Related Experiment Video
Updated: May 10, 2026
Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
Published on: December 22, 2023
Malignant arrhythmogenic right ventricular dysplasia/cardiomyopathy with a normal 12-lead electrocardiogram: a rare
Anneline S J M te Riele1, Cynthia A James, Aditya Bhonsale
1Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Insights
A normal electrocardiogram (ECG) does not rule out Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) in patients with ventricular arrhythmias. Comprehensive clinical evaluation is crucial for accurate diagnosis and risk assessment in ARVD/C.
Area of Science:
- Cardiology
- Electrophysiology
- Genetics
Background:
- Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is a genetic heart condition.
- A normal electrocardiogram (ECG) is typically reassuring in ARVD/C patients.
- However, some ARVD/C patients with ventricular arrhythmias present with a normal ECG.
Purpose of the Study:
- To determine the frequency of normal ECGs in ARVD/C patients experiencing ventricular arrhythmias.
- To characterize the clinical profile of ARVD/C patients with normal ECGs and arrhythmias.
Main Methods:
- Reviewed ECGs of 145 ARVD/C patients with documented sustained ventricular arrhythmias.
- Classified ECGs as normal, nonspecific, or abnormal based on Task Force Criteria (TFC).
- Assessed cardiologic investigations within 6 months of the arrhythmic event for TFC.
Main Results:
- 17 of 145 (12%) patients had nonspecific or normal ECGs.
- Patients with normal/nonspecific ECGs were similar in age and sex distribution to those with abnormal ECGs.
- Most patients with normal/nonspecific ECGs showed TFC on Holter monitoring, signal-averaged ECG, or structural evaluation.
Conclusions:
- A normal or nonspecific ECG does not exclude ARVD/C diagnosis in patients with ventricular arrhythmias.
- Alternative evidence of disease expression was present in all patients with normal/nonspecific ECGs.
- Physicians should not solely rely on ECG; comprehensive clinical evaluation is essential for assessing arrhythmic risk in ARVD/C.
Background:
In Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), a normal electrocardiogram (ECG) is considered reassuring. However, some patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG.
Objectives:
To estimate how often patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG during sinus rhythm, and to provide a clinical profile of these patients.
Methods:
We included 145 patients with ARVD/C experiencing a documented sustained ventricular arrhythmia. Conventional 12-lead sinus rhythm ECGs within 6 months of the event were reviewed for diagnostic Task Force Criteria (TFC). ECGs were classified as abnormal (≥1 TFC), nonspecific (abnormal, no TFC), or normal. Cardiologic investigations within 6 months of the event were evaluated as per TFC in those with a nonspecific or normal ECG.
Results:
The ECG was nonspecific or normal in 17 of 145 (12%) subjects. Mean age of these patients was 41.3 ± 12.4 years and 14 (82%) were men, comparable to those with an abnormal ECG. Most patients with a nonspecific or normal ECG showed ≥1 TFC on Holter monitoring (n = 9 of 10) and signal-averaged ECG (n = 4 of 5), and all had nonsustained ventricular tachycardia recorded. Among 15 patients who underwent structural evaluation, 11 (73%) showed structural TFC (9 major and 2 minor).
Conclusions:
Although most patients with ARVD/C experiencing arrhythmias have an abnormal ECG, a nonspecific or normal ECG does not preclude ARVD/C diagnosis. All patients with a nonspecific or normal ECG had alternative evidence of disease expression. These results alert the physician not to rely exclusively on ECG in ARVD/C, but to assess arrhythmic risk by comprehensive clinical evaluation.
Related Concept Videos
01:25Cardiomyopathy I: Introduction and Classification
01:29Cardiomyopathy IV: Restrictive Cardiomyopathy
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
01:30Cardiomyopathy II: Dilated Cardiomyopathy
01:29Dysrhythmias III: Characteristics of Dysrhythmias
01:30Dysrhythmias V: Evaluating Dysrhythmias