Chest. 2015 Oct;148(4):1043-54. doi: 10.1378/chest.15-0300.
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.
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.
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.
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.
ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.
肺动脉高压（PAH）是一种罕见的严重疾病，其特征为右心衰竭恶化、功能状态下降和生存预后不良。本研究通过基线功能分级（FC）对新诊断和既往诊断的 PAH 患者进行分层，以评估美国 PAH 患者的 5 年生存率。登记评估早期和长期 PAH 疾病管理（REVEAL 登记）是一个由 55 个中心组成的美国观察性登记，纳入了符合世界卫生组织（WHO）第 1 组 PAH 标准的患者的人口统计学、疾病过程和管理情况。
REVEAL 登记从 2006 年 3 月至 2009 年 12 月连续纳入了年龄≥3 个月的新诊断和既往诊断的 WHO 第 1 组 PAH 患者。入组时收集了人口统计学、疾病特征和血流动力学数据。对年龄≥18 岁的患者进行了 FC 和其他亚组的生存分析。
1 年时，新诊断和既往诊断患者的生存差异（90.4%比 86.3%）保持到 5 年；既往诊断患者的 5 年生存率为 65.4%，而新诊断患者为 61.2%。FC I、II、III 和 IV 的既往诊断患者的 5 年生存率估计分别为 88.0%、75.6%、57.0%和 27.2%，而 FC I、II、III 和 IV 的新诊断患者的 5 年生存率分别为 72.2%、71.7%、60.0%和 43.8%。
尽管治疗进展，晚期 PAH 患者的 5 年生存率仍较差。纽约心脏协会 FC 仍然是未来生存的最重要预测因素之一。这些观察结果强调了在 PAH 患者中持续监测 FC 的重要性。
ClinicalTrials.gov；编号：NCT00370214；网址：www.clinicaltrials.gov。
