Abstract Background Incomplete penetrance and variable expressivity of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) complicate family screening. Objectives The objective of the ...present study was to determine the optimal approach to longitudinal follow-up regarding: 1) screening interval; and 2) testing strategy in at-risk relatives of ARVD/C patients. Methods We included 117 relatives (45% male, age 33.3 ± 16.3 years) from 64 families who were at risk of developing ARVD/C by virtue of their familial predisposition (72% mutation carriers 92% plakophilin-2; 28% first-degree relatives of a mutation-negative proband). Subjects were evaluated by electrocardiography (ECG), Holter monitoring, signal-averaged ECG, and cardiac magnetic resonance (CMR). Disease progression was defined as the development of a new criterion by the 2010 Task Force Criteria (not the “Hamid criteria”) at last follow-up that was absent at enrollment. Results At first evaluation, 43 subjects (37%) fulfilled an ARVD/C diagnosis according to the 2010 Task Force Criteria. Among the remaining 74 subjects (63%), 11 of 37 (30%) with complete re-evaluation experienced disease progression during 4.1 ± 2.3 years of follow-up. Electrical progression (n = 10 27%, including by ECG 14%, Holter monitoring 11%, or signal-averaged ECG 14%) was more frequently observed than structural progression (n = 1 3% on CMR). All 5 patients (14%) with clinical ARVD/C diagnosis at last follow-up had an abnormal ECG or Holter monitor recording, and the only patient with an abnormal CMR already had an abnormal ECG at enrollment. Conclusions Over a mean follow-up of 4 years, our study showed that: 1) almost one-third of at-risk relatives have electrical progression; 2) structural progression is rare; and 3) electrical abnormalities precede detectable structural changes. This information could be valuable in determining family screening protocols.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Abstract Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy that is characterized clinically by ventricular arrhythmias and an increased risk of sudden ...death and pathologically by fibrofatty replacement of the myocardium. Soon after the initial description of the disease, investigators and clinicians caring for patients with ARVD/C recognized a link between exercise and ARVD/C. In this article, we review the considerable body of epidemiologic data, basic research, and clinical research supporting the link between exercise and the development and outcomes of ARVD/C. Based on these data, we now advise that patients with ARVD/C avoid participation in competitive athletics and limit exercise as much as possible.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Abstract Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy and is also called ARVD/C. In most cases, ARVD is inherited in an autosomal-dominant pattern and clinically ...is characterized by ventricular arrhythmias with an increased risk of sudden death. Although it is prominently a disease of the right ventricle (RV), involvement of the left ventricle is not uncommon. Pathologically, there is RV myocardial atrophy with thinning of the wall, aneurysm, and global RV dilatation. Genetic testing and magnetic resonance imaging of the RV are an increasing aspect of the diagnosis. Diagnosis relies on a scoring system with major or minor criteria. Prevention of sudden death is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and implantable cardioverter defibrillator are the mainstay of treatment of ARVD/C.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Persistent and long-standing persistent atrial fibrillation (AF) often requires extensive and/or repeat radiofrequency (RF) ablation procedures.
The Tailored Treatment of Persistent Atrial ...Fibrillation (TTOP-AF) study assessed the effectiveness and safety of the phased RF system in a randomized controlled comparison of medical therapy against phased RF ablation for the management of persistent and long-standing persistent AF.
Patients who had failed at least 1 antiarrhythmic drug (AAD) were randomized (2:1) to ablation management (AM) or medical management (MM). AM patients were allowed up to 2 ablations. Index and retreatment procedures consisted of pulmonary vein isolation and ablation of complex fractionated atrial electrograms. MM patients received AAD changes and/or cardioversion. The primary end points of the TTOP-AF study included chronic effectiveness and safety at 6 months and acute safety within 7 days of ablation.
At 6 months, a greater proportion of AM patients achieved effectiveness off AAD (77 of 138 55.8%) compared to MM patients (19 of 72 26.4%) (P < .0001). Acutely, 92.8% (128/138) of the procedures were successful while 12.3% (17/138) experienced a serious procedure and/or device-related adverse event. The predefined acute safety end point was not met. The proportion of patients with chronic safety events did not differ significantly between groups.
Catheter ablation of persistent/long-standing persistent AF with the phased RF ablation system is effective with greater reduction of AF compared with MM. More intense anticoagulation strategies, careful attention to catheter placement relative to the pulmonary vein ostia, and elimination of electrode interaction are expected to reduce the risk of stroke, pulmonary vein stenosis, and asymptomatic cerebral emboli.
