Ablation of Ventricular Tachycardia Originating from the Right Ventricle Associated with Scleroderma Cardiomyopathy

A 49-year-old male was referred to the hospital because of syncope caused by ventricular tachycardia (VT) with a pulse of 240 bpm and QRS morphology with a LBBB configuration and superior axis. The patient had been on a long-term regimen of steroids to treat his scleroderma. Satisfying 2 major criteria (QRS widening and an epsilon wave in the right chest leads) and 3 minor criteria (a slight enlargement and akinesis of the right ventricle, positive late potential in the signal averaged electrocardiogram and left bundle branch block-type VT) he was diagnosed with arrhythmogenic right ventricular cardiomyopathy. A voltage map of his right ventricle (RV) during sinus rhythm was obtained using an electroanatomical mapping system (CARTO, Biosense-Webster, Diamond, CA, USA). Two islet-like low voltage areas were found and linear double potentials were recognized between areas in the lateral wall of the right ventricle (RV) very close to the tricuspid annulus. The earliest activation of the double potential line during VT was 70 msec prior to the QRS onset. We applied radiofrequency energy at that point during the VT and it successfully slowed and terminated the VT. Thereafter the VT could not be induced by any stimulation from multiple RV sites.