Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Screening, diagnosis, and treatment

Kiès, Philippine; Bootsma, Marianne; Bax, Jeroen J.; Schalij, Martin J.; Wall, Ernst E. van der

doi:10.1016/j.hrthm.2005.10.018

Cited by 115 publications

(84 citation statements)

References 34 publications

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“…These key areas identified have been correlated to relevant histopathologic findings (myocyte loss with fibrofatty replacement) and critical VT circuits confirming the involvement of these areas in the arrhythmogenic mechanism [14] . The endocardial distribution of electroanatomic scar in patients with VT and ARVC/D typically extends from the triscuspid valve and/or the pulmonary valve to the RV free wall.…”

Section: Endocardial Substratementioning

confidence: 60%

Ventricular tachycardia mapping and ablation in arrhythmogenic right ventricular cardiomyopathy/dysplasia: Lessons Learned

Tschabrunn

Marchlinski

2014

WJC

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Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is primarily believed to be an inherited cardiomyopathy that subsequently results in significant myocardial fibrosis. The arrhythmogenic consequences that result from the development of fibrosis are similar to other nonischemic cardiomyopathies, but the unique endocardial-epicardial disease process of ARVC/D requires a specialized approach for arrhythmia treatment in the electrophysiology laboratory. Although the association between ARVC/D and development of ventricular arrhythmias has become increasingly clear over the last 2 decades, our understanding of the arrhythmia mechanisms, underlying electrophysiologic substrate, and treatment strategies were significantly limited. Prospective studies performed in the electrophysiology laboratory allowed detailed characterization of the electrophysiologic and electroanatomic substrate underlying ventricular tachycardia in patients with ARVC/D. This has allowed clinician scientists to better characterize the arrhythmia mechanism and develop the necessary strategies to perform successful catheter ablation. Early in this experience, catheter ablation was considered a limited and largely unsuccessful treatment for patients experiencing painful and recurrent defibrillator therapy. Through our increased understanding of the disease process, catheter ablation has evolved to become an effective and preferred therapy for a majority of these patients. Our understanding of the disease and necessary approaches to provide successful treatment continues to evolve as the clinical experience grows. This article will review these important insights from the electrophysiology laboratory and how application of this knowledge has facilitated the development of a methodical approach to successfully perform ventricular tachycardia ablation in patients with ARVC/D.

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Section: Endocardial Substratementioning

confidence: 60%

Ventricular tachycardia mapping and ablation in arrhythmogenic right ventricular cardiomyopathy/dysplasia: Lessons Learned

Tschabrunn

Marchlinski

2014

WJC

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“…Major and minor criteria have been proposed in an attempt to diagnose ARVD more definitively (5). In our case the only criteria was the fatty replacement of myocardium; however, for this to be considered as a major criterion it has to be proven on endomyocardial biopsy (5).…”

Section: Case Reportmentioning

confidence: 92%

Lipomatous hypertrophy of the interatrial septum associated with fatty replacement of the ventricular myocardium: A case report

Arroyuelos

Imaz

Padilla

et al. 2007

Magnetic Resonance Imaging

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Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare benign entity characterized by the excessive fat deposition in the interatrial septum with typical sparing of the fossa ovalis. We report a case of a 61-year-old woman with an incidentally found LHIS associated with fatty replacement of the ventricular myocardium and epicardial mediastinal lipomatosis diagnosed by computed tomography (CT) and MRI.

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“…6,8 Arrhythmogenic right ventricular dysplasia / cardiomyopathy Due to exceptional right ventricle morphological and functional characterisation, CMR imaging is considered the noninvasive diagnostic modality of choice for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). 2 Black blood T1-weighed turbo spin echo (TSE) imaging sequences demonstrated fat and fibrous collagen deposition of the right ventricle, typical for ARVD. 2 …”

Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

“…2 Black blood T1-weighed turbo spin echo (TSE) imaging sequences demonstrated fat and fibrous collagen deposition of the right ventricle, typical for ARVD. 2 …”

Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

Cardiac magnetic resonance imaging in daily practice in a peripheral medical centre: description of the first 383 patients

et al. 2010

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Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Screening, diagnosis, and treatment

Cited by 115 publications

References 34 publications

Ventricular tachycardia mapping and ablation in arrhythmogenic right ventricular cardiomyopathy/dysplasia: Lessons Learned

Ventricular tachycardia mapping and ablation in arrhythmogenic right ventricular cardiomyopathy/dysplasia: Lessons Learned

Lipomatous hypertrophy of the interatrial septum associated with fatty replacement of the ventricular myocardium: A case report

Cardiac magnetic resonance imaging in daily practice in a peripheral medical centre: description of the first 383 patients

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