Atrial fibrillation (AF) is an age-related arrhythmia of enormous socioeconomic significance. In recent years, our understanding of the basic mechanisms that initiate and perpetuate AF has evolved ...rapidly, catheter ablation of AF has progressed from concept to reality, and recent studies suggest lifestyle modification may help prevent AF recurrence. Emerging developments in genetics, imaging, and informatics also present new opportunities for personalized care. However, considerable challenges remain. These include a paucity of studies examining AF prevention, modest efficacy of existing antiarrhythmic therapies, diverse ablation technologies and practice, and limited evidence to guide management of high-risk patients with multiple comorbidities. Studies examining the long-term effects of AF catheter ablation on morbidity and mortality outcomes are not yet completed. In many ways, further progress in the field is heavily contingent on the feasibility, capacity, and efficiency of clinical trials to incorporate the rapidly evolving knowledge base and to provide substantive evidence for novel AF therapeutic strategies. This review outlines the current state of AF prevention and treatment trials, including the foreseeable challenges, as discussed by a unique forum of clinical trialists, scientists, and regulatory representatives in a session endorsed by the Heart Rhythm Society at the 12th Global CardioVascular Clinical Trialists Forum in Washington, DC, December 3-5, 2015.
Revision of the Task Force diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) has increased their sensitivity for the diagnosis of early and familial forms of ...the disease. The epsilon wave is a major diagnostic criterion in the context of ARVC/D, which, however, remains not quantifiable and therefore may leave room for substantial subjective interpretation.
The purpose of this study was to assess interobserver agreement in epsilon wave definition and epsilon wave importance for ARVC/D diagnosis.
Electrocardiographic (ECG) tracings depicting leads V1, V2, and V3 collected from individuals evaluated for ARVC/D (n = 30) were given to panel members who were asked to respond to the question whether ECG patterns meet epsilon wave definition outlined by the Task Force diagnostic criteria. The prevalence and importance of epsilon waves for ARVC/D diagnosis were assessed in a pooled data set of patients with definite ARVC/D from European and American registries (n = 815).
The number of ECG patterns identified as epsilon waves varied from 5 to 18 per reviewer (median 13 per reviewer). A unanimous agreement was reached for only 10 cases (33%), 2 of which qualified as epsilon waves and 8 as non-epsilon waves by all panel members. From a pooled data set, 106 patients reportedly had epsilon waves (13%). In 105 of 106 patients with epsilon waves (99%), exclusion of epsilon waves from the diagnostic score would not affect the "definite" diagnostic category.
Interobserver variability in the assessment of epsilon waves is high; however, the impact of epsilon waves on ARVC/D diagnosis is negligibly low. The results urge to exercise caution in the assessment of epsilon waves, especially in patients who would not otherwise meet diagnostic criteria.
A need exists to develop alternative approaches to VT ablation that provide an improved delineation of the arrhythmogenic substrate.
The aim of this study was to evaluate the hypotheses that: (1) the ...heterogeneous zone (HZ, a mixture of normal-appearing tissue and scar) in magnetic resonance imaging (MRI) contains the critical isthmus(es) for ventricular tachycardia (VT), (2) successful ablation of VT would include ablation in the HZ, and (3) inadequate ablation of HZ allows for VT recurrence.
MRI and an electrophysiology study (EP) were performed in a model of chronic myocardial infarction in 17 pigs. In animals that were inducible for VT, ablations were done guided by standard EP criteria and blinded to the MRI. After ablation, electroanatomic mapping results were co-registered with MRI.
In 8 animals, 22 sustained monomorphic VTs were generated. The HZ was substantially larger in inducible (n = 8) compared with noninducible animals (n = 9) 25% ± 10% vs 13% ± 5% of total scar, respectively, P = .007. Acutely, all targeted VTs were successfully ablated, and postprocedure analysis showed that at least 1 ablation was in the HZ in each animal. In 5 animals, a second EP and MRI were performed 1 week after ablation. Three animals had inducible VTs, and MRI showed that the HZ had not been completely ablated. In contrast, the 2 animals without inducible VT revealed no remaining HZ.
These findings show that MRI can define an HZ and determine the location of ablated lesions. The HZ may be a promising ablation target to cure ischemic VTs. Remnants of HZ after ablation may be the substrate for clinical relapses.
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