Background

Pulmonary arterial hypertension (PAH) is a vascular disease characterised by restricted flow and high pressure through the pulmonary arteries, leading to progressive right heart failure and death. This study reports the global burden of PAH, leveraging all available data and using methodology of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) to understand the epidemiology of this under-researched and morbid disease.

Methods

Prior to the current effort, the burden of PAH was included in GBD as a non-specific contributor to “other cardiovascular and circulatory disease” burden. In this study, PAH was distinguished as its own cause of death and disability in GBD, producing comparable and consistent estimates of PAH burden. We used epidemiological and vital registry data to estimate the non-fatal and fatal burden of PAH in 204 countries and territories from 1990 to 2021 using standard GBD modelling approaches. We specifically focused on PAH (group 1 pulmonary hypertension), and did not include pulmonary hypertension groups 2–5.

Findings

In 2021, there were an estimated 192 000 (95% uncertainty interval [UI] 155 000–236 000) prevalent cases of PAH globally. Of these, 119 000 (95 900–146 000) were in females (62%) and 73 100 (58 900–89 600) in males (38%). The age-standardised prevalence was 2·28 cases per 100 000 population (95% UI 1·85–2·80). Prevalence increased with age such that the highest prevalence was among individuals aged 75–79 years. In 2021, there were 22 000 deaths (18 200–25 400) attributed to PAH globally, with an age-standardised mortality rate of 0·27 deaths from PAH per 100 000 population (0·23–0·32). The burden of disease appears to be improving over time (38·2% improvement in age-standardised years of life lost [YLLs] in 2021 relative to 1990). YLLs attributed to PAH were similar to estimates for conditions such as chronic myeloid leukaemia, multiple sclerosis, and Crohn's disease.

Interpretation

PAH is a rare but fatal disease that accounts for a considerable health-associated burden worldwide. PAH is disproportionally diagnosed among females and older adults.

Funding

Cardiovascular Medical Research and Education Fund and the Bill & Melinda Gates Foundation.

中文翻译：

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1990-2021 年全球、区域和国家肺动脉高压负担：2021 年全球疾病负担研究的系统分析

 背景

肺动脉高压 （PAH） 是一种血管疾病，其特征是肺动脉血流受限和高压，导致进行性右心衰竭和死亡。本研究报告了 PAH 的全球负担，利用所有可用数据并使用全球疾病、伤害和风险因素负担研究 （GBD） 的方法来了解这种研究不足和病态疾病的流行病学。

 方法

在目前的努力之前，PAH 的负担被包含在 GBD 中，作为“其他心血管和循环疾病”负担的非特异性贡献者。在这项研究中，PAH 被区分为 GBD 中其自身的死亡和残疾原因，产生了 PAH 负荷的可比且一致的估计值。我们使用流行病学和生命体征登记数据，使用标准 GBD 建模方法估计 1990 年至 2021 年期间 204 个国家和地区的 PAH 非致命和致命负担。我们特别关注 PAH （1 组肺动脉高压），不包括肺动脉高压 2-5 组。

 发现

2021 年，全球估计有 192 000 例 （95% 不确定性区间 [UI] 155 000–236 000） 多环芳烃 （PAH） 患病病例。其中，女性 （62%） 为 119 000 （95 900–146 000），男性为 73 100 （58 900–89 600） （38%）。年龄标准化患病率为每 100 000 人 2·28 例 （95% UI 1·85–2·80）。患病率随着年龄的增长而增加，因此 75-79 岁的个体患病率最高。2021 年，全球有 22 000 人（18 200-25 400 人）死于多环芳烃，年龄标准化死亡率为每 100 000 人中有 0·27 人死于多环芳烃 （0·23-0·32）。随着时间的推移，疾病负担似乎正在改善（与 1990 年相比，2021 年年龄标准化寿命损失年 [YLL] 提高了 38·2%）。归因于 PAH 的 YLL 与慢性粒细胞白血病、多发性硬化症和克罗恩病等病症的估计值相似。

 解释

PAH 是一种罕见但致命的疾病，在世界范围内造成相当大的健康相关负担。PAH 在女性和老年人中被诊断出的比例过高。

 资金

心血管医学研究和教育基金以及比尔和梅琳达·盖茨基金会。