BACKGROUND Prior research has suggested that the prevalence and outcomes of pulmonary arterial hypertension (PAH) may vary by race or ethnicity. However, these studies have been limited by small sample size or methodological techniques relying on epidemiologic data. The purpose of this study is to evaluate the relationship between race/ethnicity and survival in a large U.S.-based prospective multicenter registry. METHODS Patients in the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL), a 5-year observational study of Group 1 PAH, were categorized by race/ethnicity. Baseline hemodynamic characteristics, clinical characteristics, and medication use was described. The relationship between race/ethnicity and outcome was evaluated by Kaplan-Meier and Cox proportional hazards modeling techniques. Left-truncation analysis, which adjusted for time from diagnosis to study enrollment, was used to minimize the effect of survivor bias. RESULTS This analysis included 3,046 patients; 2,202 identified as white, 393 as black, 263 as Hispanic, 100 as Asian or Pacific Islander, and 88 as other. Unadjusted Kaplan-Meier survival analysis indicated that white patients had the lowest survival rates. After adjusting for variables of prognostic impact, race/ethnicity was no longer significantly associated with survival. Other results showed that black patients were more likely to have connective tissue disease-associated PAH, Hispanic patients were more likely to have portopulmonary hypertension, and Asian patients were more likely to have congenital heart disease-associated PAH. CONCLUSIONS Analysis of the REVEAL registry did not find race/ethnicity to be a significant predictor of mortality. This is the largest analysis to date evaluating the role of race/ethnicity on outcomes in PAH.

中文翻译：

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种族对肺动脉高压存活率的影响：来自REVEAL注册中心的结果。

背景技术先前的研究表明，肺动脉高压（PAH）的患病率和预后可能因种族或种族而异。但是，这些研究受到小样本量或依赖流行病学数据的方法学方法的限制。这项研究的目的是评估一个大型的美国前瞻性多中心注册表中种族/族裔与生存之间的关系。方法：对登记在册的评估早期和长期PAH疾病管理（REVEAL）的患者（第1组PAH的5年观察研究）按种族/民族进行分类。描述了基线血流动力学特征，临床特征和药物使用。种族/民族与结果之间的关系通过Kaplan-Meier和Cox比例风险建模技术进行了评估。左截断分析 从诊断到研究入组的时间进行了调整，以最小化幸存者偏见的影响。结果该分析包括3,046例患者；其中，有2202人被定为白人，393人为黑人，263人为西班牙裔，100人为亚裔或太平洋岛民，88人为其他人。未经调整的Kaplan-Meier生存分析表明，白人患者的生存率最低。在调整了预后影响变量后，种族/民族不再与生存显着相关。其他结果表明，黑人患者更可能患有结缔组织疾病相关的PAH，西班牙裔患者更可能患有肺动脉高压，而亚洲患者更可能患有先天性心脏病相关的PAH。结论对REVEAL注册中心的分析未发现种族/民族是死亡率的重要预测指标。这是迄今为止评估种族/民族对PAH结局的作用的最大分析。