ARVC/D 2010 Revised Task Force Guidelines
Other criteria
-notched S V1-V3 (Espilon)
-LBBB VT with PRWP V1-V4

ETIOLOGY
Mayocarditis as a trigger for expression of genes that produce fibrosis in response to inflammation rather than a degenerative
pocess?

ABLATION
Linear ablation lesions
There is data about improved outcome with epicardial mapping and ablation


ICD IIa
ARVD/C with 1 or more risk factors for SCD
(IIa - LOE: C)
         
 + EPS, NSVT on noninvasive monitoring, male, severe RV dilation, and extensive RV involvement. Young age at presentation (less than 5 years), LV
involvement, prior cardiac arrest, and unexplained syncope. Genotypes of ARVD/C associated with a high risk for SCD should be considered for ICD therapy (locus on
chromosome 1q42–43 for RV aneurysm, C3P25). Previous cardiac arrest, syncope due to VT, Polymorphic VT and RVA aneurysm, decreased EF

If history of sustained VT => 2 Prevention ICD (85% ICD discharge, 28% VT/VF>240 bpm)
If no history of sustained VT => 1 Prevention ICD (39% CD discharge, 15% VT/VF > 240 bpm)
Piccini Heart Rhythm 2005;2:1188
Circ 2010 : 1144-52 - Experience on syncope and NSVT

Med Rx: SOTALOL

GENETICS:
-ARVC2-RYR2 gene locus on chromosome C1q42-43 for RV aneurysm, which codes for the ryanodine receptor
-C3P25 genotype
-Naxos disease (wooly hair + palmoplantar keratoma) - mutation JUP gene C17q21 encoding for desmosomal protein
plakoglobin
-ARVC2-DSP geneon C6p24 codes for desmosomal protein desmoplakin
-ARVC9-PKP2 gene on C12p11 codes for desmosomal arm repeat protein plakophilin 2 (27-43% of patients)