Background: Left dominant arrhythmogenic cardiomyopathy (LDAC) is a rare condition characterised by progressive\nfibrofatty replacement of the myocardium of the left ventricle (LV) in combination with ventricular arrhythmias of LV\norigin.\nCase presentation: A thirty-five-year-old male was referred for evaluation of recurrent sustained monomorphic\nventricular tachycardia (VT) of 200 bpm and right bundle branch block (RBBB) morphology. Cardiac magnetic resonance\nimaging showed late gadolinium enhancement distributed circumferentially in the epicardial layer of the LV free wall\nmyocardium including the rightward portion of the interventricular septum (IVS). The clinical RBBB VT was reproduced\nduring the EP study. Ablation at an LV septum site with absence of abnormal electrograms and a suboptimum pacemap\nrendered the VT of clinical morphology noninducible. Three other VTs, all of left bundle branch block (LBBB) pattern, were\ninduced by programmed electrical stimulation. The regions corresponding to abnormal electrograms were identified and\nablated at the mid-to-apical RV septum and the anteroseptal portion of the right ventricular outflow tract. No abnormalities\nwere found at the RV free wall including the inferolateral peritricuspid annulus region. Histological examination confirmed\nthe presence of abnormal fibrous and adipose tissue with myocyte reduction in endomyocardial samples taken from both\nthe left and right aspects of the IVS.\nConclusion: LDAC rarely manifests with sustained monomorphic ventricular tachycardia. In this case, several VTs of both\nRBBB and LBBB morphology were amenable to endocardial radiofrequency catheter ablation.
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