The Electrocardiographic Manifestations of Arrhythmogenic Right Ventricular Dysplasia

Abstract

The ECG is abnormal in most patients with arrhythmogenic right ventricular dysplasia (ARVD). Right ventricular parietal block, reduced QRS amplitude, epsilon wave, T wave inversion in V1-3 and ventricular tachycardia in the morphology of left bundle branch block are the characteristic changes that reflect the underlying genetic predetermined pathology and pathoelectrophysiology. Recognizing the characteristic ECG changes in ARVD will be of help in making a correct diagnosis of this rare disease.

Is the Phenotype–Genotype Relationship Necessary to Understand Cardiomyopathies?

“…The ECG phenotype of ARVD is characteristic 19 in the majority patients meeting the revised task force criteria. 20 Among PLN R14del mutation carriers, no epsilon waves have been reported.…”

“…The most striking ECG change is the reduced QRS amplitude especially in those with LV dilatations. 9,12,21 Reduced QRS amplitude is common in patients with heart failure of all causes, ARVD, 19 presence of peripheral edema, and in other conditions. 22 Patients with all types of cardiomyopathies, including congenital and acquired forms, are prone to ventricular arrhythmias and sudden cardiac death.…”

Electrocardiographic criteria in founder mutations related to Arrhythmogenic cardiomyopathy

“…Typical ECG manifestations in TMEM43 mutations are poor R-wave progression in right precordial leads (Figure 1), often associated with T-wave inversions in lateral leads [4]. Typical ECG manifestations in phospholamban mutations are low voltage ECG [5], more than complete right bundle branch block [6], epsilon waves in right precordial leads and in lead aVR [7] and right precordial T-wave inversions ( Figure 2). Atrial ibrillation occurs frequently.…”

Electrocardiographic characteristics of arrhythmogenic right ventricular dysplasia, cardiac sarcoidosis and arrhythmogenic biventricular cardiomyopathy

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Complete right bundle branch block with localised right precordial QRS prolongation has been published under the term "more than complete right bundle branch block" by Guy Fontaine and coworkers [1]. This ECG criterion defines arrhythmogenic right ventricular dysplasia (cardiomyopathy), cardiac sarcoidosis or arrhythmogenic biventricular cardiomyopathy.

In 391 patients with typical arrhythmogenic cardiomyopathy (214 males, mean age 46.1 ± 11.3 years) 24 patients had the ECG finding of more than complete right bundle branch block.

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“…Complete right bundle branch block with localised right precordial QRS prolongation has been published under the term "more than complete right bundle branch block" by Guy Fontaine and coworkers [1]. This ECG criterion defines arrhythmogenic right ventricular dysplasia (cardiomyopathy), cardiac sarcoidosis or arrhythmogenic biventricular cardiomyopathy.…”