Key result
Patients with Ehlers-Danlos syndrome had a significantly increased aortic augmentation index compared to matched healthy controls (22.8% vs 14.8%, p<0.001), indicating increased arterial stiffness.
Why the study?
Reliable prognostic factors predicting vascular disease progression in Ehlers-Danlos syndrome are lacking, while augmentation index was shown to predict aortic disease progression in Marfan syndrome.
Does Ehlers-Danlos Syndrome increase aortic augmentation index compared to healthy controls?
Cross-Sectional (n=122)
Yes
Does Ehlers-Danlos Syndrome increase aortic augmentation index compared to healthy controls?
Patients with Ehlers-Danlos Syndrome have significantly increased arterial stiffness as measured by augmentation index, which may serve as a potential prognostic marker for vascular complications.
Suggests heightened vascular risk in Ehlers-Danlos syndrome; leaves open whether stiffness predicts outcomes or warrants intervention.
BACKGROUND: Ehlers-Danlos Syndrome (EDS) comprises a heterogeneous group of diseases characterized by joint hypermobility, connective tissue friability, and vascular fragility. Reliable prognostic factors predicting vascular disease progression (e.g. arterial aneurysms, dissections, and ruptures) in EDS patients are still missing. Recently, applanation tonometry derived augmentation index (AIx), an indirect marker of arterial stiffness, has shown to be positively associated with progression of aortic disease in Marfan syndrome. In this study, we assessed aortic AIx in patients with EDS and matched healthy controls. METHODS: We performed noninvasive applanation tonometry in 61 adults with EDS (43 women and 18 men aged 39.3 ± 14.6 years) and 61 age-, gender-, height-, and weight-matched healthy controls. Radial artery pulse waveforms were recorded and analyzed using the SphygmoCor System (AtCor Medical, Sydney, NSW, Australia). Calculated AIx was adjusted to a heart rate of 75/min. Groups were compared and association between AIx and EDS was determined by univariate and multivariate regression analysis. RESULTS: EDS patients were categorized in classical type EDS (34%), hypermobile type EDS (43%), vascular type EDS (5%), or remained unassignable (18%) due to overlapping features. EDS patients showed a significantly increased aortic AIx compared to healthy controls (22.8% ± 10.1 vs 14.8% ± 14.0, p < 0.001). EDS showed a positive association with AIx; independent of age, sex, height, blood pressure, medication, and pack years of smoking. CONCLUSIONS: Patients with EDS showed elevated AIx, indicating increased arterial stiffness when compared to healthy controls. Further investigations are needed in order to assess the prognostic value of increased AIx for cardiovascular outcomes in patients with EDS.
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Roeder et al. (2020) conducted a cross-sectional in Ehlers-Danlos syndrome (n=122). Ehlers-Danlos syndrome vs. Healthy controls was evaluated on Aortic augmentation index (AIx) adjusted to a heart rate of 75/min (p=<0.001). Patients with Ehlers-Danlos syndrome had a significantly increased aortic augmentation index compared to matched healthy controls (22.8% vs 14.8%, p<0.001), indicating increased arterial stiffness.
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