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Review

Arrhythmogenic Right Ventricular Cardiomyopathy Overview

Elizabeth McNally  1 , Heather MacLeod  2 , Lisa Dellefave-Castillo  1
Margaret P Adam, Jerry Feldman, Ghayda M Mirzaa, Roberta A Pagon, Stephanie E Wallace, Anne Amemiya
, editors.
In: GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993.
[updated ].
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Review

Arrhythmogenic Right Ventricular Cardiomyopathy Overview

Elizabeth McNally et al.
Free Books & Documents

Excerpt

The purpose of this overview is to:

  1. 1

    Describe the clinical characteristics of arrhythmogenic right ventricular cardiomyopathy (ARVC);

  2. 2

    Review the genetic causes of ARVC;

  3. 3

    Provide an evaluation strategy to identify the genetic cause of ARVC in a proband;

  4. 4

    Review management of ARVC;

  5. 5

    Inform genetic risk assessment in family members of a proband.

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References

    1. Abicht A, Schön U, Laner A, Holinski-Feder E, Diebold I. Actionable secondary findings in arrhythmogenic right ventricle cardiomyopathy genes: impact and challenge of genetic counseling. Cardiovasc Diagn Ther. 2021;11:637-49. - PMC - PubMed
    1. Akdis D, Saguner AM, Shah K, Wei C, Medeiros-Domingo A, von Eckardstein A, Lü-scher TF, Brunckhorst C, Chen HSV, Duru F. Sex hormones affect outcome in ar-rhythmogenic right ventricular cardiomyopathy/dysplasia: from a stem cell derived car-diomyocyte-based model to clinical biomarkers of disease outcome. Eur Heart J. 2017;38:1498-508. - PMC - PubMed
    1. Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, Deal BJ, Dickfeld T, Field ME, Fonarow GC, Gillis AM, Granger CB, Hammill SC, Hlatky MA, Joglar JA, Kay GN, Matlock DD, Myerburg RJ, Page RL. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018;15:e73-e189. - PubMed
    1. Ammirati E, Raimondi F, Piriou N, Sardo Infirri L, Mohiddin SA, Mazzanti A, Shenoy C, Cavallari UA, Imazio M, Aquaro GD, Olivotto I, Pedrotti P, Sekhri N, Van de Heyning CM, Broeckx G, Peretto G, Guttmann O, Dellegrottaglie S, Scatteia A, Gentile P, Merlo M, Goldberg RI, Reyentovich A, Sciamanna C, Klaassen S, Poller W, Trankle CR, Abbate A, Keren A, Horowitz-Cederboim S, Cadrin-Tourigny J, Tadros R, Annoni GA, Bonoldi E, Toquet C, Marteau L, Probst V, Trochu JN, Kissopoulou A, Grosu A, Kukavica D, Trancuccio A, Gil C, Tini G, Pedrazzini M, Torchio M, Sinagra G, Gimeno JR, Bernasconi D, Valsecchi MG, Klingel K, Adler ED, Camici PG, Cooper LT Jr. Acute myocarditis associated with desmosomal gene variants. JACC Heart Fail. 2022;10:714-27. - PubMed
    1. Bao J, Wang J, Yao Y, Wang Y, Fan X, Sun K, He DS, Marcus FI, Zhang S, Hui R, Song L. Correlation of ventricular arrhythmias with genotype in arrhythmogenic right ventricular cardiomyopathy. Circ Cardiovasc Genet. 2013;6:552-6. - PubMed

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