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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...

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Autores principales: Apostolou, Fay, Ioannides, Marios, Mitsis, Andreas, Koutsofti, Constantina, Deltas, Constantinos, Avraamides, Panayiotis
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10400284/
https://www.ncbi.nlm.nih.gov/pubmed/37547253
http://dx.doi.org/10.3389/fcvm.2023.1171226
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author Apostolou, Fay
Ioannides, Marios
Mitsis, Andreas
Koutsofti, Constantina
Deltas, Constantinos
Avraamides, Panayiotis
author_facet Apostolou, Fay
Ioannides, Marios
Mitsis, Andreas
Koutsofti, Constantina
Deltas, Constantinos
Avraamides, Panayiotis
author_sort Apostolou, Fay
collection PubMed
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.
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spelling pubmed-104002842023-08-04 Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature Apostolou, Fay Ioannides, Marios Mitsis, Andreas Koutsofti, Constantina Deltas, Constantinos Avraamides, Panayiotis Front Cardiovasc Med Cardiovascular Medicine 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. Frontiers Media S.A. 2023-07-20 /pmc/articles/PMC10400284/ /pubmed/37547253 http://dx.doi.org/10.3389/fcvm.2023.1171226 Text en © 2023 Apostolou, Ioannides, Mitsis, Koutsofti, Deltas and Avraamides. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Cardiovascular Medicine
Apostolou, Fay
Ioannides, Marios
Mitsis, Andreas
Koutsofti, Constantina
Deltas, Constantinos
Avraamides, Panayiotis
Case report: Aborted sudden cardiac death as a first presentation of severe mitral annulus disjunction—a case series and review of the literature
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 Cardiovascular Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10400284/
https://www.ncbi.nlm.nih.gov/pubmed/37547253
http://dx.doi.org/10.3389/fcvm.2023.1171226
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