implantable cardioverter defibrillator
arrhythmogenic right ventricular dysplasia
Springer Online Journal Archives 1860-2000
Abstract Arrhythmogenic right ventricular dysplasia is a clinical entitycharacterized by fatty infiltration of the right ventricle and left bundlemorphology ventricular tachycardia occurring in young patients. The mostcommon cause of death is tachyarrhythmic. Pharmacological andnonpharmacological therapies, including implantable cardioverterdefibrillators, have been used to treat the arrhythmias. However, rightventricular endocardial leads in this population may be associated with anincreased risk of perforation and suboptimal sensing and defibrillationefficacy due to the diseased right ventricle. We report on 12 patients witharrhythmogenic right ventricular dysplasia who were treated with implantablecardioverter defibrillators. The mean age was 31± 9 years (range15-48). Patients presented with presyncope (5), syncope (4), or cardiacarrest (3). All patients had electrocardiographic abnormalitiescharacteristic of the condition. Follow-up averaged 22 ± 13months (range 1-45). There was one sudden death at 1 month of follow-up. Ofthe 12 patients, 8 have had appropriate therapy delivered by the implantabledefibrillator. Six patients are currently on sotalol to reduce the frequencyof implantable defibrillator discharges. In conclusion, implantablecardioverter defibrillators with nonthoracotomy leads are feasible and safein patients with arrhythmogenic right ventricular dysplasia. The frequencyof appropriate therapy is high, supporting the use of implantablecardioverter defibrillators in this population. During programmedelectrical stimulation nine patients had sustained ventricular tachycardia,while three patients had no inducible arrhythmia. Transvenous leads wereplaced in nine patients. In these patients pacing thresholds weresignificantly higher, R-wave amplitudes were significantly lower, anddefibrillation thresholds were not significantly different than in a cohortof patients without right ventricular dysplasia. There were no acute orchronic complications of right ventricular lead placement.
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