Arrhythmogenic right ventricular dysplasia/cardiomyopathy: an electrocardiogram-based review

Abstract

Arrhythmogenic right ventricular dysplasia or cardiomyopathy (ARVD/C) is a pathologic condition where the right ventricle is partially or totally replaced by fatty and fibrous tissue. The electrocardiogram (ECG) has a central role for diagnosis since it comprises two major and two minor criteria in the diagnostic criteria published by the Task 1 Force, although it is not 100% necessary to make a final diagnosis, because around 10% of patients with ARVD/C present with a normal ECG. In this article, we review the 12-lead electrocardiographic findings of patients with ARVD/C. ECG criteria observed during depolarization [prolonged terminal activation duration, epsilon wave, partial right bundle branch block (RBBB) and advanced RBBB of peripheral origin with characteristic ECG patterns] and repolarization abnormalities, (negative T waves) are reviewed in detail. More common ventricular arrhythmias and risk of sudden death, and how to use the surface ECG to stratify the risk, are part of our final comments.

Electrocardiographic phenotype of exercise-induced arrhythmogenic cardiomyopathy: A retrospective observational study

“…- The same was observed for VA: in Gen-ACM and ExI-ACM VA were much more prevalent and certainly more complex than in End and Sed. This also is in concordance with many previous observations ( 7 ). Indeed, the presence of LBBB morphology ventricular arrhythmias is, depending on the axis, considered a minor (inferior or indeterminate axis) or major (superior axis) criterion for the diagnosis of ACM ( 12 ).…”

“…One of the ECG traits of ACM with an identified desmosomal gene mutation (Gen-ACM) is activation delay over the RV ( 7 ). This can manifest as:…”

Deep T wave inversions in a young patient with chest pain

“…The rest of the answers cause a pseudo-RBBB morphology, the epsilon wave in the case of ARVC, the J wave in the case of Brugada Syndrome and hypothermia, and the retrograde P wave in the case of AVNRT being confounded with the second R wave. 2 …”

Repolarization abnormalities unmasked with a 252‐lead BSM system in patients with ARVC and healthy gene carriers

“…The 12‐lead ECG has a central role in the diagnosis of ARVC revealing both depolarization and repolarization abnormalities, of which T wave inversions in V1‐V3 and beyond is a pathognomonic feature in ARVC 2,4,5 . The initial ECG can be normal and T wave inversion may evolve with time as part of a disease progression 6,7 .…”