Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points

Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a cont...

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Autores principales:	Drake A. Comber, MSc, Ashley DeGraaf, MSc, Derek Human, BM BCh, MA, Amanda Barlow, MD, Praveen Indraratna, MBBS, Gnalini Sathananthan, MBBS, Zachary Laksman, MD, MSc
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Diseases of the circulatory (Cardiovascular) system RC666-701
Acceso en línea:	https://doaj.org/article/7ef99fff747d48e2ac4b84eb48be33b7
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spelling	oai:doaj.org-article:7ef99fff747d48e2ac4b84eb48be33b72021-11-30T04:17:23ZArrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points2589-790X10.1016/j.cjco.2021.06.013https://doaj.org/article/7ef99fff747d48e2ac4b84eb48be33b72021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2589790X21001669https://doaj.org/toc/2589-790XPatients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD. This case series describes 3 patients in an adult HAD clinic who have concomitant mitral valve prolapse, MAD, and malignant arrhythmias. These cases may represent a unique disease entity or overlap syndrome, and they introduce MAD as a potential arrhythmogenic risk marker in HAD. Résumé: Les patients atteints de maladie aortique héréditaire (MAH) présentent un risque accru d’arythmie ventriculaire et de mort subite d’origine cardiaque. Bien que le prolapsus valvulaire mitral soit fréquent dans les cas de MAH, la disjonction annulaire mitrale (DAM) n’a été décrite que récemment chez ces patients. Cet état méconnu peut être un facteur contribuant à des arythmies ventriculaires autrement inexpliquées et à la mort subite d’origine cardiaque chez les patients atteints de MAH. Cette série de cas décrit trois patients d’une clinique de MAH pour adultes qui présentent un prolapsus valvulaire mitral, une DAM et des arythmies malignes en concomitance. Ces cas peuvent représenter une entité morbide unique ou un syndrome de chevauchement, et laissent entendre que la DAM pourrait être un nouveau marqueur du risque arythmogène associé à la MAH.Drake A. Comber, MScAshley DeGraaf, MScDerek Human, BM BCh, MAAmanda Barlow, MDPraveen Indraratna, MBBSGnalini Sathananthan, MBBSZachary Laksman, MD, MScElsevierarticleDiseases of the circulatory (Cardiovascular) systemRC666-701ENCJC Open, Vol 3, Iss 11, Pp 1396-1399 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the circulatory (Cardiovascular) system RC666-701
spellingShingle	Diseases of the circulatory (Cardiovascular) system RC666-701 Drake A. Comber, MSc Ashley DeGraaf, MSc Derek Human, BM BCh, MA Amanda Barlow, MD Praveen Indraratna, MBBS Gnalini Sathananthan, MBBS Zachary Laksman, MD, MSc Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points
description	Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD. This case series describes 3 patients in an adult HAD clinic who have concomitant mitral valve prolapse, MAD, and malignant arrhythmias. These cases may represent a unique disease entity or overlap syndrome, and they introduce MAD as a potential arrhythmogenic risk marker in HAD. Résumé: Les patients atteints de maladie aortique héréditaire (MAH) présentent un risque accru d’arythmie ventriculaire et de mort subite d’origine cardiaque. Bien que le prolapsus valvulaire mitral soit fréquent dans les cas de MAH, la disjonction annulaire mitrale (DAM) n’a été décrite que récemment chez ces patients. Cet état méconnu peut être un facteur contribuant à des arythmies ventriculaires autrement inexpliquées et à la mort subite d’origine cardiaque chez les patients atteints de MAH. Cette série de cas décrit trois patients d’une clinique de MAH pour adultes qui présentent un prolapsus valvulaire mitral, une DAM et des arythmies malignes en concomitance. Ces cas peuvent représenter une entité morbide unique ou un syndrome de chevauchement, et laissent entendre que la DAM pourrait être un nouveau marqueur du risque arythmogène associé à la MAH.
format	article
author	Drake A. Comber, MSc Ashley DeGraaf, MSc Derek Human, BM BCh, MA Amanda Barlow, MD Praveen Indraratna, MBBS Gnalini Sathananthan, MBBS Zachary Laksman, MD, MSc
author_facet	Drake A. Comber, MSc Ashley DeGraaf, MSc Derek Human, BM BCh, MA Amanda Barlow, MD Praveen Indraratna, MBBS Gnalini Sathananthan, MBBS Zachary Laksman, MD, MSc
author_sort	Drake A. Comber, MSc
title	Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points
title_short	Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points
title_full	Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points
title_fullStr	Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points
title_full_unstemmed	Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points
title_sort	arrhythmic mitral valve prolapse and mitral annulus disjunction in heritable aortic diseasenovel teaching points
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/7ef99fff747d48e2ac4b84eb48be33b7
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Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic DiseaseNovel Teaching Points

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