SHM Converge Scientific Abstract ePoster Gallery-Diagnostic Odyssey: From Arvc to Avnrt

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A 39-year-old man was evaluated because of a family history of arrhythmogenic right ventricular cardiomyopathy (ARVC). Genetic testing was positive for a plakophilin-2 mutation, and initial ECG findings suggested ARVC, including sinus bradycardia, possible epsilon wave in V1, and right-axis deviation. Although transthoracic echocardiography was normal, cardiac MRI showed right ventricular free wall thinning and abnormal enhancement interpreted as fatty infiltration, further supporting ARVC.<br /><br />Additional rhythm monitoring showed intermittent conduction changes, with rhythms varying from narrow to wide complex and changing PR intervals, consistent with intermittent right bundle branch block. Mobile cardiac telemetry captured a wide-complex tachycardia at 150 bpm without visible P waves, raising concern for monomorphic ventricular tachycardia in the setting of suspected ARVC, though supraventricular tachycardia with aberrancy was also possible.<br /><br />An electrophysiology study ultimately identified atrioventricular node reentrant tachycardia (AVNRT) with intermittent RBBB. A second opinion questioned the MRI findings and concluded they were not consistent with ARVC. The patient was therefore diagnosed with AVNRT with aberrant conduction rather than clinically manifest ARVC. He underwent catheter ablation and did not require a defibrillator at that time.<br /><br />This case highlights the diagnostic challenge of distinguishing ARVC from AVNRT, especially when a patient is gene-positive but phenotypically negative for ARVC. Although both conditions may be treated with beta blockers and ablation, their prognoses differ substantially: ARVC carries risk of sudden cardiac death and may warrant implantable cardioverter-defibrillator placement, whereas AVNRT is typically curable with ablation. Accurate diagnosis is essential to guide therapy and avoid unnecessary or missed interventions.

Asset Subtitle

Jesse E. Doyle

Meta Tag

Author List Douglas Sheffer, Jesse E. Doyle, Kengo Z. Soghoyan, Muhammad Ishaq, Robert Ficalora, Umang Patel

Category Clinical Vignettes

Concept Arrhythmogenic Right Ventricular Cardiomyopathy

Concept Wide-Complex Tachycardia

Concept Genetic Basis

Concept Supraventricular Tachycardia

Concept Atrioventricular Nodal Reentrant Tachycardia

Distinguished Non-Finalist

Presenter Organization Indiana University

Presenting Author Jesse E. Doyle

Track Adult

Keywords

arrhythmogenic right ventricular cardiomyopathy

ARVC

plakophilin-2 mutation

AVNRT

right bundle branch block

ventricular tachycardia

cardiac MRI

epsilon wave

catheter ablation

sudden cardiac death

Arrhythmogenic Right Ventricular Cardiomyopathy

Wide-Complex Tachycardia

Genetic Basis

Supraventricular Tachycardia

Atrioventricular Nodal Reentrant Tachycardia