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20823389 10.1161/CIRCULATIONAHA.109.913871 This paper describes outcomes among106 ARVD/C patients who received an ICD for primary prevention. During a mean follow up of 58 ± 35 months, 24 % had an appropriate ICD discharge, and 16 % a shock for ventricular fibrillation/flutter. A history of syncope was associated with appropriate ICD interventions. None of 27 asymptomatic patients with isolated familial sudden death required appropriate ICD therapy. Twenty patients had inappropriate ICD interventions and 18 had device-related complications
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Corrado D, Calkins H, Link MS, et al. Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation. 2010;122:1144-52. This paper describes outcomes among106 ARVD/C patients who received an ICD for primary prevention. During a mean follow up of 58 ± 35 months, 24 % had an appropriate ICD discharge, and 16 % a shock for ventricular fibrillation/flutter. A history of syncope was associated with appropriate ICD interventions. None of 27 asymptomatic patients with isolated familial sudden death required appropriate ICD therapy. Twenty patients had inappropriate ICD interventions and 18 had device-related complications.
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Corrado, D.1
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36
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Incidence and predictors of implantable cardioverter-defibrillator therapy in patients with arrhythmogenic right ventricular dysplasia/ cardiomyopathy undergoing implantable cardioverter-defibrillator implantation for primary prevention
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21939834 10.1016/j.jacc.2011.06.043 This study described 84 North American ARVD/C patients who underwent ICD implantation for primary prevention over a mean follow up of 4.7 ± 3.4 years. Appropriate ICD therapy was seen in 40 (48 %) patients, of which 16 (19 %) received interventions for rapid ventricular arrhythmias. Inducibility at EP study and history of nonsustained ventricular tachycardia were significant predictors of appropriate ICD discharge in multivariable analysis. These findings are important, as they demonstrate that nearly half of the ARVD/C patients treated with an ICD for primary prevention experienced appropriate ICD interventions
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Bhonsale A, James CA, Tichnell C, et al. Incidence and predictors of implantable cardioverter-defibrillator therapy in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy undergoing implantable cardioverter-defibrillator implantation for primary prevention. J Am Coll Cardiol. 2011;58:1485-96. This study described 84 North American ARVD/C patients who underwent ICD implantation for primary prevention over a mean follow up of 4.7 ± 3.4 years. Appropriate ICD therapy was seen in 40 (48 %) patients, of which 16 (19 %) received interventions for rapid ventricular arrhythmias. Inducibility at EP study and history of nonsustained ventricular tachycardia were significant predictors of appropriate ICD discharge in multivariable analysis. These findings are important, as they demonstrate that nearly half of the ARVD/C patients treated with an ICD for primary prevention experienced appropriate ICD interventions.
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Bhonsale, A.1
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37
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22238189 10.1161/CIRCGENETICS.111.960898 This study reports psychological outcomes for 87 adult ARVD/C patients with ICDs. One-third had clinically significant anxiety and one-tenth had clinically significant depression. Young ARVD/C patients had significant body image concerns and distress related to their device. Both generalized and device-specific anxiety were elevated beyond what is the norm for ICD patients. Poor device adjustment, while unfavorable in and of itself, was additionally associated with increased likelihood of clinically significant depression and anxiety. Factors associated with poor adjustment included: 1) younger age, 2) having a device implanted recently, 3) having at least one ICD shock, and 4) having a poorer functional capacity
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James CA, Tichnell C, Murray B, et al. General and disease-specific psychosocial adjustment in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy with implantable cardioverter defibrillators: a large cohort study. Circ Cardiovasc Genet. 2012;5:18-24. This study reports psychological outcomes for 87 adult ARVD/C patients with ICDs. One-third had clinically significant anxiety and one-tenth had clinically significant depression. Young ARVD/C patients had significant body image concerns and distress related to their device. Both generalized and device-specific anxiety were elevated beyond what is the norm for ICD patients. Poor device adjustment, while unfavorable in and of itself, was additionally associated with increased likelihood of clinically significant depression and anxiety. Factors associated with poor adjustment included: 1) younger age, 2) having a device implanted recently, 3) having at least one ICD shock, and 4) having a poorer functional capacity.
