Arrhythmogenic right ventricular dysplasia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

ARVD1

This variant is due to a heterozygous mutation in the TGFB3 gene (190230) on chromosome 14q24.

ARVD2

This variant (600996) is associated with a mutation in the RYR2 gene (180902) on chromosome 1q42-q43.

ARVD3

This variant (602086) is associated with a mutation in the chromosome 14q12-q22 region.

ARVD4

This variant (602087) is associated with a mutation in the chromosome 2q32.1-q32.3 region.

ARVD5

This variant (604400) is associated with a mutation in the TMEM43 gene (612048) on chromosome 3p23 region.

ARVD6

This variant (604401), is associated with a mutation in the chromosome 10p14-p12 region.

ARVD7

This variant 609160) is associated with a mutation in the chromosome 10q22.3 region.

ARVD8

This variant 607450) is associated with a mutation in the DSP gene (125647) on chromosome 6p24.

ARVD9

This variant (609040) is associated with a mutation in the PKP2 gene (602861) on chromosome 12p11.

ARVD10

This variant (610193) is associated with a mutation in the DSG2 gene (125671) on chromosome 18q12.1-q12.

ARVD11

This variant (610476) is associated with a mutation in the DSC2 gene (125645) on chromosome 18q12.1.

ARVD12

This variant (611528) is associated with a mutation in the JUP gene (173325) on chromosome 17q21.

Ventricular arrhythmias

Monomorphic ventricular tachycardia Right ventricular outflow tract tachycardia

Monomorphic ventricular tachycardia originating from the right ventricular outflow tract.

Ventricular arrhythmias due to ARVD typically arise from the diseased right ventricle. The type of arrhythmia ranges from frequent premature ventricular complexes (PVCs) to ventricular tachycardia (VT) to ventricular fibrillation (VF).

While the initiating factor of the ventricular arrhythmias is unclear, it may be due to triggered activity or reentry.

Ventricular arrhythmias are usually exercise-related, suggesting that they are sensitive to catecholamines. The ventricular beats typically have a right axis deviation. Multiple morphologies of ventricular tachycardia may be present in the same individual, suggesting multiple arrhythmogenic foci or pathways.

Right ventricular outflow tract (RVOT) tachycardia is the most common VT seen in individuals with ARVD. In this case, the EKG shows a left bundle branch block (LBBB) morphology with an inferior axis.