Pediatric pulmonary arterial hypertension: global epidemiology and disease burden during the period 1990 to 2021.

Objectives: Pediatric pulmonary arterial hypertension (PAH) is a severe and potentially fatal disease; however, the global epidemiological burden of pediatric PAH has not been comprehensively elucidated. Study design: The Global Burden of Diseases Study 2021 has incorporated PAH for the first time. This study analyzed the incidence, prevalence, mortality, and disability-adjusted life years (DALYs) associated with pediatric PAH from 1990 to 2021. Results: Globally, the age-standardized prevalence rate (ASPR) of pediatric PAH in children aged 0-14 years showed a relatively stable, from 0.44 per 100,000 in 1990 to 0.43 per 100,000 in 2021. Females exhibited higher age-standardized incidence rates (ASIR) and ASPR compared to males. The ASIR of pediatric PAH increased modestly, with an average annual growth of 0.12%. Children aged 10-14 years had the highest prevalence, with rates of 0.66 per 100,000 in 1990 and 0.65 per 100,000 in 2021. In contrast, newborns aged 0-6 days experienced the highest mortality rates (34.16 and 13.67 per 100,000) and DALYs rates (3,073.56 and 1,229.88 per 100,000) during the same period. Countries with high-middle SDI levels had the highest ASPR, while high-SDI countries reported the lowest age-standardized mortality rate and age-standardized disability rate. Conclusions: Globally, prevalence, mortality, and DALYs of pediatric PAH show a declining trend from 1990 to 2021. Females exhibited higher incidence and prevalence rate but lower mortality and DALYs. 10-14 years had the highest prevalence, whereas high PAH-related mortality in neonates remains a critical concern. Tailored health policies for PAH management are needed.