Ablation of Ventricular Arrhythmias in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Arrhythmia-Free Survival After Endo-Epicardial Substrate Based Mapping and Ablation
In patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy, freedom from ventricular arrhythmias (VAs) after endocardial ablation is limited. We compared the long-term freedom from recurrent VAs by using endocardial-alone ablation versus endo-epicardial substrate-based ablation. Forty-nine patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy undergoing ablation of ventricular tachycardia (VT) were divided into 2 groups: endocardial-alone ablation (group 1, n = 23) and endo-epicardial ablation (group 2, n = 26). All patients had an implantable cardioverter-defibrillator (ICD). Conventional and 3D mappings were used to determine the mechanism of induced VTs and to identify area of "scar" or "abnormal" myocardium. All critical sites responsible for VTs and points with "abnormal" potential were targeted for ablation from endocardium (group 1) or from both endocardium and epicardium (group 2). The procedural end point was noninducibility of sustained, monomorphic VT with isoproterenol. The presence of frequent premature ventricular contractions at the end of ablation was recorded. Patients were followed up by ECG, Holter, and ICD interrogation. After a follow-up of at least 3 years, freedom from VAs or ICD therapy was 52.2% (12/23) in group 1 and 84.6% (22/26) in group 2 (P = 0.029), with 21.7% (5/23) and 69.2% (18/26) patients off antiarrhythmic drugs (P < 0.001), respectively. Compared with patients with no premature ventricular contractions after ablation, patients with frequent premature ventricular contractions after ablation were more likely to have VA recurrence/ICD therapy [3/33 (9%) versus 12/16 (75%); log-rank P<0.001]. An endo-epicardial-based ablation strategy achieves higher long-term freedom from recurrent VAs off antiarrhythmic therapy in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy when compared with endocardial-alone ablation. The presence of ≥ 10 premature ventricular contractions per minute after ablation is associated with more VA recurrence.
[Show abstract] [Hide abstract] ABSTRACT: Cardiomyopathies arising due to a single gene defect represent various pathways that evoke adverse remodeling and cardiac dysfunction. While the gene therapy approach is slowly evolving and has not yet reached clinical "prime time" and gene correction approaches are applicable at the bench but not at the bedside, major advances are being made with molecular and drug therapies. This review summarizes the contemporary drugs introduced or being tested to help manage these unique disorders bearing a major impact on the quality of life and survival of the affected individuals. The restoration of the RNA reading frame facilitates the expression of partly functional protein to salvage or alleviate the disease phenotype. Chaperones are used to prevent the degradation of abnormal but still functional proteins, while other molecules are given for pathogen silencing, to prevent aggregation or to enhance clearance of protein deposits. Absence of protein may be managed by viral gene delivery or protein therapy. Enzyme replacement therapy is already a clinical reality for a series of metabolic diseases. The progress in molecular biology, based on the knowledge of the gene defect, helps generate small molecules and pharmaceuticals targeting the key events occurring in the malfunctioning element of the sick organ. Cumulatively, these tools augment the existing armamentarium of phenotype oriented symptomatic and evidence-based therapies for patients with inherited cardiomyopathies. Copyright © 2015. Published by Elsevier Inc.0Comments 0Citations
- "Patients with aborted SCD and syncope have an indication for implantable cardioverter-defibrillator implantation. Another option for recurrent arrhythmia would be a catheter ablation of VT focus (Bai et al., 2011; Tabib et al., 2003 ). There is no established therapy for isolated right ventricular dysfunction. "
[Show abstract] [Hide abstract] ABSTRACT: Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is one of the most arrhythmogenic forms of inherited cardiomyopathy and a frequent cause of sudden death in the young. Affected individuals typically present between the second and fourth decade of life with arrhythmias coming from the right ventricle. Pathogenic mutations in genes encoding the cardiac desmosome can be found in approximately 60% of index patients, leading to our current perception of ARVC as a desmosomal disease. Although ARVC is known to preferentially affect the right ventricle, early and/or predominant left ventricular involvement is increasingly recognized. Diagnosis is made by combining multiple sources of diagnostic information as prescribed by the "Task Force" criteria. Recent research suggests that electrical abnormalities precede structural changes in ARVC. Cardiovascular Magnetic Resonance (CMR) is an ideal technique in ARVC workup, as it provides comprehensive information on cardiac morphology, function, and tissue characterization in a single investigation. Prevention of sudden cardiac death using implantable cardioverter-defibrillators is the most important management consideration. This purpose of this paper is to provide an updated review of our understanding of the genetics, diagnosis, current state-of-the-art CMR acquisition and analysis, and management of patients with ARVC.0Comments 14Citations
- "In addition, radiofrequency ablation for ventricular arrhythmia in ARVC has gained enormous popularity over the last years. Although the results of endocardial ablation have been moderate  , good arrhythmia control (but not complete cure) has been obtained using epicardial ablation [52,122123124. This is understandable, given the primary (sub)epicardial location of the abnormal substrate in ARVC. "
[Show abstract] [Hide abstract] ABSTRACT: Arrhythmogenic cardiomyopathy (AC), also known as arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), is a hereditary disease characterised by ventricular arrhythmias, right ventricular and/or left ventricular dysfunction, and fibrofatty replacement of cardiomyocytes. Patients with AC typically present between the second and the fourth decade of life with ventricular tachycardias. However, sudden cardiac death (SCD) may be the first manifestation, often at young age in the concealed stage of disease. AC is diagnosed by a set of clinically applicable criteria defined by an international Task Force. The current Task Force Criteria are the essential standard for a correct diagnosis in individuals suspected of AC. The genetic substrate for AC is predominantly identified in genes encoding desmosomal proteins. In a minority of patients a non-desmosomal mutation predisposes to the phenotype. Risk stratification in AC is imperfect at present. Genotype-phenotype correlation analysis may provide more insight into risk profiles of index patients and family members. In addition to symptomatic treatment, prevention of SCD is the most important therapeutic goal in AC. Therapeutic options in symptomatic patients include antiarrhythmic drugs, catheter ablation, and ICD implantation. Furthermore, patients with AC and also all pathogenic mutation carriers should be advised against practising competitive and endurance sports.0Comments 7Citations
- "Accordingly, in a recent study by Philips et al.  the overall freedom from VT of 175 ablation procedures in 87 AC patients was 47 %, 21 %, and 15 %, at 1, 5, and 10 years, respectively, over a mean follow-up of 88.3 ± 66 months. The outcomes of VT ablation are improved with a combined endocardial and epicardial approach, incorporating a substrate-based strategy [69, 70]. "