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

Adult Humans Female Male Loeys-Dietz Syndrome / diagnostic imaging Prevalence Retrospective Studies Patient Acuity Heart Arrest Magnetic Resonance Imaging
Aortic aneurysm Aortic dissection Arrhythmia Loeys-Dietz syndrome Mitral annular disjunction Mitral valve prolapse

Journal

International journal of cardiology
ISSN: 1874-1754
Titre abrégé: Int J Cardiol
Pays: Netherlands
ID NLM: 8200291

Informations de publication

Date de publication:
01 12 2023
Historique:
received: 25 01 2023
revised: 04 08 2023
accepted: 17 08 2023
medline: 9 10 2023
pubmed: 21 8 2023
entrez: 20 8 2023
Statut: ppublish

Résumé

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. 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). 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. 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.

Sections du résumé

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.

Identifiants

DOI: 10.1016/j.ijcard.2023.131276 PMID: 37598908
pubmed: 37598908
pii: S0167-5273(23)01155-5
doi: 10.1016/j.ijcard.2023.131276
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

131276

Informations de copyright

Copyright © 2023 Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest None.

Auteurs

Felipe 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.

Victor Siang Hua Chan (VSH)

Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada.

Jillian Murphy (J)

Department of Molecular Genetics, Fred A. Litwin Family Centre for Genomic Medicine, University Health Network, University of Toronto, Ontario, Canada.

Dalia Abdulmonem L Hashem (DAL)

Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada.

Kate Hanneman (K)

Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, 1 PMB-298, Toronto, Ontario M5G 2N2, Canada.

Rachel M 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.

Paaladinesh 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.

Maral Ouzounian (M)

Division of Cardiac Surgery, Department of Surgery, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada.

Erwin Oechslin (E)

Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada.

Gauri R 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.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Mitral annular disjunction on cardiac MRI:...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Mitral annular disjunction on cardiac MRI:...
questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Maladies génétiques congénitales › Syndrome de Loeys-Dietz › Mitral annular disjunction on cardiac MRI:...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Collecte de données › Registre civil › Morbidité › Prévalence › Mitral annular disjunction on cardiac MRI:...
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questionsmedicales.fr › Maladies cardiovasculaires › Cardiopathies › Arrêt cardiaque › Mitral annular disjunction on cardiac MRI:...
questionsmedicales.fr › Diagnostic › Techniques et procédures diagnostiques › Imagerie diagnostique › Tomographie › Imagerie par résonance magnétique › Mitral annular disjunction on cardiac MRI:...