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Increased augmentation index in patients with Ehlers-Danlos syndrome
Maurice Roeder1, Sira Thiel1, Frederic Baumann2
1Department of Pulmonology, University Hospital Zurich, Rämistrasse 100, 8091, Zurich, Switzerland.
Insights
Patients with Ehlers-Danlos Syndrome (EDS) have increased arterial stiffness, indicated by a higher augmentation index (AIx). This finding suggests AIx may be a useful marker for vascular complications in EDS.
Area of Science:
- Cardiology
- Vascular Biology
- Genetics
Background:
- Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders.
- EDS is characterized by joint hypermobility, tissue fragility, and vascular issues.
- Predictive markers for vascular disease progression in EDS are lacking.
Purpose of the Study:
- To investigate aortic augmentation index (AIx) in Ehlers-Danlos Syndrome (EDS) patients.
- To determine if AIx, a marker of arterial stiffness, is elevated in EDS.
- To compare AIx in EDS patients versus healthy controls.
Main Methods:
- Noninvasive applanation tonometry was used to measure radial artery pulse waveforms.
- 61 adults with EDS and 61 matched healthy controls participated.
- Augmentation index (AIx) was calculated and adjusted for heart rate.
Main Results:
- EDS patients exhibited significantly higher aortic AIx than healthy controls (22.8% vs 14.8%).
- A positive association was found between EDS and AIx.
- This association remained significant after adjusting for confounding factors like age, sex, and blood pressure.
Conclusions:
- Patients with Ehlers-Danlos Syndrome demonstrate increased arterial stiffness.
- Elevated AIx in EDS warrants further investigation for its prognostic value in cardiovascular outcomes.
- Augmentation index may serve as a potential biomarker for vascular risk in EDS.
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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