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Patient
45-year-old man with right ventricular arrhythmogenic dysplasia; treatment by beta-blocker; 2 ablation procedures for a ventricular tachycardia originating from the pulmonary infundibulum; implantation of a dual-chamber defibrillator given the recurrence of tachycardias;
Trace
Pacing of the patient's atrium by his defibrillator with wide and fragmented atrial activity; the ventricles are spontaneous; the axis is extreme-right with low voltage QRS in the precordium and a right delay pattern; possible epsilon wave at the end of QRS; negative T waves throughout the precordium and inferior leads;
Trace
Relatively slow ventricular tachycardia (130 bpm) with left delay; QRS transition (R/S>1) in V5 (late); atrioventricular dissociation confirming the diagnosis of ventricular tachycardia (atrial activity clearly identifiable in V1); this tracing corresponds to a right ventricular outflow tract (pulmonary infundibulum) tachycardia;
Trace
This tracing shows an anti-tachycardia pacing sequence delivered by the implantable defibrillator which allows the interruption of the tachycardia and the return to sinus rhythm;
Exergue
The sinus rhythm electrocardiogram of a patient with arrhythmogenic dysplasia may reveal an epsilon wave (specific but not very sensitive sign), a reversal of T waves in right precordial leads, a complete or incomplete right bundle branch block, ventricular extrasystoles with left monomorphic or polymorphic delay.
arrhythmogenic right ventricular dysplasia is an inherited disease characterized by the gradual replacement of the cardiac muscle cells by fibro-adipose tissue. Transmission is autosomal dominant with variable penetrance and expression by mutation of the genes encoding for desmosome proteins.