Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy

Quarta, Giovanni; Elliott, Perry

doi:10.1016/j.rec.2012.02.015

Cited by 18 publications

(15 citation statements)

References 35 publications

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“…This has been empirically demonstrated by differences in groups with the proposed set of parameters. These findings are consistent with the ECG Task Force diagnostic criteria, since fragmentation of QRS or epsilon wave among others suggest a higher level of heterogeneity of the electrical activation in ACM patients [4][5][6]. However, it would be advisable to use a combination of parameters to improve these results.…”

Section: Discussionsupporting

confidence: 86%

“…Certain ECG morphological changes have been identified as indicative of possible ACM. Among others, T-wave inversion in the right precordial leads (V1-V3), parameters related to the duration, amplitude, and fragmentation of the QRS complex along with epsilon wave appearance in different derivations have been described [4][5][6]. However, other indicators that have proven ECG diagnostic efficacy in other diseases of ventricular origin have not been tested in ACM.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Study of New Criteria Based on Eigenvalue Decomposition to Assist Arrhythmogenic Cardiomyopathy Diagnosis

Jiménez-Serrano

Sánchez

Cebrián

et al. 2016

2016 Computing in Cardiology Conference (CinC)

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Section: Discussionsupporting

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Study of New Criteria Based on Eigenvalue Decomposition to Assist Arrhythmogenic Cardiomyopathy Diagnosis

Jiménez-Serrano

Sánchez

Cebrián

et al. 2016

2016 Computing in Cardiology Conference (CinC)

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“…4 It is among the most common causes of sudden cardiac death (SCD) in people ≤35 years of age. 3 The actual prevalence may be greater because the diagnosis may be missed clinically and at postmortem examination.…”

Section: Natural History Of Arrhythmogenic Cardiomyopathymentioning

confidence: 99%

Pathogenesis of Arrhythmogenic Cardiomyopathy

Asimaki

Kléber

Saffitz

2015

Canadian Journal of Cardiology

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Arrhythmogenic cardiomyopathy (ACM) is a primary myocardial disease. It is characterized by frequent ventricular arrhythmias and increased risk of sudden cardiac death typically arising as an early manifestation before the onset of significant myocardial remodeling. Myocardial degeneration, often confined to the right ventricular free wall, with replacement by fibrofatty scar tissue, develops in many patients. ACM is a familial disease but genetic penetrance can be low and disease expression is highly variable. Inflammation may promote disease progression. It also appears that exercise increases disease penetrance and accelerates its development. More than 60% of probands harbor mutations in genes encoding desmosomal proteins, which has raised the possibility that defective cell-cell adhesion may play a role in disease pathogenesis. Recent advances have implicated changes in the canonical Wnt/β-catenin and Hippo signaling pathways and defects in forwarding trafficking of ion channels and other proteins to the intercalated disk in cardiac myocytes. This review summarizes current understanding of the pathogenesis of ACM and highlights future research directions.

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“…1 , Заклязьминская E. В. 1,3 Цель. Анализ результатов практического применения ДНК-диагностики двух наиболее частых форм аритмогенной кардиомиопатии правого желудочка (АКПЖ) в работе кардиологического и кардиохирургического стационаров.…”

Section: опыт практического использования днк-диагностики при аритмогunclassified

“…Полученные на российской выборке больных результаты согла-суются с данными о представленности двух частых генетических форм АКПЖ в других этнических группах. По разным оценкам, распространенность этого вида кардиомиопатии в мире варьирует от 1:1000 до 1:5000 населения [3]. В России масштабных эпиде-миологических исследований проблемы не проводи-лось, поэтому частота встречаемости АКПЖ досто-верно неизвестна.…”

Section: опыт практического использования днк-диагностики при аритмогunclassified

A Case of Dna-Diagnostics Application for Arrhythmogenic Right Ventricle Cardiomyopathy

Shestak¹,

Lutokhina²,

Фролова³

et al. 2016

Russ J Cardiol

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Аритмогенная кардиомиопатия правого желу-дочка (АКПЖ, MIM*#107970) -генетически детер-минированное заболевание миокарда с высоким риском внезапной сердечной смерти (ВСС), характе-ризующееся фиброзным и/или жировым замеще-нием кардиомиоцитов преимущественно правого желудочка (ПЖ), частыми желудочковыми наруше-ниями ритма, ведущее к развитию сердечной недо-статочности. В ряде случаев жизнеугрожающие желу-дочковые аритмии могут возникнуть до развития явного структурного и функционального ремодели-рования миокарда (так называемая "скрытая" фаза АКПЖ), что значительно затрудняет раннюю диагно-стику заболевания. Нередко первым и единственным симптомом болезни является ВСС. Патоморфологи-ческая диагностика лиц, умерших внезапно в возрас-те до 35 лет показала, что на долю недиагностирован-ной АКПЖ приходится значительная часть случаев ВСС: 12,5-25% в Италии, 17% в США, 14,1% Шестак А. Г.* -н. с. лаборатории медицинской генетики, Благова О. В. -д. м.н., профессор кафедры факультетской терапии №1 лечебного факультета, Луто-хина Ю. А. -аспирант по специальности "Кардиология" кафедры факультет-ской терапии №1 лечебного факультета, Фролова Ю. В. -д. м.н., в. н.с. отделе-ния хирургического лечения дисфункций миокарда и сердечной недостаточно-сти, Дземешкевич С. Л. -д. м.н., профессор, зав. отделением хирургического лечения дисфункций миокарда и сердечной недостаточности, Заклязьмин-ская Е. В. -д. м.н., профессор, зав. лабораторией медицинской генетики. Aim. Analysis of the results of dnA-diagnostics in practice, for the two most common types of arrhythmogenic cardiomyopathies of the right ventricle (ACRV) in cardiovascular and surgery setting. Material and methods. Clinical and laboratory study performed, of the selection of 38 patients with a diagnosis at presentation -ACRV, with consequent genetic counseling and dnA-diagnostics for 2 most common types of ACRV. Results. Mutations in two genes responsible for the most common ACRV types, were found in 22,9% of patients. Analysis of occurrence rate was done for mutations in groups with definite, probable and possible diagnoses of ACRV, and of total impact of genetic data on diagnostics of the disease. Conclusion. data obtained on the Russian selection of patients shows similarity with the data on prevalence of two most common genetic forms of ACRV in other ethnicities. Russ

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Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy

Cited by 18 publications

References 35 publications

Study of New Criteria Based on Eigenvalue Decomposition to Assist Arrhythmogenic Cardiomyopathy Diagnosis

Study of New Criteria Based on Eigenvalue Decomposition to Assist Arrhythmogenic Cardiomyopathy Diagnosis

Pathogenesis of Arrhythmogenic Cardiomyopathy

A Case of Dna-Diagnostics Application for Arrhythmogenic Right Ventricle Cardiomyopathy

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