Arrhythmogenic ventricular cardiomyopathy: A paradigm shift from right to biventricular disease

doi:10.4330/wjc.v6.i4.154

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World Journal of Cardiology

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1949-8462

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

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World J Cardiol. Apr 26, 2014; 6(4): 154-174
Published online Apr 26, 2014. doi: 10.4330/wjc.v6.i4.154

Table 2 Arrhythmogenic ventricular cardiomyopathy classification, from OMIMTM Online Mendelian inheritance in Man

AVC subtype	Chromosome/locus	Mode of transmission	Encoded protein
ARVC/D 1	14q23-q24	Autosomal-dominant	TGFβ3
ARVC/D 2	1q42-q43	Autosomal-dominant	RyR2
ARVC/D 3	14q12-q22	Autosomal-dominant	-
ARVC/D 4	2q32	Autosomal-dominant	TTN
ARVC/D 5	3p23	Autosomal-dominant	TMEM43
ARVC/D 6	10p12-p14	Autosomal-dominant	-
ARVC/D 7	10q22	Autosomal-dominant	-
ARVC/D 8	6p24	Autosomal-dominant	DSP
ARVC/D 9	12p11	Autosomal-dominant	PKP2
ARVC/D 10	18q12	Autosomal-dominant	DSG2
ARVC/D 11	18q12.1	Autosomal-dominant	DSC2
ARVC/D 12	17q21	Autosomal-dominant	JUP
Naxos disease	17q21	Autosomal-recessive	JUP

AVC: Arrhythmogenic ventricular cardiomyopathy; ARVC/D: Arrhythmogenic right ventricular cardiomyopathy/dysplasia; TGF: Transforming growth factor; RyR2: Ryanodine receptor 2; TTN: Titin; TMEM43: Transmembrane protein 43; DSP: Desmoplakin; PKP2: Plakophilin-2; DSG2: Desmoglein-2; DSC2: Desmocollin-2; JUP: Junction plakoglobin.

Citation: Saguner AM, Brunckhorst C, Duru F. Arrhythmogenic ventricular cardiomyopathy: A paradigm shift from right to biventricular disease. World J Cardiol 2014; 6(4): 154-174
URL: https://www.wjgnet.com/1949-8462/full/v6/i4/154.htm
DOI: https://dx.doi.org/10.4330/wjc.v6.i4.154