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Arrhythmogenic right ventricular dysplasia Kumaresan Dr. Kim Vaiphei
Introduction ,[object Object],[object Object],[object Object]
Incidence/prevalence ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pathophysiology  ,[object Object],[object Object],[object Object]
Triangle of dysplasia
Genetic factors ,[object Object],[object Object],[object Object],[object Object],[object Object]
Chromosomal loci and causal genes Chromosome   Symbol  Protein  Function  ARVD/C 1 14q24.3 TGF  3 TGF  3 Mitotic factor ARVD/C 2 14q42.2 RYR 2 Ryanodine receptor 2 Ca channel ARVD/C 3 1q12 ARVD/C 4 2q32.1 ARVD/C 5 3p23 ARVD/C 6 10p12 ARVD/C 7 10q22 ARVD/C 8 6p24 DSP Desmoplakin Desmosomes ARVD/C 9 12p11 PKP 2 Plakoglobin 2 Desmosomes 18q12.1 DSG 2 Desmoglein 2 Desmosomes Naxos disease 17q21 JUP Plakoglobin Desmosomes
[object Object],[object Object],[object Object],[object Object]
Autosomal recessive variant of ARVD/C  ,[object Object],[object Object],[object Object],[object Object]
 
Etiopathogenesis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object]
[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object]
[object Object]
Gross  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
Histology ,[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object]
Fatty infiltration ,[object Object],[object Object],[object Object],[object Object],[object Object]
Fibro-fatty infiltration ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object]
 
 
Phases of ARVD ,[object Object],[object Object],[object Object]
[object Object],[object Object]
Clinical presentation ,[object Object],[object Object],[object Object],[object Object]
 
Diagnosis  ,[object Object],[object Object]
Diagnosis ,[object Object],[object Object],[object Object]
Diagnostic Criteria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
ECG ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Epsilon wave
RV contrast angiography ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Echocardiography ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
Uhl’s anomaly ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object]
Pathology  ,[object Object],[object Object],[object Object]
Exercise and ventricular arrhythmias ,[object Object],[object Object],[object Object],[object Object],[object Object]
Natural history ,[object Object],[object Object],[object Object],[object Object],[object Object]
Treatment ,[object Object],[object Object],[object Object],[object Object],[object Object]

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Arrhythmogenic right ventricular 2003

  • 1. Arrhythmogenic right ventricular dysplasia Kumaresan Dr. Kim Vaiphei
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  • 7. Chromosomal loci and causal genes Chromosome Symbol Protein Function ARVD/C 1 14q24.3 TGF  3 TGF  3 Mitotic factor ARVD/C 2 14q42.2 RYR 2 Ryanodine receptor 2 Ca channel ARVD/C 3 1q12 ARVD/C 4 2q32.1 ARVD/C 5 3p23 ARVD/C 6 10p12 ARVD/C 7 10q22 ARVD/C 8 6p24 DSP Desmoplakin Desmosomes ARVD/C 9 12p11 PKP 2 Plakoglobin 2 Desmosomes 18q12.1 DSG 2 Desmoglein 2 Desmosomes Naxos disease 17q21 JUP Plakoglobin Desmosomes
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