PURPOSE: To determine the prevalence of proximal aortic abnormalities in patients with Ehlers-Danlos syndrome (EDS). METHODS: In a prospective cohort study, aortic measurements by two-dimensional echocardiography were performed on consecutive EDS patients. RESULTS: Twenty-eight percent (20 of 71) had aortic root dilation (ARD) (> +2 SD above population based norms). Fourteen of 42 individuals with the classical form of EDS (types I/II) and 6 of 29 individuals with the hypermobile form (type III) had ARD, with no gender differences. CONCLUSION: ARD is a common finding in EDS. Longitudinal studies are indicated to determine progression of ARD and its clinical significance.
PURPOSE: To determine the prevalence of proximal aortic abnormalities in patients with Ehlers-Danlos syndrome (EDS). METHODS: In a prospective cohort study, aortic measurements by two-dimensional echocardiography were performed on consecutive EDS patients. RESULTS: Twenty-eight percent (20 of 71) had aortic root dilation (ARD) (> +2 SD above population based norms). Fourteen of 42 individuals with the classical form of EDS (types I/II) and 6 of 29 individuals with the hypermobile form (type III) had ARD, with no gender differences. CONCLUSION: ARD is a common finding in EDS. Longitudinal studies are indicated to determine progression of ARD and its clinical significance.
Authors: Janine Meienberg; Marianne Rohrbach; Stefan Neuenschwander; Katharina Spanaus; Cecilia Giunta; Sira Alonso; Eliane Arnold; Caroline Henggeler; Stephan Regenass; Andrea Patrignani; Silvia Azzarello-Burri; Bernhard Steiner; Anders O H Nygren; Thierry Carrel; Beat Steinmann; Gábor Mátyás Journal: Eur J Hum Genet Date: 2010-07-21 Impact factor: 4.246
Authors: Arick C Park; Noel Phan; Dawiyat Massoudi; Zhenjie Liu; John F Kernien; Sheila M Adams; Jeffrey M Davidson; David E Birk; Bo Liu; Daniel S Greenspan Journal: Am J Pathol Date: 2017-07-19 Impact factor: 4.307
Authors: Adam J Brownstein; Bulat A Ziganshin; Helena Kuivaniemi; Simon C Body; Allen E Bale; John A Elefteriades Journal: Aorta (Stamford) Date: 2017-02-01