Background

The incidence of Pulmonary Hypertension (PH) and Pulmonary Arterial Hypertension (PAH) is believed to be on the rise and is associated with poor outcomes.

Methods

We extracted age-standardized mortality rates (ASMRs) for decedents  18 years of age from the World Health Organization Mortality Database, using International Classification of Diseases 10th edition codes for PH and PAH, covering the period from 2001 to 2019. The UK and European Union countries with at least 1,000,000 inhabitants and at least 75% of available data points over the study period were included.

Results

Between 2001 and 2019, in countries with available data, the median ASMR for PH increased by +1.19 per 1,000,000 (+22.51%) in females and +0.36 per 1,000,000 (+6.06%) in males. Out of 19 countries, 13 demonstrate an increase in female PH ASMR, and 12 reported an increase in male PH ASMR. In contrast, median PAH ASMR decreased by -0.29 per 1,000,000 (-28.74%) in females and remained relatively unchanged in males, with a minor increase of +0.01 per 1,000,000 (+1.07%). Notably, there was significant inter-country heterogeneity, with countries like Hungary, Romania, and Poland displaying results incongruous with the rest of Europe.

Conclusions

While publicly available mortality statistics for PH may be unreliable, these data suggest an overall increase in mortality across Europe from 2001 to 2019. However, mortality from PAH has shown a decrease in females and a modest increase in males. This underscores the urgent need for robust and high-quality mortality reporting, including international registries, for both PH and PAH.