Global, regional, and national burden of pulmonary arterial hypertension, 1990–2021, with projections to 2045: a systematic analysis for the Global Burden of Disease Study 2021

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Abstract

Introduction Pulmonary arterial hypertension (PAH) remains a rare but highly disabling vascular disease associated with substantial morbidity, mortality, and health care costs. However, its global burden patterns and future trends have not been comprehensively assessed across countries, age groups, and socio-demographic development levels. Methods We used data from the Global Burden of Disease Study 2021 to estimate the incidence, mortality, and disability-adjusted life years (DALYs) of PAH across 204 countries and territories from 1990 to 2021. Age-standardised rates were calculated, and trends were evaluated using estimated annual percentage changes (EAPCs). Inequality was assessed using concentration indices, and drivers of burden changes were identified through decomposition analysis. Future trends were projected up to 2045 using Bayesian age–period–cohort models. Results In 2021, the global incidence of PAH was 43.25 per 1,000 population (95% UI: 34.70–52.44), mortality was 22.02 per 1,000 (95% UI: 18.24–25.35), and DALY rate were 8.24 per 100,000 (95% UI: 7.14–9.39). From 1990 to 2021, the age-standardised incidence rate (ASIR) slightly increased (EAPC: 0.68; 95% CI: 0.66–0.70), while the age-standardised mortality rate (ASMR) declined steadily. The burden was higher among older adults and females and became increasingly concentrated in high-SDI countries. Projections suggest that global ASIR will rise slightly and stabilize, while ASMR will continue to decline by 2045. Conclusion PAH continues to impose a substantial and evolving burden worldwide, with growing disparities across development levels. Targeted strategies for early detection, secondary prevention, and resource allocation are urgently needed, especially in high-burden and rapidly aging populations.

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