。结果：总体而言，27%的患者FC改善。FC改善(84%2%，n=263)的患者的生存率高于保持不变(66%+/-2%，n=645)或恶化(29%+/-6%，n=74)的患者(均为P<；.001)。FC改善的患者亚组的存活率也高于保持不变的患者亚组(特发性/家族性[P<；.001]、CTD相关的PAH[P=.009]，无论是新诊断的[P=.004]还是以前诊断的[P<；.0011])。6与总体(P<；.001)和CTD(P=.028)队列中保持不变的患者相比，FC改善的患者的MWD改善更大。结论：无论是新诊断的还是以前诊断的PAH患者，与保持FC III的患者相比，存活率都更高。试验注册：ClinicalTrials.gov；编号：NCT00370214；URL：www.Clinicaltrials.gov
Objective: New York Heart Association/World Health Organization functional class (FC) is associated with outcomes in pulmonary arterial hypertension (PAH). We assessed whether patients with PAH who improve from FC III to FC I/III have improved survival vs patients who remain at FC HI or worsen to FC IV.Methods: Patients aged >= 19 years with FC III PAH from the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL Registry) (N = 982) were categorized as improved, unchanged, or worsened according to their change in FC from enrollment to first follow-up assessment within 1 year of enrollment. Kaplan-Meier estimates of 3-year survival from first follow-up and changes in 6-min walk distance (6MWD) from enrollment to first follow-up were determined. Subgroup analyses were conducted by cause (ie, idiopathic/familial, connective tissue disease [CTD], congenital heart disease) and time of diagnosis (ie, newly or previously diagnosed [diagnostic right-sided heart catheterization within or >= 3 months of enrollment, respectively]).Results: Overall, 27% of patients improved FC. Survival was better in patients whose FC improved (84% 2%, n = 263) vs those who remained unchanged (66% +/- 2%, n = 645) or worsened (29% +/- 6%, n = 74) (all P < .001). Survival was also better in patient subgroups whose FC improved vs those who remained unchanged (idiopathic/familial [P < .001], CTD-associated PAH [P = .009], whether newly [P = .004] or previously diagnosed [P < .0011). 6MWD improvements were greater in patients whose FC improved vs those who remained unchanged in the overall (P < .001) and CTD (P = .028) cohorts.Conclusion: Patients with PAH who improve from FC III to I/III, whether newly or previously diagnosed and regardless of PAH cause, have better survival vs patients who remain FC III. Trial registry: ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov