ARVD2

Arrhythmogenic right ventricular dysplasia Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 2; arrhythmogenic right ventricular cardiomyopathy 2; ARVC2

Overview

Arrhythmogenic right ventricular dysplasia type 2 is a "concealed form" of ARVD. There is no change in heart size. There are no EKG changes on the resting electrocardiogram, but there may be exercise induced polymorphic ventricular tachycardia. This variant is associated with premature death.

Pathophysiology

Although the heart is normal in size, on pathologic examination, there are large areas of fibro-fatty replacement in the subepicardial layer of the right ventricle.

There are also abnormalities in calcium hemostasis in the myocytes which may contribute to the occurrence of ventricular arrhythmias.

Genetics

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

Natural History, Complications, Prognosis

This ARVD variant is associated with premature death.

Diagnosis

Symptoms

Exercise induced polymorphic VT may be present.

Electrocardiogram

There are no EKG changes on the resting electrocardiogram, but there may be exercise induced polymorphic ventricular tachycardia.