Abstract
OBJECTIVE: This study aimed to assess the global, regional and national burden of pulmonary arterial hypertension (PAH) from 1990 to 2021 using data from the Global Burden of Disease Study 2021. The focus was on evaluating trends in incidence, prevalence, mortality and disability-adjusted life-years (DALYs) associated with PAH and examining these trends by age, gender and sociodemographic index (SDI). DESIGN: This is a systematic analysis leveraging data from the Global Burden of Disease Study 2021. The analysis focused on both crude and age-standardised rates to track temporal trends in PAH burden, with data stratified by region and SDI. SETTING: The study used global, regiona, and national data from 204 countries and regions, spanning from 1990 to 2021. PARTICIPANTS: The participants in this study include individuals diagnosed with PAH, with data representing populations globally, categorised by age, gender and SDI. PRIMARY AND SECONDARY OUTCOME MEASURES: Primary outcome measures included global, regional and national incidence, prevalence, mortality and DALYs related to PAH. Secondary outcomes consisted of age-standardised rates (age-standardised incidence rate (ASIR), age-standardised mortality rate (ASMR)) and trends over the study period. A key strength of this study is the detailed stratification by SDI, revealing how PAH burden varies across different socio-economic settings. This extended temporal analysis offers new insights into long-term trends, highlighting the rising burden in lower-SDI regions and significant regional disparities in disease management and outcomes. RESULTS: From 1990 to 2021, global PAH cases showed substantial increases in both incidence (85.62%) and prevalence (81.46%), while age-standardised rates remained stable. Across SDI levels, high-SDI regions maintained stable ASIRs (0.37 per 100 000) with a slight decline (estimated average percentage change (EAPC) -0.06%), while low-SDI regions demonstrated the most significant reduction (EAPC -0.30%). Deaths increased by 48.36% globally, though the ASMR decreased from 0.35 to 0.27 per 100 000. The disease burden measured by DALYs decreased by 6.59%, with high-SDI regions showing better improvements in age-standardised DALY rates (-1.39% EAPC) compared with other SDI levels. Gender analysis revealed persistent female predominance (female-to-male ratio 1.62:1), particularly pronounced in populations over 50 years across all SDI quintiles. CONCLUSIONS: While global age-standardised rates have declined, PAH remains a significant global health burden, particularly in low-SDI regions. These findings underscore the need for targeted prevention and intervention strategies, especially for high-risk populations, such as females and the elderly, to reduce the global health impact of PAH.