Evaluation of Structural Progression in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Mast, Thomas P.; James, Cynthia A.; Calkins, Hugh; Teske, Arco J.; Tichnell, Crystal; Murray, Brittney; Loh, Peter; Russell, Stuart D.; Velthuis, Birgitta K.; Judge, Daniel P.; Dooijes, Dennis; Tedford, Ryan J.; Heijden, Jeroen F. van der; Tandri, Harikrishna; Hauer, Richard N.W.; Abraham, Theodore P.; Doevendans, Pieter A.; Riele, Anneline S.J.M. te; Cramer, Maarten J.

doi:10.1001/jamacardio.2016.5034

Cited by 57 publications

(58 citation statements)

References 34 publications

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“…The endpoint for trials targeting cardiac arrhythmias might include time to the first episode of sustained ventricular tachycardia/fibrillation or premature ventricular complex frequency, as these appear to predict the risk of sustained ventricular arrhythmias. Trials targeting heart failure endpoints are challenging due to slow and variable disease progression . Serial evaluation of structural myocardial abnormalities is difficult due to the limitations of conventional echocardiography in assessing the right ventricle and because the presence of an implantable cardioverter‐defibrillator in many patients precludes serial magnetic resonance imaging.…”

Section: What Are the Prospects For Trials In Arrhythmogenic Cardiomymentioning

confidence: 99%

Definition and treatment of arrhythmogenic cardiomyopathy: an updated expert panel report

Elliott

Anastasakis

Asimaki

et al. 2019

European J of Heart Fail

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109

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It is 35 years since the first description of arrhythmogenic right ventricular cardiomyopathy (ARVC) and more than 20 years since the first reports establishing desmosomal gene mutations as a major cause of the disease. Early advances in the understanding of the clinical, pathological and genetic architecture of ARVC resulted in consensus diagnostic criteria, which proved to be sensitive but not entirely specific for the disease. In more recent years, clinical and genetic data from families and the recognition of a much broader spectrum of structural disorders affecting both ventricles and associated with a propensity to ventricular arrhythmia have raised many questions about pathogenesis, disease terminology and clinical management. In this paper, we present the conclusions of an expert round table that aimed to summarise the current state of the art in arrhythmogenic cardiomyopathies and to define future research priorities.

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Section: What Are the Prospects For Trials In Arrhythmogenic Cardiomymentioning

confidence: 99%

Definition and treatment of arrhythmogenic cardiomyopathy: an updated expert panel report

Elliott

Anastasakis

Asimaki

et al. 2019

European J of Heart Fail

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109

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“…Exercise restriction has been shown to be important to decrease the progression of the disease to reduce the likelihood of sustained ventricular arrhythmias 22–27 .The fourth leg is to prevent progression of the disease. A recent study compared paired echocardiograms obtained an average of 6.4 years apart in 85 patients with ARVC 28 . During this time, the RV size increased, and the RV and LV function decreased.…”

Section: Management Of Arvcmentioning

confidence: 99%

Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

2017

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Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited cardiomyopathy that is characterized by ventricular arrhythmias, and an increased risk of sudden death (SCD). Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common particularly in advanced disease, and that left dominant forms occur. The pathologic characteristic of ARVC/D is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease. Once the diagnosis of ARVC is established, the single most important clinical decision is whether a particular patient’s SCD risk is sufficient to justify placement of an implantable cardioverter defibrillator (ICD). The importance of this decision reflects the fact that ARVC is a common cause of sudden death in young people, and that sudden cardiac death may be the first manifestation of the disease. This decision is particularly important since these are often young patients who are expected to live for many years. While an ICD can save lives in individuals with this disease, it is also well recognized that ICD therapy is associated with both short and long term complications. Decisions regarding placement of an ICD are based on an estimate of a patient’s risk of SCD, as well as their preferences and values. The primary purpose of this article is to provide a review of literature that concerns risk stratification in patients with ARVC and to place this literature into the framework of the three authors considerable lifetime experiences in caring for patients with ARVC. The most important parameters to consider when determining arrhythmic risk include: 1) electrical instability including frequency of PVC’s and sustained VA, 2) proband status, 3) extent of structural disease, 4) cardiac syncope, 5) male gender, 6) the presence of multiple mutations or a mutation in TMEM 43, and 7) the patient’s willingness to restrict exercise and eliminate participation in competitive or endurance exercise.

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“…The phenomenon of the Epsilon wave is attractive but represents a small part of the modification of activation in the diseased myocardium. This is easily explained by the histology of the tissue affected by the dysplastic phenomenon (Fontaine, ; Fontaine & Chen, ; Fontaine et al., ; Marcus et al., ; Mast et al., ). The same phenomenon also occurs in CAD.…”

Section: Emerging Methods Of Recording Epsilon Wavesmentioning

confidence: 99%

“…All of these ECG anomalies were the result of the special histologic structure of ARVD with surviving fibers embedded in fat and fibrous tissue mostly located in the RV (Fontaine et al., ; Mallat et al., ; Marcus et al., ; Mast et al., ; Zhang, Liu, Kowey, & Fontaine, ). Nevertheless, it was possible to correlate this phenomenon with prolongations of the QRS located in the right precordial leads.…”

Section: Complex Expression Of Ecg Anomalies Identified As Epsilon Wavesmentioning

confidence: 99%

Epsilon waves: Milestones in the discovery and progress

Saguner

Fontaine

et al. 2018

Noninvasive Electrocardiol

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The Epsilon wave was first identified in 1977. Four decades of progress help people to better understand its pathological electrogenesis and diagnostic value. Currently, the Epsilon wave is on the list of the 2010 Task Force recommendations for the diagnosis of arrhythmogenic right ventricular dysplasia (ARVD). In this review, we provide the history of the first recording of the Epsilon wave in coronary artery disease and Uhl's anomaly, subsequently leading to the signal averaging technique to record late potentials. Based on our experience, we discuss some existing controversies. When we look back at the decades of progress of the Epsilon wave, we conclude that the Epsilon wave is only the tip of the iceberg of ECG abnormalities in ARVD.

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Evaluation of Structural Progression in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Cited by 57 publications

References 34 publications

Definition and treatment of arrhythmogenic cardiomyopathy: an updated expert panel report

Definition and treatment of arrhythmogenic cardiomyopathy: an updated expert panel report

Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy

Epsilon waves: Milestones in the discovery and progress

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