Primary Pulmonary Hypertension Mortality Trends and Demographic Disparities in the United States, 2018–2024

Background/Objectives: Prior U.S. surveillance studies described changes in pulmonary hypertension mortality through 2020, but contemporary national data focused specifically on deaths coded as primary pulmonary hypertension (PPH; ICD-10 I27.0) are limited. We examined temporal, sex, racial, and geographic patterns in I27.0-coded mortality in the United States from 2018 to 2024. Methods: CDC WONDER Underlying Cause of Death data were analyzed. Annual deaths and mortality rates were examined by year, sex, Single Race 6 category, and U.S. Census region. Results: A total of 1831 deaths were coded as I27.0. Annual deaths declined from 344 in 2018 to 199 in 2024, while the exact crude rate declined from 0.105 to 0.059 per 100,000. Women accounted for 69.5% of deaths. Black individuals had a higher age-adjusted mortality rate than White individuals (0.10 vs. 0.06 per 100,000). The South accounted for the largest share of deaths (43.1%), although the West had a marginally higher exact crude rate. Conclusions: Deaths assigned to I27.0 declined substantially during 2018–2024. This trend occurred during a period of major evolution in PAH care, but the ecological design cannot determine whether therapeutic advances caused the decline. Changes in coding, classification, and assignment of underlying cause may also have contributed. Persistent demographic disparities warrant further investigation.