Global disease burden of pulmonary arterial hypertension: patterns and inequality in 1990-2021, with projections to 2035

Abstract

Aims: This study investigates the global burden of pulmonary arterial hypertension (PAH) from 1990 to 2021, examines its association with sociodemographic factors, and predict the trends from 2020 to 2035.

Methods: Utilizing the Global Burden of Disease database, we collected global PAH burden indicators from 1990 to 2021, stratified by age, sex, and Socio-Demographic Index (SDI). Global burden of PAH were assessed using comparative analyses, decomposition analyses, cluster analyses, and health inequality analysis. Bayesian age-period cohort model was used to predict PAH burden from 2020 to 2035.

Results: From 1990 to 2021, global PAH age-standardized prevalence rate (ASPR) and age-standardized incidence rate (ASIR) increased by 22.8% and 4.0%, respectively, while the age-standardized mortality rate (ASMR) and disability-adjusted life years rate (ASDR) decreased by 22.9% and 37.6%. The global PAH burden is strongly linked to sociodemographic factors, with notable gender and age disparities, especially among women and the elderly. Decomposition analysis showed population growth drove the global increase in PAH burden, while aging was the main factor in high and upper-middle SDI regions. Frontier analysis and health inequality analysis revealed health improvements but widening disparities. Clustering analysis identified three distinct trajectory groups. By 2035, global ASPR, ASMR, and ASDR are expected to decline, with the largest reduction in ASDR (approximately 31.8%), while ASIR is projected to increase slightly.

Conclusions: Despite overall improvements in PAH mortality and disability rates, substantial inequalities persist across regions and demographic groups. Addressing aging challenges and ensuring equitable healthcare distribution will help reduce the burden.

Keywords: Bayesian age-period-cohort model; Forecasting; Global burden of disease study; Pulmonary arterial hypertension; Socio-demographic index.

Fig. 1

Crude prevalence, incidence, mortality, and DALYs rate of PAH across 204 countries and territories in 2021. Crude prevalence (A), incidence (B), mortality (C) and DALYs (D) rate across all age groups. Abbreviations: DALYs, disability-adjusted life years; PAH, Pulmonary arterial hypertension

Fig. 2

Association between global PAH burden and SDI from 1990 to 2021. Association between global PAH ASPR (A), ASIR (B), ASMR (C), ASDR (D) and SDI. The black line represents the 2021 expected age-standardized rate based on SDI. For each region, the points from left to right indicate annual estimates from 1990 to 2021. Abbreviations: ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized DALY rate; DALYs, disability-adjusted life years; PAH, Pulmonary arterial hypertension; SDI, socio-demographic index

Fig. 3

Global Burden of PAH in different age and sex groups from 1990 to 2021. The number of PAH prevalence (A), incidence (B), mortality (C) and DALYs (D) in different age and sex groups in 2021; The number of PAH prevalence and ASPR (E), incidence and ASIR (F), mortality and ASMR (G), and DALYs and ASDR (H) in different sex groups from 1990 to 2021. Abbreviations: ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized DALY rate; DALYs, disability-adjusted life years; PAH, Pulmonary arterial hypertension

Fig. 4

Decomposition analysis of changes in PAH incidence, prevalence, mortality, and DALYs by SDI from 1990 to 2021. Decomposition analysis of changes in PAH prevalence (A), incidence (B), mortality (C) and DALYs (D). Black dots indicate the overall change attributed to population, aging, and epidemiological change. For each component, positive values represent an increase in PAH attributable to that factor, while negative values indicate a corresponding decrease. Abbreviations: DALY, disability-adjusted life years; PAH, pulmonary arterial hypertension; SDI, socio-demographic index

Fig. 5

Frontier analysis of PAH based on ASDR and SDI across 204 countries and territories. A Frontier analysis of ASDR and SDI trends from 1990 to 2021. The frontier is represented by a solid black line, with each country or territory denoted by a point. Color gradients indicate progression over time, from light blue (1990) to dark blue (2021). B Frontier analysis based on 2021 SDI and PAH-related DALY rates. The 15 countries with the largest effective differences are marked in black. The five countries with the lowest SDI (< 0.38) and smallest effective differences are highlighted in blue, while the five high-SDI countries (> 0.85) with the largest effective differences are marked in red. Abbreviations: ASDR, age-standardized DALYs rate; DALYs, disability-adjusted life years; SDI, socio-demographic index; PAH, pulmonary arterial hypertension

Fig. 6

Cluster analysis based on the EAPC in incidence and DALYs for PAH from 1990 to 2021. Color key: Different colors represent trends in EAPC values across regions: green indicates stable or slight decrease; yellow, significant decrease; blue, significant increase. Abbreviations: EAPC, estimated annual percentage change; DALYs, disability-adjusted life years; PAH, pulmonary arterial hypertension

Fig. 7

Global health inequality analysis in PAH for 1990 and 2021. A Health inequality regression curves in global PAH DALYs and SDI for 1990 and 2021. B Health inequality concentration curves in global PAH DALYs and SDI for 1990 and 2021. Abbreviations: DALYs, disability-adjusted life years; SDI, socio-demographic index; PAH, pulmonary arterial hypertension

Fig. 8

Trends and Forecasts of Global PAH-Related Burden from 2021 to 2035. ASPR (A), ASIR (B), ASMR (C), and ASDR (D) from 1990 to 2035. Shaded regions represent a ± 1% range based on 2021 values. Abbreviations: ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized DALY rate; DALYs, disability-adjusted life years; PAH, Pulmonary arterial hypertension