Value of the signal-averaged electrocardiogram in arrhythmogenic right ventricular cardiomyopathy/dysplasia

doi:10.1016/j.hrthm.2010.10.007

Heart Rhythm

Volume 8, Issue 2, February 2011, Pages 256-262

https://doi.org/10.1016/j.hrthm.2010.10.007 Get rights and content

Background

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited disease that causes structural and functional abnormalities of the right ventricle (RV). The presence of late potentials as assessed by the signal-averaged electrocardiogram (SAECG) is a minor task force criterion.

Objective

The purpose of this study was to examine the diagnostic and clinical value of the SAECG in a large population of genotyped ARVC/D probands.

Methods

We compared the SAECGs of 87 ARVC/D probands (age 37 ± 13 years, 47 males) diagnosed as affected or borderline by task force criteria without using the SAECG criterion with 103 control subjects. The association of SAECG abnormalities was also correlated with clinical presentation, surface ECG, ventricular tachycardia (VT) inducibility at electrophysiologic testing, implantable cardioverter-defibrillator therapy for VT, and RV abnormalities as assessed by cardiac magnetic resonance imaging (cMRI).

Results

Compared with controls, all three components of the SAECG were highly associated with the diagnosis of ARVC/D (P <.001). They include the filtered QRS duration (97.8 ± 8.7 ms vs 119.6 ± 23.8 ms), low-amplitude signal (24.4 ± 9.2 ms vs 46.2 ± 23.7 ms), and root mean square amplitude of the last 40 ms of the QRS (50.4 ± 26.9 μV vs 27.9 ± 36.3 μV). The sensitivity of using SAECG for diagnosis of ARVC/D was increased from 47% using the established 2 of 3 criteria (i.e., late potentials) to 69% by using a modified criterion of any 1 of 3 criteria, while maintaining a high specificity of 95%. Abnormal SAECG as defined by this modified criterion was associated with a dilated RV volume and decreased RV ejection fraction detected by cMRI (P <.05). SAECG abnormalities did not vary with clinical presentation or reliably predict spontaneous or inducible VT and had limited correlation with ECG findings.

Conclusion

Using 1 of 3 SAECG criteria contributed to increased sensitivity and specificity for the diagnosis of ARVC/D. This finding is incorporated in the recent modification of the task force criteria.

Introduction

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited myocardial disease characterized pathologically by fibrofatty replacement that causes structural and functional abnormalities of the right ventricle (RV). Clinical manifestations of the disease include ventricular arrhythmias, congestive heart failure, and sudden death. The diagnosis of ARVC/D is based on task force criteria established in 1994 that are divided into major and minor components.¹ Localized conduction delay by a variety of ECG measurements is seen in a large number of ARVC/D patients.2, 3, 4 The high-resolution signal-averaged electrocardiogram (SAECG) is particularly well suited to detect localized slowed conduction and has been viewed as an adjunct for the diagnosis of ARVC/D. Currently, the presence of late potentials on SAECG is a minor task force criterion; however, data corroborating its diagnostic value are limited.

The purpose of this study was to reexamine the value of SAECG in a large population of genotyped ARVC/D probands enrolled in the Multidisciplinary Study of ARVC/D.5, 6 The specific aims of the present study were as follows: (1) to test the contribution of SAECG to the diagnosis of ARVC/D, (2) to determine the optimal cutoff values for the SAECG for the diagnosis of ARVC/D, and (3) to compare the diagnostic sensitivity and specificity of one, two, and three standard individual SAECG components. Additional substudies evaluated the association of the abnormalities of SAECG using the optimal criteria with (1) clinical presentation of ARVC/D, (2) abnormalities on the surface ECG, (3) ventricular tachycardia (VT) inducibility at electrophysiologic testing, (4) implantable cardioverter-defibrillator (ICD) therapy for sustained VT, and (5) volume/morphologic features as defined by cardiac magnetic resonance imaging (cMRI).

Section snippets

Clinical characteristics of study subjects

The study included 87 ARVC/D probands (age 37 ± 13 years, 47 males) diagnosed as affected (n = 62) or borderline (n = 25) by task force criteria¹ without using the SAECG criterion (Table 1). Probands were excluded if that test was crucial for the diagnosis of the individual patient. This was done to eliminate bias in estimating the sensitivity and specificity of that particular test. In general, when determining the sensitivity and specificity of a new screening test, it is recommended that

