Global pulmonary arterial hypertension trends and projections to 2046: a multi-method analysis of epidemiologic and demographic drivers using GBD 2021

Abstract

Background: Pulmonary arterial hypertension (PAH) is a progressive disease that affects millions of individuals worldwide. This investigation predicted PAH burden pattern and temporal trends to provide epidemiologic evidence.

Methods: Data on prevalence, mortality and disability-adjusted life years (DALYs) was extracted from the Global Burden of Diseases (GBD) 2021. Subgroup analyses were performed based on sex, geographical regions, and socio-demographic index (SDI). Joinpoint model, Bayesian age-period-cohort (BAPC) model, age-period-cohort analysis, decomposition methodology and frontier analysis were employed to evaluate the temporal trends from 1992 to 2021, forecast the disease burden up to 2046 and decompose prevalence, mortality and DALYs by population age structure, population growth and epidemiologic changes.

Results: From 1992 to 2021, the worldwide prevalence of PAH remained steady, while mortality and DALYs associated with PAH declined. There were substantial disparities in the PAH burden and trends across sex, age and SDI regions. Population aging and growth were key factors driving the increase in PAH prevalence, while epidemiological changes affected DALYs differently across regions. Frontier analysis indicated that countries or regions with a higher SDI quintile tend to have greater improvement potential. Predictions suggested that from 2022 to 2046, global PAH prevalence, mortality, and DALYs were expected to decline, with persisting but decreasing gender differences.

Conclusions: The study provided a comprehensive global assessment of the PAH burden. Socioeconomic factors significantly influence PAH outcomes, highlighting the need for equitable access to healthcare access. Future projections indicate ongoing improvements in PAH management, underscoring the importance of continued research and policy development.

Keywords: Global Burden of Disease 2021 (GBD 2021); Pulmonary arterial hypertension (PAH); disability-adjusted life years (DALYs); mortality; prevalence.

Figure 1

Sex‑specific age‑standardised rates of PAH burden by SDI quintile, 1992–2021. ASDR, age-standardized death rate; ASDALYs, age-standardized rate of disability-adjusted life years; ASPR, age-standardized prevalence rate; ASR, age-standardized rate; PAH, pulmonary arterial hypertension; SDI, socio-demographic index.

Figure 2

Global PAH burden by sex, age group, and SDI region. (A-C) Age-standardised prevalence rates (ASPR, per 100,000) and accompanying absolute case counts for 1992, 2019, and 2021. (D-F) Age-standardised death rates (ASDR, per 100,000) and absolute death counts for 1992, 2019, and 2021. (G-I) Age-standardised DALY rates (ASDALYs, per 100,000) and absolute DALY counts for 1992, 2019, and 2021. ASDR, age-standardized death rate; ASDALYs, age-standardized rate of disability-adjusted life years; ASPR, age-standardized prevalence rate; PAH, pulmonary arterial hypertension; DALYs, disability-adjusted life years; SDI, socio-demographic index; y, years.

Figure 3

Annual change in PAH burden, 1992–2021. (A) The EAPCs for age-standardised rates (ASPR, ASDR, ASDALYs) by sex and SDI region. (B-D) Country‑level EAPC for age-standardised rates (ASPR, ASDR, ASDALYs), respectively. In the current legend, each interval is “closed” at its lower limit and “open” at its upper limit. ASDR, age-standardized death rate; ASDALYs, age-standardized rate of disability-adjusted life years; ASPR, age-standardized prevalence rate; EAPC, estimated annual percentage change; PAH, pulmonary arterial hypertension; SDI, socio-demographic index.

Figure 4

Relationship between SDI and PAH burden. (A) Age‑standardised rates (ASPR, ASDR, ASDALYs) across 21 GBD regions, 1992–2021. (B) Age‑standardised rates (ASPR, ASDR, ASDALYs) for 204 countries and territories in 2021. ASDR, age-standardized death rate; ASDALYs, age-standardized rate of disability-adjusted life years; ASPR, age-standardized prevalence rate; GBD, global burden of disease; PAH, pulmonary arterial hypertension; SDI, socio-demographic index.

Figure 5

Jionpoint regression analysis of global PAH burden, 1992–2021. (A-C) APC in age-standardised rates (ASPR, ASDR, ASDALYs) by sex. (D) Association between AAPC and SDI. *, P<0.05 for the correlation coefficient r. AAPC, average annual percent change; APC, average percent change; ASDR, age-standardized death rate; ASDALYs, age-standardized rate of disability-adjusted life years; ASPR, age-standardized prevalence rate; PAH, pulmonary arterial hypertension; SDI, socio-demographic index.

Figure 6

Longitudinal age curves of PAH absolute prevalence, mortality and DALYs (per 100,000 person-years) adjusted for period deviations. DALYs, disability-adjusted life years; PAH, pulmonary arterial hypertension; SDI, socio-demographic index.

Figure 7

Decomposition of changes in PAH burden, 1992–2021. (A) Change in absolute prevalence; (B) change in absolute mortality; (C) change in absolute DALYs, each partitioned into contributions from population growth, population ageing, and epidemiological change. DALYs, disability-adjusted life years; GBD, Global Burden of Disease; PAH, pulmonary arterial hypertension; SDI, socio-demographic index.

Figure 8

Frontier analysis based on SDI and age-standardised rates—ASPR (A,D), ASDR (B,E) and ASDALYs (C,F). The red dot indicates that the rate of 2021 is higher than that of 1992, the blue dot indicates that the rate of 2021 is lower than that of 1992, and the black dot indicates the 15 countries with the greatest distance between frontier and the true value. The red dot indicates the five countries with the largest distance between frontier and true value in the countries above the high SDI threshold, and the blue dot indicates the five countries with the smallest distance between frontier and true value in the countries below the low SDI threshold. ASDR, age-standardized death rate; ASDALYs, age-standardized rate of disability-adjusted life years; ASPR, age-standardized prevalence rate; PAH, pulmonary arterial hypertension; SDI, socio-demographic index.

Figure 9

Predicted global PAH burden, 2022–2046. (A) Age‑standardised rates of prevalence, mortality, and DALYs; (B) corresponding absolute counts of cases, deaths, and DALYs. DALYs, disability-adjusted life years; PAH, pulmonary arterial hypertension.