Arrhythmogenic right ventricular dysplasia (ARVD) is a heritable form of cardiomyopathy, characterized by the replacement of myocytes with adipose and fibrous tissue leading to arrhythmias, right ventricular failure, and sudden cardiac death (SCD)
The areas of the myocardium most affected are localized to the the inflow tract, outflow tract, and apex of the right ventricle (triangle of dysplasia)
Most common symptoms are palpitations, syncope, and SCD in 27, 26, and 23% of patients, respectively
ECG findings include T-wave inversions in V1–V3 (85% ), epsilon waves (in 33%), as well as a QRS duration >110 ms in V1-V3 (64%)
Dx is based on a combination of characteristics family history, ECG/arrhythmia, cardiac imaging (MRI/Echo), and endomyocardial biopsy
ARVD patients are at high risk for sudden cardiac death and often recommended ICD placement
References
Azaouagh A, Churzidse S, Konorza T, Erbel R. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a review and update. Clin Res Cardiol (2011) 100:383–394.
