Electrocardiographic features of arrhythmogenic right ventricular dysplasia

Abstract

Background: The purpose of this study was to reevaluate the ECG features of arrhythmogenic right ventricular dysplasia (ARVD). The second objective was to evaluate the sensitivity and specificity of the standard and newly proposed diagnostic ECG markers in the presence of a right bundle-branch block (RBBB).

Methods and results: One hundred patients with ARVD (57 men; aged 39+/-15 years) and 57 controls (21 men; aged 40+/-17 years) were included. Among the 100 patients with ARVD, a complete RBBB was present in 17 patients, and 15 patients had an incomplete RBBB. T-wave inversion through V(3) demonstrated optimal sensitivity and specificity in both ARVD patients without a complete RBBB or incomplete RBBB (71% [95% confidence interval, 58% to 81%] and 96% [95% confidence interval, 81% to 100%], respectively) and in ARVD patients with incomplete RBBB (73% [95% confidence interval, 45% to 92%] and 95% [95% confidence interval, 77% to 100%], respectively). Between ARVD patients and controls with a complete RBBB, the only 2 parameters that differed were the prevalence of T-wave inversion through V(4) (59% versus 12%, respectively; P<0.05) and an r'/s ratio in V(1) <1 (88% versus 14%, respectively; P<0.005). In ARVD patients with complete RBBB, the most sensitive and specific parameter was an r'/s ratio <1.

Conclusions: We evaluated comprehensively the diagnostic value of ECG markers for ARVD. On the basis of the findings, we propose an algorithm, with examination of QRS morphology being the first step, for ECG evaluation of ARVD patients. Definite criteria are then applied on the basis of the presence of no RBBB, incomplete RBBB, and complete RBBB to obtain the best diagnostic utility of the ECG.

Figure 1

Representative ECG obtained from an ARVD patient without IRBBB or CRBBB. This patient had an advanced form of ARVD. The arrow indicates an Epsilon wave. ECG also illustrates TWI in V1–V5, TAD ≥ 55ms in V1 and QRSd in V3 > 110 ms. This ECG also demonstrates low voltage, which in our experience is an uncommon finding in patients with ARVD and when seen is usually with advanced disease. There was no parietal block or localized right precordial QRS prolongation.

Figure 2

A, Representative ECG obtained from an ARVD patient with IRBBB. ECG illustrates TWI in V1–V5, TAD ≥ 55 ms, QRSd > 110 ms, ratio < 1.2 and PVC. B, Representative ECG of control with IRBBB illustrating TWI in V1–V2.

Figure 2

A, Representative ECG obtained from an ARVD patient with IRBBB. ECG illustrates TWI in V1–V5, TAD ≥ 55 ms, QRSd > 110 ms, ratio < 1.2 and PVC. B, Representative ECG of control with IRBBB illustrating TWI in V1–V2.

Figure 3

A, Representative ECG obtained from an ARVD patient with CRBBB. It shows TWI in V1–V4 and r’/s ratio <1. B, Representative ECG of control with CRBBB. It shows TWI in V1 and r’/s >1.

Figure 3

A, Representative ECG obtained from an ARVD patient with CRBBB. It shows TWI in V1–V4 and r’/s ratio <1. B, Representative ECG of control with CRBBB. It shows TWI in V1 and r’/s >1.

Figure 4

Flow chart summarizing an algorithm that can be used based on the presence or absence of an incomplete or complete RBBB to identify patients with ARVD. *The depolarization criteria used to screen patients with either an incomplete or no RBBB are QRS duration in V1–V3 more than 110 ms, TAD and Epsilon wave. * Repolarization criteria used in the patients with either an Incomplete or no RBBB are TWI V1–V3 and/or TWI in 2 out of the 3 inferior leads. In ARVD patients with CRBBB the depolarization criterion used is r’/s ratio < 1 in lead V1. The repolarization criteria used in ARVD patients with CRBBB are TWI through V4 and/or TWI in 2 out of the three inferior leads.