Characteristics of Left-Dominant Arrhythmogenic Cardiomyopathy

Imamura, Yasutaka; Uto, Kenta; Nagao, Michinobu; Nagara, Kimiko; Yoshizawa, Saeko; Kikuchi, Noriko; Kimura, Yuya; Hagiwara, Nobuhisa

doi:10.1253/circj.cj-21-0571

Cited by 2 publications

(2 citation statements)

References 2 publications

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“…Numerous clinical cases described in the literature report whether left arrhythmogenic cardiomyopathy is associated with left ventricular dysfunction and genetic mutations [20][21][22][23][24][25][26][27][28][29][30][31].…”

Section: Left Ventricular Involvement In Arrhythmogenic Cardiomyopathymentioning

confidence: 99%

Arrhythmogenic Cardiomyopathy: Definition, Classification and Arrhythmic Risk Stratification

Varrenti,

Preda,

Frontera

et al. 2024

JCM

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Arrhythmogenic cardiomyopathy (ACM) is a heart disease characterized by a fibrotic replacement of myocardial tissue and a consequent predisposition to ventricular arrhythmic events, especially in the young. Post-mortem studies and the subsequent diffusion of cardiac MRI have shown that left ventricular involvement in arrhythmogenic cardiomyopathy is common and often develops early. Regarding the arrhythmic risk stratification, the current scores underestimate the arrhythmic risk of patients with arrhythmogenic cardiomyopathy with left involvement. Indeed, the data on arrhythmic risk stratification in this group of patients are contradictory and not exhaustive, with the consequence of not correctly identifying patients at a high arrhythmic risk who deserve protection from arrhythmic death. We propose a literature review on arrhythmic risk stratification in patients with ACM and left involvement to identify the main features associated with an increased arrhythmic risk in this group of patients.

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Section: Left Ventricular Involvement In Arrhythmogenic Cardiomyopathymentioning

confidence: 99%

Arrhythmogenic Cardiomyopathy: Definition, Classification and Arrhythmic Risk Stratification

Varrenti,

Preda,

Frontera

et al. 2024

JCM

View full text Add to dashboard Cite

show abstract

“…In addition to ARVC, a left dominant arrhythmogenic cardiomyopathy (LDAC) also exists, wherein the lateral and postero-lateral aspects of the LV are preferentially involved and early on in the disease course. LDAC can be difficult to differentiate from ARVC, as patients often meet Task Force Criteria for ARVC [14]. ARVC is classically associated with a right bundle branch block compared to left bundle branch block in the case of LDAC.…”

Section: Definition and Epidemiologymentioning

confidence: 99%

Arrhythmogenic Right Ventricular Cardiomyopathy: The Role of Genetics in Diagnosis, Management, and Screening

et al. 2022

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a predominantly autosomal dominant genetic condition in which fibrous and fatty tissue infiltrate and replace healthy myocardial tissue. This uncommon yet debilitating condition can cause ventricular arrhythmias, cardiac failure, and sudden cardiac death. Management focuses primarily on prevention of syndrome sequelae in order to prevent morbidity and mortality. Genetic testing and screening in affected families, although utilized clinically, has not yet been incorporated in guidelines due to lack of larger studies and data. We aim herein to identify causative gene mutations, present advancements in diagnosis and management, and describe the role of genetic screening and counseling in patients with ARVC. With the advancement of genetic testing and therapy, diseases such as ARVC may become more accurately diagnosed and more effectively managed, ultimately significantly reducing morbidity and mortality.

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Characteristics of Left-Dominant Arrhythmogenic Cardiomyopathy

Cited by 2 publications

References 2 publications

Arrhythmogenic Cardiomyopathy: Definition, Classification and Arrhythmic Risk Stratification

Arrhythmogenic Cardiomyopathy: Definition, Classification and Arrhythmic Risk Stratification

Arrhythmogenic Right Ventricular Cardiomyopathy: The Role of Genetics in Diagnosis, Management, and Screening

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