What are the electrocardiogram (ECG) signs of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

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ECG Signs of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

The ECG is abnormal in over 60% of ARVC patients and demonstrates characteristic depolarization and repolarization abnormalities that predominantly affect the right precordial leads. 1

Primary Depolarization Abnormalities

T-Wave Inversion (Most Common Finding)

  • T-wave inversion in right precordial leads V1-V3 is the most frequent ECG abnormality in ARVC, occurring in approximately 29% of patients at baseline 1, 2
  • When left ventricular involvement is present, T-wave inversion extends to lateral leads (V4-V6, I, aVL) 1
  • T-wave inversion in inferior leads (II, III, aVF) reflects right ventricular infero-posterior wall involvement 1
  • This finding is considered an abnormal ECG pattern requiring further cardiac evaluation to exclude ARVC 1

QRS Complex Abnormalities

  • Prolonged QRS duration >110 ms in right precordial leads (V1-V3), often with right bundle branch block pattern 1
  • Localized QRS prolongation specifically in V1-V3 (>110 ms) is a diagnostic marker 1, 3
  • Delayed S-wave upstroke >55 ms in leads V1-V2 is a specific finding 1
  • Poor R-wave progression in right precordial leads is commonly observed 3
  • Low QRS voltages in precordial leads occur significantly more frequently than in healthy subjects 3

Epsilon Wave

  • A terminal notch in the QRS complex appearing as a low-amplitude signal between the end of QRS and onset of T-wave in leads V1-V3 1
  • This is characteristic but relatively infrequent, seen in only 16% of patients at baseline 1, 2
  • Must be distinguished from benign QRS notching patterns and technical artifacts 4

Repolarization Abnormalities

  • ST-segment elevation may be present in V1-V3 3
  • Repolarization changes reflect myocardial areas of disease involvement 1

Left Ventricular Variant Patterns

When ARVC predominantly affects the left ventricle:

  • Low voltages of R/S wave in limb leads 1
  • Diffuse T-wave abnormalities extending beyond right precordial leads 1
  • Inferior and/or lateral T-wave inversion with ventricular arrhythmias suggesting left ventricular origin 1

Signal-Averaged ECG Findings

  • Late potentials are observed in >50% of ARVC patients 1
  • Terminal activation delay (TAD) of QRS ≥55 ms in right precordial leads occurs in 34% of patients 2
  • While not highly specific, signal-averaged ECG provides a marker of slow conduction and arrhythmic risk 1

Arrhythmic Manifestations

  • Ventricular arrhythmias with left bundle branch block morphology and superior axis are typical of ARVC 1
  • Premature ventricular contractions, ventricular tachycardia, and ventricular fibrillation may occur 1
  • Left bundle branch block morphology with inferior axis requires differentiation from benign right ventricular outflow tract tachycardia 1

Dynamic ECG Changes

Serial ECGs are critical because 23-39% of ARVC patients demonstrate dynamic or new ECG abnormalities during follow-up 2, 5:

  • New or dynamic T-wave inversion develops in approximately 10-13% of patients 2, 5
  • Epsilon waves can appear, disappear, or fluctuate dynamically 2, 5
  • These changes may occur before major structural abnormalities are detectable on imaging 5

Critical Clinical Considerations

Normal ECG Does Not Exclude ARVC

  • Approximately 10% of ARVC patients present with a completely normal ECG 6
  • A diagnosis based solely on imaging criteria with a completely normal ECG should be considered suspicious and warrants careful review 1
  • Normal ECGs are mostly associated with mild or early forms of disease 3
  • ECG changes are often the first disease manifestation, preceding structural abnormalities 1

Differential Diagnosis Pitfalls

  • Distinguish epsilon waves from benign terminal QRS notching seen in early repolarization patterns 4
  • Differentiate from right ventricular outflow tract tachycardia, which lacks the ECG abnormalities of ARVC and is more common in women 1
  • Consider technical factors such as precordial lead misplacement that can affect R-wave progression 7
  • Rule out other conditions mimicking ARVC including sarcoidosis, myocarditis, and athletic cardiac adaptation 8

Extent of ECG Abnormalities Correlates with Disease Severity

  • T-wave inversion beyond V3 characterizes severe right ventricular dilatation 3
  • The overall extent of electrocardiographic abnormalities relates to disease extent 3
  • Multiple ECG abnormalities suggest more advanced disease requiring comprehensive evaluation 1

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Guideline

Significance and Management of Notched QRS on ECG

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Research

Arrhythmogenic right ventricular dysplasia/cardiomyopathy: an electrocardiogram-based review.

Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, 2018

Guideline

Interpretation of Abnormal R-wave Progression and Early Transition on EKG

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Research

Arrhythmogenic right ventricular cardiomyopathy, clinical manifestations, and diagnosis.

Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, 2016

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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