Arrhythmogenic Right Ventricular Cardiomyopathy

Area of Science:

• Cardiology
• Genetics
• Pathology

Background:

• Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disease.
• It is characterized by fibrofatty replacement of the right ventricular myocardium.
• ARVC presents with ventricular arrhythmias and can lead to sudden cardiac death.

Observation:

• A young male patient presented with symptoms suggestive of ARVC.
• Electrocardiogram (EKG) revealed early transition inverted T waves in leads V1-V4.
• Cardiac Magnetic Resonance Imaging (MRI) demonstrated right ventricle (RV) dyskinesia and reduced RV ejection fraction (EF) < 40%.

Findings:

• The patient met two major diagnostic criteria for ARVC.
• EKG findings satisfied major criteria for ARVC diagnosis.
• MRI findings also satisfied major criteria for ARVC diagnosis, confirming the condition.

Implications:

• This case underscores the importance of combined EKG and MRI in diagnosing ARVC.
• Accurate diagnosis is crucial for risk stratification and management of patients with ARVC.
• Early identification and management can potentially prevent adverse cardiac events in individuals with ARVC.