The Global Burden of Disease Study found that 192,000 cases of pulmonary hypertension—elevated pressure in the pulmonary circulation—were registered worldwide in 2021. As reported in The Lancet Respiratory Medicine, the highest age-standardized prevalence of pulmonary hypertension was among those aged 75–79.
According to the World Health Organization classification, pulmonary arterial hypertension (PAH) is a rare disease leading to progressive remodeling and narrowing of the pulmonary vasculature, right heart failure, and death. Despite the development of targeted therapies for PAH that help reduce morbidity and mortality, persistent disability and persistently high medical costs remain a burden for both patients and public health. However, comprehensive global estimates of the burden of PAH remain scarce and incomplete.
The Global Burden of Disease Study collaboration characterized the global epidemiology of PAH, including regional differences. The researchers focused specifically on pulmonary arterial hypertension (PAH) type 1 (according to the WHO classification), in which the mean resting pulmonary artery pressure is greater than 20 mmHg and the pulmonary capillary pressure is less than 15 mmHg. Using these criteria, the researchers included the largest epidemiological studies on PAH in their analysis.
In 2021, 192,000 cases of PAH were registered (95% confidence interval: 155,000 to 236,000). The age-standardized prevalence of PAH was 2.28 cases per 100,000 population. Notably, the prevalence of PAH appears to have remained relatively stable over time: in 1990, it was 2.30 cases per 100,000 population.
PAH was more common among women: 2.75 cases per 100,000 women versus 1.78 cases per 100,000 men in 2021. Slightly higher prevalence was observed in Western Europe, Central Latin America, and the Asia-Pacific region, while lower prevalence was observed in South Asia, North America, and Oceania. The age group most susceptible to developing PAH was 75–79 years.
In 2021, 22,000 people died from PAH worldwide, with an age-standardized mortality rate of 0.27 deaths per 100,000 population. Despite a higher prevalence of PAH in women, cause-specific mortality rates were similar for women and men. Disability and work disability rates associated with PAH were comparable to those for thyroid disease, chronic myelogenous leukemia, testicular cancer, and Crohn's disease.
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