Prophylactic Implantable Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia and No Prior Ventricular Fibrillation or Sustained Ventricular Tachycardia

Corrado, Domenico; Calkins, Hugh; Link, Mark S.; Leoni, Loira; Favale, Stefano; Bevilacqua, Michela; Basso, Cristina; Ward, Deirdre; Boriani, Giuseppe; Ricci, Renato Pietro; Piccini, Jonathan P.; Dalal, Darshan; Santini, Massimo; Buja, Gianfranco; Iliceto, Sabino; Estes, N.A. Mark; Wichter, Thomas; McKenna, William J.; Thiene, Gaetano; Marcus, Frank I.

doi:10.1161/circulationaha.109.913871

Cited by 279 publications

(224 citation statements)

References 32 publications

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“…Nevertheless, this study is unique in that only patients who met diagnostic criteria were included, and a greater proportion of our ARVD/C population underwent ICD implantation for primary prevention than has been reported in prior series 10, 11, 12, 13, 16, 17, 18, 21. In addition, this cohort includes a lower proportion of male patients than most series of arrhythmogenic right ventricular cardiomyopathy patients with ICDs,10, 11, 14, 17, 20 and the median age at ICD implantation in our series was somewhat higher than in prior studies 17, 21…”

Section: Discussionmentioning

confidence: 99%

“…Since the first major description of ARVD/C in 1992 by Marcus et al,9 a number of articles have been published describing the clinical features and outcomes of ARVD/C patients who undergo placement of an ICD. Although these articles have provided important information, they also have important limitations including small sample sizes ranging from 26 to 132 patients,10, 11, 12, 13, 14, 15, 16, 17, 18, 19 the use of the 1994 task force diagnostic criteria,10, 11, 12, 13, 14, 16, 20, 21 inclusion of participants who did not fully meet diagnostic criteria for ARVD/C,13, 15 and lack of genetic testing 10, 11, 12, 13, 14, 16, 20, 21. Furthermore, the primary end point in many of these studies was the prediction of appropriate ICD therapy, including both sustained VT and VF/VFL 11, 12, 13, 15, 16, 17, 18, 19, 21.…”

Section: Discussionmentioning

confidence: 99%

“…In the second study looking at predictors of VF/VFL,14 syncope was the only independent predictor of VF/VFL therapy in a primary‐prevention cohort. A recently published study19 looking at a cohort of ARVD/C patients that had ICDs predominantly for primary prevention of SCD (68/81, 84%) also found that syncope independently increased the risk of life‐threatening arrhythmic events, defined as SCD, aborted cardiac arrest, syncopal VT or electrical storm, or cardiovascular mortality.…”

Section: Discussionmentioning

confidence: 99%

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Implantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications

Orgeron

James

Riele

et al. 2017

JAHA

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BackgroundArrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by ventricular arrhythmias and sudden cardiac death. Once the diagnosis is established, risk stratification to determine whether implantable cardioverter‐defibrillator (ICD) placement is warranted is critical.Methods and ResultsThe cohort included 312 patients (163 men, age at presentation 33.6±13.9 years) with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy who received an ICD. Over 8.8±7.33 years, 186 participants (60%) had appropriate ICD therapy and 58 (19%) had an intervention for ventricular fibrillation/flutter. Ventricular tachycardia at presentation (hazard ratio [HR]: 1.86; 95% confidence interval [CI], 1.38–2.49; P<0.001), inducibility on electrophysiology study (HR: 3.14; 95% CI, 1.95–5.05; P<0.001), male sex (HR: 1.62; 95% CI, 1.20–2.19; P=0.001), inverted T waves in ≥3 precordial leads (HR: 1.66; 95% CI, 1.09–2.52; P=0.018), and premature ventricular contraction count ≥1000/24 hours (HR: 2.30; 95% CI, 1.32–4.00; P=0.003) were predictors of any appropriate ICD therapy. Inducibility at electrophysiology study (HR: 2.28; 95% CI, 1.10–4.70; P=0.025) remained as the only predictor after multivariable analysis. The predictors for ventricular fibrillation/flutter were premature ventricular contraction ≥1000/24 hours (HR: 4.39; 95% CI, 1.32–14.61; P=0.016), syncope (HR: 1.85; 95% CI, 1.10–3.11; P=0.021), aged ≤30 years at presentation (HR: 1.76; 95% CI, 1.04–3.00; P<0.036), and male sex (HR: 1.73; 95% CI, 1.01–2.97; P=0.046). Younger age at presentation (HR: 3.14; 95% CI, 1.32–7.48; P=0.010) and high premature ventricular contraction burden (HR: 4.43; 95% CI, 1.35–14.57; P<0.014) remained as independent predictors of ventricular fibrillation/flutter. Complications occurred in 66 participants (21%), and 64 (21%) had inappropriate ICD interventions. Overall mortality was low at 2%, and 4% underwent heart transplantation.ConclusionThese findings represent an important step in identifying predictors of ICD therapy for potentially fatal ventricular fibrillation/flutter and should be considered when developing a risk stratification model for arrhythmogenic right ventricular dysplasia/cardiomyopathy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Implantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications

Orgeron

James

Riele

et al. 2017

JAHA

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“…ICD placement is the only proven mortality benefit treatment for ARVC [11,23,24,27,29,52–54]. ICD placement can also be used for primary or secondary prevention [23,24,54].…”

Section: Icd Placementmentioning

confidence: 99%

“…ICD placement can also be used for primary or secondary prevention [23,24,54]. Multiple observational studies have shown clinical efficacy of ICD placement for preventing SCD in patients with ARVC [11,23,54–57].…”

Section: Icd Placementmentioning

confidence: 99%

Right ventricular dysplasia: management and treatment in light of current evidence

Idris

Shah

Park

2018

Journal of Community Hospital Internal Medicine Perspectives

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiovascular disease that predisposes to ventricular arrhythmias potentially leading to sudden cardiac death (SCD). ARVC varies considerably with multiple clinical presentations, ranging from no symptoms to cardiac arrhythmias to SCD. ARVC prevalence is not well known, but the estimated prevalence in the general population is 1:5000. Diagnosis of ARVC can be made by using the Revised European Society of Cardiology criteria for ARVC that includes ventricular structural and functional changes, ECG abnormalities, arrhythmias, family and genetic factors. The management of ARVC is focused on prevention of lethal events such as SCD. Implantable cardioverter defibrillator placement is the only proven mortality benefit in treatment of ARVC. Other treatment strategies include medications such as beta blockers and antiarrhythmics, radiofrequency ablation, surgery, cardiac transplantation, and lifestyle changes. All these interventions help in symptomatic treatment but none of them have proved to decrease mortality rates. ARVC is a progressive disease that leads to SCD if not treated appropriately. Management of these diseases has been a challenge for physicians. With the advent of technology and many new drugs/devices under clinical investigation, this might change in the future. However, while advances in technologies have helped elucidate many aspects of these diseases, many mysteries still remain of this unique disease. With continued research, we can expect more cost-effective and patient-friendly drug therapies and ablation techniques to be developed in the near future.

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Indications for implantable cardioverter defibrillator therapy, device, and lead selection

Cohen

Etheridge

2017

Cardiac Pacing and Defibrillation in Pediatric and Congenital Heart Disease

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Prophylactic Implantable Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia and No Prior Ventricular Fibrillation or Sustained Ventricular Tachycardia

Abstract: Background-The

Cited by 279 publications

References 32 publications

Implantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications

Implantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications

Right ventricular dysplasia: management and treatment in light of current evidence

Indications for implantable cardioverter defibrillator therapy, device, and lead selection

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