Increased augmentation index in patients with Ehlers-Danlos syndrome
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(2020) 20:417
RESEARCH ARTICLE
Open Access
Increased augmentation index in patients with Ehlers-Danlos syndrome Maurice Roeder1 , Sira Thiel1, Frederic Baumann2, Noriane A. Sievi1, Marianne Rohrbach3, Malcolm Kohler1,4*† and Thomas Gaisl1†
Abstract 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. Keywords: Ehlers-Danlos syndrome, Arterial stiffness, Augmentation index, Cardiovascular risk
Background Ehlers-Danlos Syndrome (EDS) represents a heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, connective tissue friability, as well as skin and vascular fragility. The combined prevalence of all * Correspondence: [email protected] † Malcolm Kohler and Thomas Gaisl contributed equally to this work. 1 Department of Pulmonology, University Hospital Zurich, Rämistrasse 100, 8091 Zurich, Switzerland 4 Centre for Interdisciplinary Sleep Research, University of Zurich, Zurich, Switzerland Full list of author information is available at the end of the article
EDS subtypes appears to be nearly 1 to 5000 individuals world- wide [1]. The current international classification of EDS distinguishes 13 clinical subtypes, for which molecular mutations have been identified in 19 different genes [2]. Vascular fragility with easy bruising
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