Background: The Global Burden of Disease (GBD) 2017 study provides estimates of cause-specific mortality for 282 causes from 1980 to 2017, but does not include pulmonary arterial hypertension (PAH). As part of the GBD 2020 study, we used all available cause-specific death data to estimate the global and regional patterns of PAH mortality.
Methods: The GBD mortality database includes vital registration (VR), verbal autopsy (VA), registry, and surveillance data. For each data source, underlying causes of death were mapped from International Classification of Disease (ICD) codes to GBD causes. ICD codes 416.0, I27.0, and I27.2 were mapped to pulmonary arterial hypertension. Deaths coded to nonspecific or inappropriate causes such as senility were redistributed across all causes, including a small fraction to PAH. We used the Cause of Death Ensemble model (CODEm) to estimate death rates across all location-years.
Results: Between 1990 and 2019, there were 4.54 (95% uncertainty interval 3.15 – 5.80) million deaths due to pulmonary arterial hypertension, of which 2.62 (1.58 – 3.63) million were female. The all-age death rate in 2019 was 2.52 (1.61 – 3.04) per million males and 3.81 (1.99 – 4.91) per million females, an increase of 74% and 150% respectively from 1990 rates. Across all years, the death rate among females was 34% higher than among males. The highest rates were observed in the United States. Healthcare access and quality (HAQ index), Sociodemographic Index (SDI), and HIV prevalence were predictive for estimating death rates due to PAH.
Conclusions: Deaths due to pulmonary arterial hypertension have increased since 1990, with the majority concentrated in women and high-income countries. Comparable estimates of pulmonary arterial hypertension mortality across time and geography provide insight into the epidemiology of this rare disease.