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https://doi.org/10.1007/s11886-022-01806-1
CARDIAC PET, CT, ANDMRI (P CREMER, SECTION EDITOR)
Mitral Annular Disjunction: Associated Pathologies andClinical
Consequences
JonasVerbeke1 · AnthonyDemolder1 · JulieDeBacker1 · FrankTimmermans1
Accepted: 10 October 2022
© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022
Abstract
Purpose of Review To provide an overview of mitral annular disjunction (MAD) and to discuss important challenges in
diagnosis and management of MAD.
Recent Findings MAD has regained interest in the context of sudden cardiac death (SCD) in patients with mitral valve
prolapse (MVP), coined as the “arrhythmic” MVP syndrome. In addition, MAD in isolation was recently suggested to be
associated with severe arrhythmia and SCD.
Summary There is a lack of consensus on the definition of MAD and the imaging modality to be used for diagnosing MAD,
and the therapeutic implications of MAD remain uncertain. Furthermore, the exact mechanism underlying the association
of MAD with SCD remains largely unexplored.
Keywords Mitral annular disjunction· Mitral valve prolapse· Mitral regurgitation· Sudden cardiac death
Introduction
Mitral valve prolapse (MVP) affects 1–2% of the general pop-
ulation and, in the absence of significant mitral regurgitation
(MR), it is usually considered a benign degenerative disease
[1–9, 10••]. Over the years, an increased risk of sudden car-
diac death (SCD) has been reported in patients with MVP,
including patients with mildor no MR [11–19]. The risk
of SCD appears to be confined to a small subset of patients
with MVP featuring specific electrocardiographic and
echocardiographic signs, coined as the “arrhythmic MVP”
syndrome (aMVP) [19–23]. Patients with aMVP are typi-
cally young with bileaflet MVP and extensive leaflet redun-
dancy, and present with electrocardiographic abnormalities
(T wave inversion and ST-segment depression) and (marked)
mitral annular disjunction (MAD). Among these features,
MAD has received particular interest in recent years.
In MAD, there is a discontinuation between the attach-
ment of the mitral valve leaflet and the myocardium of the
left ventricular (LV) free wall (“ventricular crest”) (Fig.1)
[24, 25]. Although the original description is often credited
to Hutchins etal. [24], other authors already documented
this anatomic alteration several years before [26–29]. How-
ever, the association with MVP or SCD appeared spurious,
and soon after the landmark report, Angelini reiterated MAD
as a normal anatomical variant, reassuring the cardiac com-
munity [30, 31]. Although the number of reports on MAD
are rapidly expanding, many questions on this topic remain,
in particular, regarding the pathophysiology of MAD, its
assessment, its relation with MVP, and the potential thera-
peutic consequences. The purpose of this review is to pro-
vide an overview on the pathophysiology of MAD, and to
discuss the diagnostic and therapeutic challenges in patients
with MAD.
Challenges inDiagnosis ofMAD
The prevalence of MAD varies considerably among reports.
This probably relates to the lack of a universal definition of
MAD, the characteristics of the specific study population
Julie De Backer and Frank Timmermans contributed equally to the
manuscript
This article is part of the Topical Collection on Cardiac PET, CT,
and MRI
* Jonas Verbeke
jdverbek.verbeke@ugent.be
1 Department ofCardiology, Ghent University Hospital,
Corneel Heymanslaan 10, 9000Ghent, Belgium
Current Cardiology Reports (2022) 24:1933–1944
/ Published online:4 November 2022
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