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Mitral annular disjunction: Beyond mitral valve prolapse

Mitral annular disjunction (MAD) is an easily identifiable entity on transthoracic echocardiography, but is still poorly recognized or ignored. It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death, but the management and r...

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Published in:Revista portuguesa de cardiologia 2023-10, Vol.42 (10), p.873-878
Main Authors: Faria, Bebiana, Ribeiro, Sílvia, Calvo, Lucy, Oliveira, Margarida, von Hafe, Pedro, Bettencourt, Nuno, Sanfins, Victor, Lourenço, António
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creator Faria, Bebiana
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description Mitral annular disjunction (MAD) is an easily identifiable entity on transthoracic echocardiography, but is still poorly recognized or ignored. It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death, but the management and risk stratification of these patients is not systematized. Two clinical cases of MAD associated with mitral valve prolapse and ventricular arrhythmias are presented. The first case is of a patient with a history of surgical intervention on the mitral valve due to Barlow's disease. He presented to the emergency department with sustained monomorphic ventricular tachycardia requiring emergent electrical cardioversion. MAD with transmural fibrosis at the level of the inferolateral wall was documented. The second report is of a young woman with palpitations and frequent premature ventricular contractions on Holter with documentation of valvular prolapse and MAD, and focuses on the risk stratification approach. The present article offers a review of the literature regarding the arrhythmic risk of MAD and mitral valve prolapse, as well as a review of risk stratification in these patients. A disjunção do anel mitral é uma entidade facilmente identificável em ecocardiografia transtorácica, mas ainda pouco reconhecida e valorizada. Encontra-se frequentemente associada ao prolapso da válvula mitral e representa por si só um marcador de risco para arritmias ventriculares e morte súbita cardíaca, contudo a orientação e a estratificação de risco destes doentes não se encontram sistematizadas.Dois casos clínicos de disjunção do anel mitral associado a prolapso da válvula mitral e arritmias ventriculares são apresentados. O primeiro caso retrata um doente com antecedentes de plastia valvular mitral por doença de Barlow, que recorre ao SU por quadro de palpitações mal tolerado com documentação de taquicardia ventricular monomórfica sustentada com necessidade de cardioversão elétrica emergente. Do estudo efetuado documenta-se disjunção do anel mitral com fibrose transmural ao nível da parede inferolateral. O segundo caso clínico relata o caso de uma jovem com palpitações e extrassistolia ventricular muito frequente no Holter com documentação de prolapso valvular e disjunção do anel mitral e centra-se na abordagem da estratificação de risco. O presente artigo oferece uma revisão da literatura relativamente ao risco arrítmico da disjunção do anel mitral e prolapso da válvula mitral
doi_str_mv 10.1016/j.repc.2019.09.024
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It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death, but the management and risk stratification of these patients is not systematized. Two clinical cases of MAD associated with mitral valve prolapse and ventricular arrhythmias are presented. The first case is of a patient with a history of surgical intervention on the mitral valve due to Barlow's disease. He presented to the emergency department with sustained monomorphic ventricular tachycardia requiring emergent electrical cardioversion. MAD with transmural fibrosis at the level of the inferolateral wall was documented. The second report is of a young woman with palpitations and frequent premature ventricular contractions on Holter with documentation of valvular prolapse and MAD, and focuses on the risk stratification approach. 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It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death, but the management and risk stratification of these patients is not systematized. Two clinical cases of MAD associated with mitral valve prolapse and ventricular arrhythmias are presented. The first case is of a patient with a history of surgical intervention on the mitral valve due to Barlow's disease. He presented to the emergency department with sustained monomorphic ventricular tachycardia requiring emergent electrical cardioversion. MAD with transmural fibrosis at the level of the inferolateral wall was documented. The second report is of a young woman with palpitations and frequent premature ventricular contractions on Holter with documentation of valvular prolapse and MAD, and focuses on the risk stratification approach. The present article offers a review of the literature regarding the arrhythmic risk of MAD and mitral valve prolapse, as well as a review of risk stratification in these patients. A disjunção do anel mitral é uma entidade facilmente identificável em ecocardiografia transtorácica, mas ainda pouco reconhecida e valorizada. Encontra-se frequentemente associada ao prolapso da válvula mitral e representa por si só um marcador de risco para arritmias ventriculares e morte súbita cardíaca, contudo a orientação e a estratificação de risco destes doentes não se encontram sistematizadas.Dois casos clínicos de disjunção do anel mitral associado a prolapso da válvula mitral e arritmias ventriculares são apresentados. O primeiro caso retrata um doente com antecedentes de plastia valvular mitral por doença de Barlow, que recorre ao SU por quadro de palpitações mal tolerado com documentação de taquicardia ventricular monomórfica sustentada com necessidade de cardioversão elétrica emergente. 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subjects Arrhythmias, Cardiac
Death, Sudden, Cardiac
Disjunção anel mitral
Echocardiography
Female
Humans
Male
Mitral annular disjunction
Mitral Valve - diagnostic imaging
Mitral valve prolapse
Mitral Valve Prolapse - complications
Morte súbita cardíaca
Prolapso mitral
Sudden cardiac death
title Mitral annular disjunction: Beyond mitral valve prolapse
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