Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart condition characterized with fibrofatty replacement of myocardium with an increased risk of arrhythmia and sudden cardiac death. Multimodality imaging including cardiac magnetic resonance (CMR) can help deep phenotype cases. Clinical case where voltage mapping identified fibrofatty myocardium undetected by CMR. N/A A 46-year-old female presented with palpitations, ventricular tachycardia (VT) and premature ventricular contractions (PVCs) burden of 17%. The intrinsic sinus ECG was normal She preferred ablation and pre-procedure CMR showed dilated right ventricle without evidence of dyskinesia, inflammation, late gadolinium enhancement, or fatty infiltration. The patient underwent an EP study arriving in sinus rhythm and had dominant clinical PVC with a left bundle inferiorly directed axis, V5 transition and negative in lead I and aVL placing it at the anterior-septal right ventricular outflow tract. Having good pace maps there burst pacing with the ablation catheter induced clinical VT similar to the PVC; radiofrequency ablation slowed and terminated it under 10 seconds. After suppression of VT and PVCs, a detailed voltage map of the RV was obtained demonstrating significant bipolar and unipolar voltage abnormalities on the RV free wall where ablation had not been performed. This scar pattern was suggestive of ARVC Subsequent genetic testing confirmed pathogenic PKP2 mutation. Voltage mapping remains a useful adjunct tool for deep phenotyping and can detect diseased myocardium which may not be visualized with other imaging modalities.

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