Background

A vascular condition known as pulmonary arterial hypertension (PAH) is characterized by high pressure and restricted flow via the pulmonary arteries, which can eventually result in right heart failure and death. To comprehend the epidemiology of this poorly studied and morbid condition, this study presents the global burden of PAH by utilizing all available data and the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) approach.

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Methods

The burden of PAH was included in GBD as a non-specific contributor to the burden of “other cardiovascular and circulatory disease” prior to the current endeavor. This study produced similar and consistent estimates of PAH burden by separating PAH as a separate cause of death and disability in GBD. Using typical GBD modeling techniques, we estimated the non-fatal and fatal burden of PAH in 204 nations and territories between 1990 and 2021 using epidemiological and vital registry data. We excluded pulmonary hypertension groups 2–5 and concentrated only on PAH (group 1 pulmonary hypertension).

Findings

In 2021, the expected number of common cases of PAH worldwide was 192 000 (95% UI = 155 000–236 000). Of them, 73 100 (58 900–89 600) were in men (38%) and 119 000 (95 900–146 000) were in females (62%). There were 2·28 instances per 100,000 people, according to the age-standardized prevalence (95% UI 1·85–2·80). As people aged, the prevalence rose, with those between the ages of 75 and 79 having the highest frequency.

Globally, PAH was responsible for 22,000 deaths in 2021 (18,200–25,400), with an age-standardized mortality rate of 0·27 deaths per 100,000 people (0·23–0·32). With a 38·2% increase in age-standardized years of life lost [YLLs] in 2021 compared to 1990, the burden of the disease seems to be decreasing with time. Estimates of YLLs linked to PAH were comparable to those for diseases including Crohn’s disease, multiple sclerosis, and chronic myeloid leukemia.

Interpretation

PAH is a rare but fatal disease that accounts for a considerable health-associated burden worldwide. PAH is disproportionally diagnosed among females and older adults.

Funding

Cardiovascular Medical Research and Education Fund and the Bill & Melinda Gates Foundation.