Diagnosis of Arrhythmogenic Right Ventricular Dysplasia‐Cardiomyopathy: Value of Standard ECG Revisited

Abstract

With regard to sensitivity and already known specificity an ECG score for the diagnosis of ARVD/C was developed with high probability of ARVD/C in cases with > or =4 points, possibly without the need for an additional imaging technique. Standard ECG with additional highly amplified and modified recording technique represents a single diagnostic test with high value in the clinical diagnosis of ARVD/C and should be used as a first line tool in noninvasive family screening.

Electrocardiographic Features of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy According to Disease Severity

“…3 This is a highly specific but insensitive criterion for ARVD/C and is observed in 25% to 33% of ARVD/C patients when evaluated by the standard ECG. 4,6 In addition to the epsilon wave, several other markers of delayed activation of the RV have been described, such as QRSd Ն110 ms in V 1 through V 3 , 3,6 the ratio of the sum of the QRSd in leads V 1 ϩV 2 ϩV 3 /V 4 ϩV 5 ϩV 6 Ն1.2, and parietal block. 5,6 T-wave inversion in leads V 1 through V 3 in the absence of RBBB is considered a minor diagnostic criterion for ARVD/C, 5 and its prevalence in ARVD/C has been reported as 55% to 94% in different series.…”

“…3 These criteria included (1) T-wave inversions in V 1 through V 3 , (2) QRS duration (QRSd) Ն110 ms in V 1 through V 3 , and (3) the presence of an epsilon wave (electric potentials after the end of the QRS complex). Additional ECG markers of ARVD/C that have been reported include (1) QRS and QT dispersion, 4 (2) parietal block, defined as a QRSd in leads V 1 through V 3 that exceeds the QRSd in lead V 6 by Ͼ25 ms, 5 and (3) a ratio of the QRSd in leads V 1 ϩV 2 ϩV 3 /V 4 ϩV 5 ϩV 6 Ն1.2. 6 The purpose of the present study was to reexamine the ECG features of ARVD/C.…”

“…Additional ECG markers of ARVD/C that have been reported include (1) QRS and QT dispersion, 4 (2) parietal block, defined as a QRSd in leads V 1 through V 3 that exceeds the QRSd in lead V 6 by Ͼ25 ms, 5 and (3) a ratio of the QRSd in leads V 1 ϩV 2 ϩV 3 /V 4 ϩV 5 ϩV 6 Ն1.2. 6 The purpose of the present study was to reexamine the ECG features of ARVD/C. Particular attention is focused on determining the relation of ECG parameters to disease severity; describing a novel ECG marker of ARVD/C; and identifying those ECG features that best distinguish ARVD/C from RV outflow tract tachycardia (RVOT), which is the most common differential diagnosis in the clinical setting, and assessing the association of ECG markers with clinical presentation and outcome of the electrophysiology study (EPS) in patients with ARVD/C.…”

Lamr1 functional retroposon causes right ventricular dysplasia in mice

“…The difficulty in generating lethal tachyarrhythmia in mice, due to the high beating rate and small size of their hearts 19 , might explain our failure to detect lethal arrhythmia. KK/Rvd mice showed electric conduction inhomogeneity leading to prolonged QRS duration, which is often seen in humans with ARVD 20,21 . If similar degradation occurs in the human heart, it will become a focus for lethal arrhythmia at some stage in life.…”

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare “disease of the desmosome” with multiple clinical presentations

“…The prevalence is estimated to range from 6/10,000 in the general population to 44/10,000 in some areas in Northern Italy 3 , 4 and Germany 5 . It is inherited in approximately 50%.…”