Arrhythmogenic right ventricular dysplasia: clinical results with implantable cardioverter defibrillators

Arrhythmogenic right ventricular dysplasia is a clinical entity characterized by fatty infiltration of the right ventricle and left bundle morphology ventricular tachycardia occurring in young patients. The most common cause of death is tachyarrhythmic. Pharmacological and nonpharmacological therapies, including implantable cardioverter defibrillators, have been used to treat the arrhythmias. However, right ventricular endocardial leads in this population may be associated with an increased risk of perforation and suboptimal sensing and defibrillation efficacy due to the diseased right ventricle. We report on 12 patients with arrhythmogenic right ventricular dysplasia who were treated with implantable cardioverter defibrillators. The mean age was 31 +/- 9 years (range 15-48). Patients presented with presyncope (5), syncope (4), or cardiac arrest (3). All patients had electrocardiographic abnormalities characteristic of the condition. During programmed electrical stimulation nine patients had sustained ventricular tachycardia, while three patients had no inducible arrhythmia. Transvenous leads were placed in nine patients. In these patients pacing thresholds were significantly higher, R-wave amplitudes were significantly lower and defibrillation thresholds were not significantly different than in a cohort of patients without right ventricular dysplasia. There were no acute or chronic complications of right ventricular lead placement. Follow-up averaged 22 +/- 13 months (range 1-45). There was one sudden death at 1 month of follow-up. Of the 12 patients, 8 have had appropriate therapy delivered by the implantable defibrillator. Six patients are currently on sotalol to reduce the frequency of implantable defibrillator discharges. In conclusion, implantable cardioverter defibrillators with nonthoracotomy leads are feasible and safe in patients with arrhythmogenic right ventricular dysplasia. The frequency of appropriate therapy is high, supporting the use of implantable cardioverter defibrillators in this population.