Implantable Cardioverter-Defibrillator Therapy for Prevention of Sudden Death in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Abstract

Background-Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition associated with the risk of sudden death (SD). Methods and Results-We conducted a multicenter study of the impact of the implantable cardioverter-defibrillator (ICD) for prevention of SD in 132 patients (93 males and 39 females, age 40Ϯ15 years) with ARVC/D. Implant indications were a history of cardiac arrest in 13 patients (10%), sustained ventricular tachycardia in 82 (62%), syncope in 21 (16%), and other in 16 (12%). During a mean follow-up of 39Ϯ25 months, 64 patients (48%) had appropriate ICD interventions, 21 (16%) had inappropriate interventions, and 19 (14%) had ICD-related complications. Fifty-three (83%) of the 64 patients with appropriate interventions received antiarrhythmic drug therapy at the time of first ICD discharge. Programmed ventricular stimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49%, negative predictive value 54%). Four patients (3%) died, and 32 (24%) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device. At 36 months, the actual patient survival rate was 96% compared with the ventricular fibrillation/flutter-free survival rate of 72% (PϽ0.001). Patients who received implants because of ventricular tachycardia without hemodynamic compromise had a significantly lower incidence of ventricular fibrillation/flutter (log rankϭ0.01). History of cardiac arrest or ventricular tachycardia with hemodynamic compromise, younger age, and left ventricular involvement were independent predictors of ventricular fibrillation/flutter. Conclusions-In

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

“…The first and largest of these studies consisted of 132 patients and was published in 2003 by Corrado et al 10. Similar to our study, the authors reported an annual incidence of VF/VFL of 3.3% per year, which is remarkably similar to the rate of 3.6% per year reported in our study.…”

“…The median age at ICD implantation was 36.5 years. The demographic features of patients undergoing ICD implantation in this study are generally similar to those in prior reports 10, 11, 12, 13, 15, 16, 17, 18, 21. 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.…”

“…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.…”

Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life‐Threatening Arrhythmias

“…In DCM, a family history of SCD/SVT/VF is associated with an increased risk of life‐threatening arrhythmias, a concept well established in other forms of arrhythmogenic cardiomyopathies such as ARVC33, 34 and hypertrophic cardiomyopathy 32, 35. Even though none of our AR‐DCM patients fulfilled new task force criteria of ARVC24 with LV involvement, the potential overlap with desmosomal diseases (ARVC, arrhythmogenic cardiomyopathy, left‐dominant arrhythmogenic cardiomyopathy), considered to be distinct disorders,10 warrants further investigations 36…”

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

“…High-risk groups with an increased risk of SCD should be identified. These include young patients who have had a previous syncopal episode, history of cardiac arrest or ventricular tachyarrhythmia, two or more ARVC genetic mutations, patients having ARVC with LV involvement, and patients with TMEM43 gene missense mutation [23–27]. …”