| Summary: | Abstract Background Endomyocardial fibrosis (EMF) is a form of restrictive cardiomyopathy that is diagnosed mainly in children and young adults and is geographically found in Africa, Latin America, and Asia. It is a condition with high morbidity and mortality, unknown etiology, and no definitive treatment. Although its main clinical presentation is congestive heart failure with or without related supraventricular arrhythmia like atrial fibrillation, it very rarely presents with ventricular arrhythmias and tachycardias (VA, VT). Case presentation We report a case of right ventricular (RV) EMF presented with recurrent attacks of hemodynamically unstable VT that required direct current (DC) cardioversion. The diagnosis was suspected by transthoracic echocardiography (TTE) and established by cardiac magnetic resonance (CMR). The patient underwent implantable cardioverter–defibrillator (ICD) implantation for secondary prevention of VT, and he was discharged safely on antiarrhythmic drugs with regular follow-up visits. Conclusion EMF presenting with VT are quite rare and to the best of our knowledge, our case is the fourth case in the literature to report VT as a clinical presentation of EMF.
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