Mitral annular disjunction on cardiac MRI: Prevalence and association with disease severity in Loeys-Dietz syndrome.

Autor: Sanchez Tijmes F; Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada., Chan VSH; Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada., Murphy J; Department of Molecular Genetics, Fred A. Litwin Family Centre for Genomic Medicine, University Health Network, University of Toronto, Ontario, Canada., Hashem DAL; Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada., Hanneman K; Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada., Wald RM; Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada; Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada., Thavendiranathan P; Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada; Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada., Ouzounian M; Division of Cardiac Surgery, Department of Surgery, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada., Oechslin E; Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada., Karur GR; Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada. Electronic address: gauri.ranikarur@uhn.ca.
Jazyk: angličtina
Zdroj: International journal of cardiology [Int J Cardiol] 2023 Dec 01; Vol. 392, pp. 131276. Date of Electronic Publication: 2023 Aug 19.
DOI: 10.1016/j.ijcard.2023.131276
Abstrakt: Background: The purpose of this study was to evaluate mitral annular disjunction (MAD) on cardiac magnetic resonance imaging (MRI) in Loeys-Dietz Syndrome (LDS) and to explore its association with adverse outcomes.
Methods: In this retrospective cohort study, adult patients with LDS who underwent cardiac MRI were evaluated for MAD, aortic dimensions, and ventricular volumetry. Aortic events were defined as aortic surgery and/or dissection and severe arrhythmic events as cardiac arrest or sustained ventricular tachycardia (VT).
Results: Among 46 LDS patients (52% female, 37.2 ± 14.3 years), 17 had MAD (37%). MAD and no MAD groups were similar in age, sex, aortic dimensions and left ventricular parameters. After a clinical follow-up of 4.3 years (IQR 1.5-8.4), 3 in MAD and 4 in no MAD groups required aortic valve sparing root replacement (VSRR) and 1 in MAD developed type A dissection. Over a similar imaging follow-up period [4.1 years (IQR 2.7-9.1) vs. 3.2 years (IQR 1.0-9.0), p = 0.65], compared to baseline, increase in native aortic root size was significant only in MAD (39.4 ± 4.6 mm vs. 38.1 ± 5.3 mm, p = 0.02, 19.3 ± 2.4 mm/m2 vs. 18.7 ± 2.4 mm/m2, p = 0.01) compared to those without MAD. Patients with MAD were younger at first aortic event compared to those without (26.7 ± 11.5 years vs. 45.0 ± 14.9 years, p = 0.03). MAD distance correlated with need for VSRR, r = 0.57, p = 0.02. Two patients in the MAD group developed sustained VT. No cardiac arrest or death was observed.
Conclusion: MAD is highly prevalent in LDS, associated with progressive aortic dilatation, and aortic events at younger age. MAD may be a marker of disease severity necessitating close surveillance.
Competing Interests: Declaration of Competing Interest None.
(Copyright © 2023 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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