Aortic flow dynamics and stiffness in Loeys–Dietz syndrome patients: a comparison with healthy volunteers and Marfan syndrome patients

• Published in: European heart journal cardiovascular imaging 2022-04, Vol.23 (5), p.641-649
• Main Authors: Ruiz-Muñoz, Aroa, Guala, Andrea, Rodriguez-Palomares, Jose, Dux-Santoy, Lydia, Servato, Luz, Lopez-Sainz, Angela, La Mura, Lucia, Granato, Chiara, Limeres, Javier, Gonzalez-Alujas, Teresa, Galián-Gay, Laura, Gutiérrez, Laura, Johnson, Kevin, Wieben, Oliver, Sao-Aviles, Augusto, Ferreira-Gonzalez, Ignacio, Evangelista, Arturo, Teixido-Tura, Gisela
• Format: Article
• Language: English
• Subjects: Aorta - diagnostic imaging; Aorta - pathology; Healthy Volunteers; Humans; Loeys-Dietz Syndrome - diagnostic imaging; Loeys-Dietz Syndrome - pathology; Marfan Syndrome - diagnostic imaging; Pulse Wave Analysis; Vascular Stiffness
• ISSN: 2047-2404; 2047-2412
• DOI: 10.1093/ehjci/jeab069

Abstract Aims To assess aortic flow and stiffness in patients with Loeys–Dietz syndrome (LDS) by 4D flow and cine cardiovascular magnetic resonance (CMR) and compare the results with those of healthy volunteers (HV) and Marfan syndrome (MFS) patients. Methods and results Twenty-one LDS and 44 MFS patients with no previous aortic dissection or surgery and 35 HV underwent non-contrast-enhanced 4D flow CMR. In-plane rotational flow (IRF), systolic flow reversal ratio (SFRR), and aortic diameters were obtained at 20 planes from the ascending (AAo) to the proximal descending aorta (DAo). IRF and SFRR were also quantified for aortic regions (proximal and distal AAo, arch and proximal DAo). Peak-systolic wall shear stress (WSS) maps were also estimated. Aortic stiffness was quantified using pulse wave velocity (PWV) and proximal AAo longitudinal strain. Compared to HV, LDS patients had lower rotational flow at the distal AAo (P = 0.002), arch (P = 0.002), and proximal DAo (P