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Biological Sex Differences in the US-CTEPH-R
Meghan M Cirulis1, William R Auger2, Raymond L Benza3
1Department of Pulmonary and Critical Care Medicine, Intermountain Health, Murray, UT.
Annals of the American Thoracic Society
|April 14, 2026
Summary
Males and females exhibit distinct chronic thromboembolic pulmonary hypertension (CTEPH) phenotypes. While overall survival is similar, females experience worse health-related quality of life and function post-pulmonary thromboendarterectomy.
Area of Science:
- Cardiopulmonary Medicine
- Pulmonary Hypertension Research
- Sex Differences in Disease
Background:
- Biological sex influences cardiopulmonary conditions, with known sex paradoxes in pulmonary arterial hypertension.
- Sex differences in chronic thromboembolic pulmonary hypertension (CTEPH) are less understood compared to pulmonary arterial hypertension (PAH).
Purpose of the Study:
- To investigate and characterize sex differences in patients diagnosed with CTEPH in the United States.
- To explore how biological sex impacts CTEPH presentation, treatment, and outcomes.
Main Methods:
- Utilized data from the multicenter United States CTEPH Registry, collecting data every six months for 48 months.
- Compared male and female characteristics using descriptive analyses and regression modeling, adjusting for age and BMI.
- Performed survival analysis using Cox proportional hazards modeling.
Main Results:
- Females had higher BMI, while males were more likely to have a history of deep vein thrombosis.
- Males demonstrated better baseline PH-specific health-related quality of life (HRQoL), WHO functional class, and six-minute walk distance.
- Males were less likely to have distally located disease or inoperable CTEPH; however, no significant difference in overall survival was observed between sexes.
Conclusions:
- Distinct CTEPH phenotypes exist between males and females, potentially influencing pulmonary thromboendarterectomy (PTE) access and feasibility.
- Sex-associated disparities exist in the disease experience and HRQoL for CTEPH patients.
- Further investigation is warranted to understand differential impacts on HRQoL and disease experience.
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