Aortic flow dynamics and stiffness in Loeys–Dietz syndrome patients: a comparison with healthy volunteers and Marfan syndrome patients
European Society of Cardiology
European Heart Journal - Cardiovascular Imaging (2021) 00, 1–9doi:10.1093/ehjci/jeab069
Aroa Ruiz-Munoz ~ 1,2,3, Andrea Guala1,2,3*, Jose Rodriguez-Palomares1,2,3, Lydia Dux-Santoy1, Luz Servato3, Angela Lopez-Sainz3, Lucia La Mura1,4, Chiara Granato3, Javier Limeres3, Teresa Gonzalez-Alujas3, Laura Galia ́n-Gay3, Laura Gutie ́rrez3, Kevin Johnson5, Oliver Wieben5, Augusto Sao-Aviles1,3, Ignacio Ferreira-Gonzalez1,2,3,6,7, Arturo Evangelista1,2,3,7,8, and Gisela Teixido-Tura1,2,3
1 Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain; 2 CIBER-CV, Instituto de Salud Carlos III, Madrid, Spain; 3 Department of Cardiology, Hospital Universitari Vall d’Hebron, Paseo Vall d’Hebron 119-129, 08035 Barcelona, Spain; 4 Department of Advanced Biomedical Sciences, University Federico II. Naples, Naples, Italy; 5 Department of Medical Physics and Radiology, University of Wisconsin, Madison, WI, USA; 6 CIBER-ESP, Instituto de Salud Carlos III, Madrid, Spain; 7 Universitat Auto`noma de Barcelona, Bellaterra, Spain; and 8 Instituto del Corazo ́n. Quiro ́nsalud-Teknon. Barcelona, Spain
Received 28 September 2020; editorial decision 23 March 2021
Overview
This study examined aortic stiffness and aortic blood flow in the thoracic aorta of 21 adults with Loeys-Dietz syndrome (LDS) and compared their results to (1) adults with Marfan syndrome and (2) individuals with no known significant health issues.
The study team found that aortic stiffness was greater in LDS than in individuals without health issues but similar to those with Marfan syndrome. Aortic blood flow was similarly impaired in individuals with LDS and Marfan syndrome when compared to those without health issues.
Prior research related greater aortic stiffness to higher rates of surgical aortic replacement and aortic root dilation in children and young adults with connective tissue disorders.