Global, Regional, and National Burden of Pulmonary Arterial Hypertension from 1990 to 2021 and Projections to 2040: A Systematic Analysis of Incidence, Mortality, and Disability-Adjusted Life Years

Abstract

Objective

Pulmonary arterial hypertension (PAH) poses a growing global health challenge, yet comprehensive epidemiological data remain limited. This study aims to assess the burden of PAH from 1990 to 2021 and project trends to 2040, addressing critical gaps in incidence, mortality, and disability-adjusted life years (DALYs) across diverse socio-demographic contexts.

Methods

Using data from the Global Burden of Disease (GBD) 2021 study, we analyzed PAH burden across 204 countries and territories, stratified by age, sex, region, and socio-demographic index (SDI). Age-standardized rates (per 100,000 populations) for incidence (ASIR), mortality (ASMR), and DALYs (ASDR) were calculated. Future trends were projected via a Bayesian age-period-cohort (BAPC) model.

Results

In 2021, there were 43,251 (95% uncertainty interval [UI]: 34,705, 52,441) global incident PAH cases (age standardized incidence rate [ASIR]: 0.52). From 1990 to 2021, PAH incidence rose by 85.62%, with the steepest increase in high-middle SDI regions (average annual percentage change [AAPC]: + 0.19%). Despite a 48.36% rise in deaths, the age-standardized mortality rate (ASMR) declined annually by 0.84%, reflecting improved management. Central Europe had the highest ASMR (1.06 per 100,000), while low SDI regions showed reduced ASIR (−0.31% AAPC), likely due to underdiagnosis. PAH caused 642,104 DALYs globally in 2021, with infants (< 1 year) bearing the highest DALY rate. Projections indicate 75,000 annual cases by 2040, emphasizing an escalating burden.

Conclusion

PAH burden is increasing disproportionately in aging populations and high-middle SDI regions, while low SDI areas face underdiagnosis and healthcare disparities. Targeted interventions, equitable resource allocation, and enhanced diagnostic capacity are urgently needed to mitigate future PAH-related morbidity and mortality.

Keywords

Pulmonary arterial hypertension / Global burden of disease / Incidence / Mortality / Disability-adjusted life years (DALYs) / Epidemiology / Health policy / Socio-demographic index (SDI)

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Funding

Hubei Provincial Natural Science Foundation of China(2025AFB068)

Wuhan Natural Science Foundation Exploration Program(2024040801020344)

RIGHTS & PERMISSIONS

The Author(s), under exclusive licence to the Huazhong University of Science and Technology