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A case of arrhythmogenic right ventricular cardiomyopathy with biventricular involvement

Filippo Brandimarte1, Alessandro Battagliese, Silvana Petronilla Pirillo

  • 1San Giovanni Addolorata Community Hospital, Cardiology and Rehabilitation Unit, Department of Medicine, Rome. brandimarte.filippo@gmail.com.

Monaldi Archives for Chest Disease = Archivio Monaldi Per Le Malattie Del Torace
|April 11, 2019
PubMed

Insights

A young adult experienced syncope, revealing advanced arrhythmogenic right ventricular cardiomyopathy. This case highlights the condition

Area of Science:

  • Cardiology
  • Genetics
  • Electrophysiology

Background:

  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart muscle disease.
  • It primarily affects the right ventricle, leading to fibro-fatty replacement of the myocardium.
  • ARVC can cause life-threatening ventricular arrhythmias and sudden cardiac death.

Observation:

  • An 18-year-old male presented with syncope during athletic activity.
  • Electrocardiogram (ECG) revealed T-wave inversion and incomplete right bundle branch block.
  • Echocardiography showed severe right ventricular enlargement, aneurysm, and reduced systolic function.

Findings:

  • Cardiac Magnetic Resonance imaging confirmed fibro-fatty replacement in both ventricles, characteristic of ARVC.
  • An epsilon wave, a hallmark ECG finding, appeared after defibrillator testing.
  • This presentation represented the initial clinical manifestation of advanced ARVC.

Implications:

  • Early diagnosis of ARVC is crucial for preventing sudden cardiac death in young athletes.
  • Advanced imaging techniques like Cardiac MRI are vital for accurate ARVC diagnosis.
  • Subcutaneous defibrillator implantation provides effective arrhythmia management in ARVC patients.

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