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Association Between Cardiac Size, Systolic Function, and Complications in Vascular Ehlers-Danlos Syndrome.
Association Between Cardiac Size, Systolic Function, and Complications in Vascular Ehlers-Danlos Syndrome.
- Source :
-
Canadian Association of Radiologists Journal . Feb2025, Vol. 76 Issue 1, p161-170. 10p. - Publication Year :
- 2025
-
Abstract
- Purpose: Vascular Ehlers-Danlos syndrome (vEDS) is a rare and aggressive heritable aortic disease caused by pathogenic variants in COL3A1 gene, characterized by spontaneous arterial dissection and organ rupture. The purpose of this study is to evaluate ventricular size and function and to explore their associations with complications in vEDS. Methods: Adults with genetically confirmed vEDS who underwent clinical cardiac MRI were retrospectively compared with controls matched for age and sex. Cardiac MRI analysis included assessment of ventricular volumetry and arterial vasculature. vEDS-related complications were evaluated including dissection, aneurysm, and pneumothorax. Multivariable logistic regression was performed. Results: We studied 26 individuals with vEDS (38.6 ± 15.6 years, 50.0% female) and 26 healthy controls. Median clinical follow-up was 2.4 (1.1-3.6) years. Left and right ventricular ejection fractions were lower in vEDS compared with controls (LVEF 58 ± 6% vs 61 ± 4%, P =.03; RVEF 54 ± 5% vs 58 ± 4%, P =.03). After controlling for age, sex, and antihypertensive medication, LV end-diastolic volume indexed to body surface area (LVEDVi) predicted dissections (OR 1.1, 95% CI 1.01-1.2, P =.04) and aneurysms (OR 1.1, 95% CI 1.01-1.3, P =.03). Indexed LV end systolic volume (LVESVi) also predicted aneurysms (OR 1.2, 95% CI 1.03-1.5, P =.02). LVEF predicted the presence of any complication (OR 0.71, 95% CI 0.52-0.99, P =.04). Pneumothorax occurred exclusively in vEDS group among those with LVEF <58% (below the mean), 50.0% versus 0.0%, P =.02. Those with LVEF <58% had more frequent dissection and/or aneurysm (75.0% vs 12.5%, P =.04). Conclusion: Lower LVEF and larger cardiac size are associated with complications in vEDS. [ABSTRACT FROM AUTHOR]
- Subjects :
- *RISK assessment
*AORTIC aneurysms
*EHLERS-Danlos syndrome
*VENTRICULAR ejection fraction
*RESEARCH funding
*ARTERIAL dissections
*MULTIPLE regression analysis
*RETROSPECTIVE studies
*MAGNETIC resonance imaging
*PNEUMOTHORAX
*DESCRIPTIVE statistics
*ODDS ratio
*CARDIAC contraction
*CASE-control method
*CONFIDENCE intervals
*THORACIC aorta
*HEART ventricles
*DIASTOLE (Cardiac cycle)
*DISEASE risk factors
*DISEASE complications
Subjects
Details
- Language :
- English
- ISSN :
- 08465371
- Volume :
- 76
- Issue :
- 1
- Database :
- Academic Search Index
- Journal :
- Canadian Association of Radiologists Journal
- Publication Type :
- Academic Journal
- Accession number :
- 182074894
- Full Text :
- https://doi.org/10.1177/08465371241278523