Pulmonary Arterial Hypertension Incidence Surges in Recent Decades

Medscape

2 days ago

The incidence of pulmonary arterial hypertension (PAH) has increased worldwide over the past three decades, although death rates and disability-adjusted life year (DALY) rates related to the disease have decreased, according to data from the Global Burden of Disease 2021 study.
The global disease burden of PAH continues to evolve not only because of medical advances but also because of the aging of the global population and the impact of socio-economic factors such as diet and exposure to environmental toxins, Zhenhao Liu, MD, of Pingxiang People's Hospital, Pingxiang, China, and colleagues wrote.
'Despite notable advancements in diagnostic and therapeutic modalities in recent years, there remains a critical need for a deeper comprehension of the global disease burden of PAH to better understand its specific impact on public health,' the researchers wrote.
In a report published in Frontiers in Public Health , the researchers analyzed data on PAH incidence, mortality, and DALYs at the national, regional, and global level from 1990 to 2021 using the Global Burden of Disease 2021 dataset.
Globally, PAH incident cases increased by 85.6% over the study period, and the age-standardized incidence rate (ASIR) increased only slightly, with a 0.05% estimated annual percentage change (EAPC).
The overall death rate from PAH decreased, with an EAPC of -0.57%, but PAH-related deaths increased from 14,842 in 1990 to 22,021 in 2021. The DALYs rate showed a downward trend, with an EAPC of -1.31%, and PAH accounted for 642,104 DALYs in 2021.
Notably, regions with the lowest sociodemographic index (SDI) quintiles had the highest ASIR, but death rates and DALYs decreased across all SDI groups.
Regionally, the highest PAH incidence occurred in Southern Sub-Saharan Africa, and the greatest increases in death and DALYs occurred in Central Asia. Nationally, Zambi, Ethiopia, and Uganda showed the highest age-standardized incidence rates for PAH in 2021 (1.06, 1.00, and 1.00 per 100,000 persons, respectively).
Latvia had the greatest rise in age-standardized mortality, while Puerto Rico had the greatest decreases in age-standardized mortality and DALYs (EAPC -6.64% and EAPC -6.72%, respectively).
Age and Gender Trends
The researchers also found a shift toward increased PAH in older adults in regions of higher SDI and a high prevalence in women. 'This increase is primarily attributed to the increased prevalence of noncommunicable diseases such as cardiovascular disease, cancer, and diabetes, which are more common in aging populations,' the authors wrote in their discussion.
The study was limited by several factors, including the variation in consistency and accessibility of data across nations, and the potential inaccuracies in the Global Burden of Disease dataset, the researchers noted.
However, the results emphasized the need for developing targeted preventive and therapeutic PAH management strategies tailored to diverse populations, especially in areas of high prevalence of disease, the researchers wrote.
Global Trends Drive Treatment
Evaluating global trends helps determine whether rising case numbers reflect improved diagnosis or a true increase in disease burden, said Ahmed Sadek, MD, assistant professor of medicine at the Lewis Katz School of Medicine at Temple University, Philadelphia, in an interview. Global trend studies also reveal region-specific factors, such as infectious causes including schistosomiasis in developing countries, said Sadek, who was not involved in the study. 'By analyzing these patterns, we can learn which public health interventions have worked and identify communities where we need to improve detection, address environmental or infectious exposures, or increase access to specialized diagnostics and treatments,' he said.
'It's encouraging, though not surprising, that overall mortality has decreased over the last 30 years, a trend that closely mirrors the introduction of multiple highly effective, PAH-specific therapies where none previously existed,' Sadek told Medscape Medical News . 'The slight rise in incidence in higher-income countries may seem surprising at first glance but likely reflects improved awareness and earlier detection rather than a true increase in disease burden,' he said. 'The rising mortality among women is more surprising, given that they have traditionally been viewed as having a more favorable prognosis,' he added. Although the reasons for some of the trends remain unclear, they may reflect increased attribution of PAH as the cause of death, especially as the population ages, and may be less able to tolerate disease at the time of diagnosis, he noted.
Policy Priorities
'At the policy level, the most cost-effective approach is to address the root causes of PAH, particularly in developing nations,' Sadek told Medscape Medical News . 'Public health initiatives focused on preventing and treating infections such as HIV, schistosomiasis, and hepatitis C can have a major downstream impact on reducing PAH incidence,' he noted. In addition, strengthening health infrastructure to support early diagnosis and management is critical to manage PAH, he said.
'At the clinical level, increasing physician education is essential to ensure timely recognition of PAH, especially in patients with known risk factors like connective tissue disease, so that we can initiate effective therapies earlier in the disease course, when outcomes are more favorable,' Sadek added.
As for additional research, greater understanding of the health disparities highlighted by the current trends is critical, said Sadek. 'We need to investigate the precise impact of factors like impaired access to expensive specialty medications and a lack of advanced diagnostic tools,' he said. 'For example, when a country has a high death rate but a low reported incidence rate, it suggests that late diagnosis is a key problem, and research should focus on identifying and overcoming these barriers to care,' he explained.
'Additionally, understanding region-specific risk factors can guide more effective, localized interventions — for instance, targeting schistosomiasis infection rates is a much more impactful strategy in Sub-Saharan Africa than in the United States, given the much higher incidence of infections in that region,' Sadek said.