SAECG findings

Table 2 compares the individual SAECG parameters of 87 ARVC/D patients and 103 matched controls. All three SAECG parameters (fQRSD, LAS, and RMS-40) were significantly more abnormal in ARVC/D probands than in controls. The difference for each parameter was highly significant (P <.001). The SAECG parameters fQRSD (124.2 ± 27.4 ms vs 118.0 ± 22.7 ms, P = .28), LAS (52.4 ± 26.9 ms vs 44.5 ± 22.4 ms, P = .16), and RMS-40 (19.3 ± 15.4 μV vs 27.4 ± 30.1 μV, P = .18) were not significantly different

Discussion

This study is the largest to date to examine the diagnostic value of SAECG in ARVC/D and the association of clinical parameters with abnormal SAECG. In the present study, the SAECG and its components fQRSD, LAS, and RMS-40 were highly associated with the diagnosis of ARVC/D. The sensitivity of using SAECG for diagnosis of ARVC/D was increased from 47% using 2 of the 3 criteria to 69% by using any 1 of the 3 criteria while maintaining high specificity of 90% to 95%. Abnormal SAECG as defined by

Conclusion

The SAECG was shown to contribute to the diagnosis of ARVC/D in a well-characterized population of ARVC/D patients with diagnosis within 3 years of onset. Using any 1 of the 3 SAECG criteria provided optimal sensitivity and specificity. This finding is incorporated in the current recent modification of the task force criteria.42, 43 The evidence that abnormal SAECG reflects functional abnormalities of the RV and RV enlargement suggests that the SAECG correlates with disease severity; however,

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is currently diagnosed using a combination of clinical features, imaging, electrocardiography, and genetic investigations. An abnormal signal-averaged electrocardiogram (SAECG) is defined as a minor diagnostic criterion by the 2010 Task Force Criteria, but doubts remain about the value of this investigation.
We evaluated the utility of the SAECG in diagnosing ARVC using the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry, a population representative registry of probands with ARVC and relatives, less influenced by referral bias.
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Arrhythmogenic right ventricular cardiomyopathy (ARVC) encompasses a group of conditions characterized by right ventricular fibrofatty infiltration, with a predominant arrhythmic presentation. First described in the late 1970s and early 1980s, it is now frequently recognized to have biventricular involvement. The prevalence is ∼1:2,000 to 1:5,000, depending on geographic location, and it has a slight male predominance. The diagnosis of ARVC is determined on the basis of fulfillment of task force criteria incorporating electrophysiological parameters, cardiac imaging findings, genetic factors, and histopathologic features. Risk stratification of patients with ARVC aims to identify those who are at increased risk of sudden cardiac death or sustained ventricular tachycardia. Factors including age, sex, electrophysiological features, and cardiac imaging investigations all contribute to risk stratification. The current management of ARVC includes exercise restriction, β-blocker therapy, consideration for implantable cardioverter-defibrillator insertion, and catheter ablation. This review summarizes our current understanding of ARVC and provides clinicians with a practical approach to diagnosis and management.
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: Funded by National Heart, Lung, and Blood Institute (NHLBI) Grants U01 HL65594, HL65652, and HL65691. Dr. Steinberg is the Al-Sabah Endowed Director of the Arrhythmia Institute at St. Luke's and Roosevelt Hospitals.

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ClinicalGeneralValue of the signal-averaged electrocardiogram in arrhythmogenic right ventricular cardiomyopathy/dysplasia

Background

Objective

Methods

Results

Conclusion

Introduction

Section snippets

Clinical characteristics of study subjects

SAECG findings

Discussion

Conclusion

Heart Rhythm

Am J Cardiol

Am J Cardiol

J Electrocardiol

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Int J Cardiol

Am J Cardiol

J Am Coll Cardiol

Heart Rhythm

Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathyTask Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology

Br Heart J

Dispersion of ventricular depolarization-repolarization: a noninvasive marker for risk stratification in arrhythmogenic right ventricular cardiomyopathy

Circulation

Diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy: value of standard ECG revisited

Ann Noninvasive Electrocardiol

Electrocardiographic features of arrhythmogenic right ventricular dysplasia/cardiomyopathy according to disease severity: a need to broaden diagnostic criteria

Circulation

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) a multidisciplinary study: design and protocol

Circulation

Signal-averaged electrocardiography

J Am Coll Cardiol

Evaluation of normal values for signal averaged electrocardiogram

J Cardiovasc Electrophysiol

ECG Manifestation of ARVC/D

Epsilon wave in arrhythmogenic right ventricular dysplasia/cardiomyopathy

Pacing Clin Electrophysiol

Magnetic resonance and computed tomography imaging of arrhythmogenic right ventricular dysplasia

J Magn Reson Imaging

Clinical
General
Value of the signal-averaged electrocardiogram in arrhythmogenic right ventricular cardiomyopathy/dysplasia