Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature

Mitral annulus disjunction (MAD) is defined as a systolic displacement between the ventricular myocardium and the posterior mitral annulus supporting the posterior mitral leaflet. This structural abnormality is associated with the loss of mechanical annular function manifested as an abnormal systoli...

Full description

Bibliographic Details
Main Authors: Fay Apostolou, Marios Ioannides, Andreas Mitsis, Constantina Koutsofti, Constantinos Deltas, Panayiotis Avraamides
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-07-01
Series:Frontiers in Cardiovascular Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2023.1171226/full
_version_ 1797776110635712512
author Fay Apostolou
Marios Ioannides
Andreas Mitsis
Constantina Koutsofti
Constantinos Deltas
Constantinos Deltas
Panayiotis Avraamides
author_facet Fay Apostolou
Marios Ioannides
Andreas Mitsis
Constantina Koutsofti
Constantinos Deltas
Constantinos Deltas
Panayiotis Avraamides
author_sort Fay Apostolou
collection DOAJ
description Mitral annulus disjunction (MAD) is defined as a systolic displacement between the ventricular myocardium and the posterior mitral annulus supporting the posterior mitral leaflet. This structural abnormality is associated with the loss of mechanical annular function manifested as an abnormal systolic excursion of the leaflet hinge point into the left atrium but with maintained electrical function, separating the left atrium and ventricle electrophysiologically. The mitro-aortic fibrous continuity limits MAD anteriorly, between the aortic cusps and the anterior leaflet of the mitral valve. Consequently, MAD has been observed only at the insertion of the posterior leaflet. It can extend preferentially at the central posterior scallop. The first diagnostic modality aiding the diagnosis is transthoracic echocardiography (TTE), although in some cases adjunctive cardiac imaging modality might be suggested. MAD carries a strong association with malignant ventricular arrhythmogenesis and a profound predisposition for sudden cardiac death (SCD). In this context, a thorough investigation of this morphological and functional abnormality is vital in estimating the risk assessment and stratification for optimal management and elimination of the risk of the patient for SCD. Based on the current scientific data and literature, we will discuss the diagnosis, clinical implications, risk stratification, and therapeutic management of MAD.
first_indexed 2024-03-12T22:45:16Z
format Article
id doaj.art-7ea6816a92624c0dba2e970bb324e122
institution Directory Open Access Journal
issn 2297-055X
language English
last_indexed 2024-03-12T22:45:16Z
publishDate 2023-07-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Cardiovascular Medicine
spelling doaj.art-7ea6816a92624c0dba2e970bb324e1222023-07-21T06:36:52ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2023-07-011010.3389/fcvm.2023.11712261171226Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literatureFay Apostolou0Marios Ioannides1Andreas Mitsis2Constantina Koutsofti3Constantinos Deltas4Constantinos Deltas5Panayiotis Avraamides6Department of Cardiology, Nicosia General Hospital,Strovolos, CyprusDepartment of Cardiology, Nicosia General Hospital,Strovolos, CyprusDepartment of Cardiology, Nicosia General Hospital,Strovolos, CyprusBiobank.cy Center of Excellence in Biobanking and Biomedical Research, University of Cyprus,Nicosia, CyprusBiobank.cy Center of Excellence in Biobanking and Biomedical Research, University of Cyprus,Nicosia, CyprusSchool of Medicine, University of Cyprus, Nicosia, CyprusDepartment of Cardiology, Nicosia General Hospital,Strovolos, CyprusMitral annulus disjunction (MAD) is defined as a systolic displacement between the ventricular myocardium and the posterior mitral annulus supporting the posterior mitral leaflet. This structural abnormality is associated with the loss of mechanical annular function manifested as an abnormal systolic excursion of the leaflet hinge point into the left atrium but with maintained electrical function, separating the left atrium and ventricle electrophysiologically. The mitro-aortic fibrous continuity limits MAD anteriorly, between the aortic cusps and the anterior leaflet of the mitral valve. Consequently, MAD has been observed only at the insertion of the posterior leaflet. It can extend preferentially at the central posterior scallop. The first diagnostic modality aiding the diagnosis is transthoracic echocardiography (TTE), although in some cases adjunctive cardiac imaging modality might be suggested. MAD carries a strong association with malignant ventricular arrhythmogenesis and a profound predisposition for sudden cardiac death (SCD). In this context, a thorough investigation of this morphological and functional abnormality is vital in estimating the risk assessment and stratification for optimal management and elimination of the risk of the patient for SCD. Based on the current scientific data and literature, we will discuss the diagnosis, clinical implications, risk stratification, and therapeutic management of MAD.https://www.frontiersin.org/articles/10.3389/fcvm.2023.1171226/fullmitral valve prolapsemitral annulus disjunctionsudden cardiac deathventricular arrhythmiatransthoracic echocardiography
spellingShingle Fay Apostolou
Marios Ioannides
Andreas Mitsis
Constantina Koutsofti
Constantinos Deltas
Constantinos Deltas
Panayiotis Avraamides
Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature
Frontiers in Cardiovascular Medicine
mitral valve prolapse
mitral annulus disjunction
sudden cardiac death
ventricular arrhythmia
transthoracic echocardiography
title Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature
title_full Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature
title_fullStr Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature
title_full_unstemmed Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature
title_short Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature
title_sort case report aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction a case series and review of the literature
topic mitral valve prolapse
mitral annulus disjunction
sudden cardiac death
ventricular arrhythmia
transthoracic echocardiography
url https://www.frontiersin.org/articles/10.3389/fcvm.2023.1171226/full
work_keys_str_mv AT fayapostolou casereportabortedsuddencardiacdeathasafirstpresentationofseveremitralannulusdisjunctionacaseseriesandreviewoftheliterature
AT mariosioannides casereportabortedsuddencardiacdeathasafirstpresentationofseveremitralannulusdisjunctionacaseseriesandreviewoftheliterature
AT andreasmitsis casereportabortedsuddencardiacdeathasafirstpresentationofseveremitralannulusdisjunctionacaseseriesandreviewoftheliterature
AT constantinakoutsofti casereportabortedsuddencardiacdeathasafirstpresentationofseveremitralannulusdisjunctionacaseseriesandreviewoftheliterature
AT constantinosdeltas casereportabortedsuddencardiacdeathasafirstpresentationofseveremitralannulusdisjunctionacaseseriesandreviewoftheliterature
AT constantinosdeltas casereportabortedsuddencardiacdeathasafirstpresentationofseveremitralannulusdisjunctionacaseseriesandreviewoftheliterature
AT panayiotisavraamides casereportabortedsuddencardiacdeathasafirstpresentationofseveremitralannulusdisjunctionacaseseriesandreviewoftheliterature