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James, C.A.1
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1617780 10.1161/01.CIR.86.1.29 1:STN:280:DyaK38zhsFChsQ%3D%3D
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21292134 10.1016/j.jacc.2010.09.046 1:CAS:528:DC%2BC3MXjtlSns7s%3D This study used a murine model of ARVD/C carrying a heterozygous plakoglobin deletion to test the utility of load-reducing therapy (furosemide and nitrates) at preventing training-induced right ventricular enlargement and arrhythmias. Load reducing therapy appeared effective at changing cardiac response to exercise as therapy prevented training-induced right ventricular enlargement and ventricular tachycardias were less often induced in isolated hearts of treated plakoglobin+ hearts than in hearts of untreated plakoglobin+ littermates
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Fabritz L, Hoogendijk MG, Scicluna BP, et al. Load-reducing therapy prevents development of arrhythmogenic right ventricular cardiomyopathy in plakoglobin-deficient mice. J Am Coll Cardiol. 2011;57:740-50. This study used a murine model of ARVD/C carrying a heterozygous plakoglobin deletion to test the utility of load-reducing therapy (furosemide and nitrates) at preventing training-induced right ventricular enlargement and arrhythmias. Load reducing therapy appeared effective at changing cardiac response to exercise as therapy prevented training-induced right ventricular enlargement and ventricular tachycardias were less often induced in isolated hearts of treated plakoglobin+ hearts than in hearts of untreated plakoglobin+ littermates.
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19620503 10.1161/CIRCULATIONAHA.108.834903
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Bai R, Di Biase L, Shivkumar K, et al. Ablation of ventricular arrhythmias in arrhythmogenic right ventricular dysplasia/cardiomyopathy: arrhythmia-free survival after endo-epicardial substrate based mapping and ablation. Circ Arrhy Electrophysiol. 2011;4:478-85. This paper assessed survival from recurrent ventricular tachycardia among 49 ARVD/C patients in the US, Italy, Colombia, and Canada undergoing catheter ablation using an epicardial-alone approach (23 patients) or an endo-epicardial ablation (26 patients). After a minimum 3-year follow-up, freedom from recurrent ventricular arrhythmia was significantly greater among those who had undergone combined endo-epicardial ablation (52 % vs. 85 %).
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10.1161/CIRCEP.111.968677 This study reported on 87 ARVD/C patients who underwent a total of 175 catheter ablation procedures. Over a mean follow-up of 88 ± 66 months, the overall freedom of ventricular tachycardia was 47 %, 21 %, and 15 % at 1, 5, and 10 years, respectively. The cumulative freedom from ventricular tachycardia following epicardial ablation was 64 % and 45 % at 1 and 5 years. Importantly, the burden of ventricular tachycardia decreased following ablation from a median of 0.16 VT episodes per month pre ablation to 0.08 episodes per month post ablation, with the best results occurring among patients treated by epicardial ablation
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Philips B, Madhavan S, James C, et al. Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia/ cardiomyopathy. Circ Arrhy Electrophysiol. 2012;5:499-505. This study reported on 87 ARVD/C patients who underwent a total of 175 catheter ablation procedures. Over a mean follow-up of 88 ± 66 months, the overall freedom of ventricular tachycardia was 47 %, 21 %, and 15 % at 1, 5, and 10 years, respectively. The cumulative freedom from ventricular tachycardia following epicardial ablation was 64 % and 45 % at 1 and 5 years. Importantly, the burden of ventricular tachycardia decreased following ablation from a median of 0.16 VT episodes per month pre ablation to 0.08 episodes per month post ablation, with the best results occurring among patients treated by epicardial ablation.
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22240135 10.1016/j.jacc.2011.09.051 This paper describes 18 ARVD/C patients who required cardiac transplant. The average age of first ARVD/C symptoms was 24 ± 13 years (median age 18 years). The two most common clinical reasons for initial presentation were heart failure in five patients (28 %) and sustained VT in five patients (28 %). The average age at cardiac transplant was 40 ± 14 years (median age 44 years). Thirteen patients received transplants primarily as a result of heart failure symptoms (biventricular failure in four patients, predominately right ventricular failure in four patients, right ventricular failure only in five patients), and five received transplants for predominately ventricular tachycardia. Most patients who received transplants for heart failure had a prolonged clinical course of ARVD/C and heart failure symptom onset (17.6 ± 13.3 years and 7.2 ± 6.4 years, respectively)
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Tedford RJ, James C, Judge DP, et al. Cardiac transplantation in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2012;59:289-90. This paper describes 18 ARVD/C patients who required cardiac transplant. The average age of first ARVD/C symptoms was 24 ± 13 years (median age 18 years). The two most common clinical reasons for initial presentation were heart failure in five patients (28 %) and sustained VT in five patients (28 %). The average age at cardiac transplant was 40 ± 14 years (median age 44 years). Thirteen patients received transplants primarily as a result of heart failure symptoms (biventricular failure in four patients, predominately right ventricular failure in four patients, right ventricular failure only in five patients), and five received transplants for predominately ventricular tachycardia. Most patients who received transplants for heart failure had a prolonged clinical course of ARVD/C and heart failure symptom onset (17.6 ± 13.3 years and 7.2 ± 6.4 years, respectively).
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15837284 10.1016/j.jacc.2005.02.011
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