Five-Year Outcomes of Patients Enrolled in the REVEAL Registry
Five-Year Outcomes of Patients Enrolled in the REVEAL Registry
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DOI：
10.1378/chest.15-0300
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发表时间：
2015-10-01
期刊：
影响因子：
9.6
通讯作者：
Benza, Raymond L.
中科院分区：
文献类型：
--
作者：
Farber, Harrison W.;Miller, Dave P.;Benza, Raymond L.
背景技术背景：肺动脉高压（PAH）是一种罕见的严重疾病，其特征是右侧心力衰竭恶化、功能状态下降和生存率低。本研究描述了美国按基线功能分级（FC）分层的新诊断和既往诊断PAH患者的5年生存率。评价早期和长期PAH疾病管理的登记研究（REVEAL登记研究）是一项55个中心的观察性美国登记研究，涉及世界卫生组织（WHO）第1组PAH患者的人口统计学、病程和管理。方法：REVEAL登记研究从2006年3月至2009年12月连续招募了年龄≥ 3个月的WHO第1组PAH新诊断和既往诊断患者。在入组时收集人口统计学、疾病特征和血流动力学数据。结果：初诊和初诊患者1年生存率差异（90.4%vs86.3%）持续到5年，5年生存率初诊和初诊患者分别为61.2%和65.4%。先前诊断为FC I、II、III和IV的患者的估计5年生存率分别为88.0%、75.6%、57.0%和27.2%，而新诊断为FC I、II、III和IV的患者的估计5年生存率分别为72.2%、71.7%、60.0%和43.8%。尽管治疗取得了进展，但晚期PAH患者的5年生存率仍然很低。纽约心脏协会FC仍然是未来生存率最重要的预测因素之一。这些观察结果强化了PAH患者中持续监测FC的重要性。
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare, severe disease characterized by worsening right-sided heart failure, decreasing functional status, and poor survival. The present study characterizes the 5-year survival in the United States of a new and previous diagnosis of PAH in patients stratified by baseline functional class (FC). The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) is a 55-center observational US registry of the demographics, disease course, and management of patients with World Health Organization (WHO) group 1 PAH.METHODS: The REVEAL Registry enrolled newly and previously diagnosed patients aged >= 3 months with WHO group 1 PAH consecutively from March 2006 to December 2009. Demographics, disease characteristics, and hemodynamic data were collected at enrollment. Survival analysis was conducted by FC and other subgroups in patients aged >= 18 years.RESULTS: Survival differences between previously diagnosed and newly diagnosed patients at 1 year (90.4% vs 86.3%) were maintained to 5 years; 5-year survival for previously diagnosed patients was 65.4% compared with 61.2% for newly diagnosed patients. Previously diagnosed patients in FC I, II, III, and IV had an estimated 5-year survival rate of 88.0%, 75.6%, 57.0%, and 27.2%, respectively, compared with 72.2%, 71.7%, 60.0%, and 43.8% for newly diagnosed patients in FC I, II, III, and IV, respectively.CONCLUSIONS: Patient survival of advanced PAH remains poor at 5 years despite treatment advances. New York Heart Association FC remains one of the most important predictors of future survival. These observations reinforce the importance of continuous monitoring of FC in patients with PAH.