Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear.

Background: Cardiac sarcoidosis (CS) may show overlap in the clinical presentation with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). We sought to investigate patients with CS who were misdiagnosed with ARVD/C and identify clinical features to distinguish these 2 groups.

Methods And Results: Among patients enrolled in the Johns Hopkins ARVD/C registry, 15 patients with definite 2010 diagnostic criteria for ARVD/C were subsequently diagnosed with CS.

Incremental value of cardiac magnetic resonance imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers.

Objectives: The aim of this study was to identify the incremental value and optimal role of cardiac magnetic resonance (CMR) imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)-associated desmosomal mutation carriers without histories of sustained ventricular arrhythmia.

Background: Risk stratification of ARVD/C mutation carriers is challenging.

Methods: Sixty-nine patients (mean age 27.

Risk stratification in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers.

Background: We investigated the role of phenotypic characteristics in stratifying the risk of sustained ventricular arrhythmias in patients harboring arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated mutations.

Methods And Results: Clinical, electrocardiographic, and arrhythmic outcome (composite measure of first occurrence of sustained ventricular tachycardia/resuscitated sudden cardiac death/sudden cardiac death/appropriate implantable cardioverter-defibrillator therapy) data were obtained for 215 patients (104 families; 85% PKP-2). During a mean follow-up of 7 years, 86 (40%) patients experienced the arrhythmic outcome.

High prevalence of catecholamine-facilitated focal ventricular tachycardia in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Background: Exercise-related ventricular tachycardia (VT) and high burden of premature ventricular contractions (PVCs) are common in arrhythmogenic right ventricular dysplasia/cardiomyopathy. We hypothesized that VT in arrhythmogenic right ventricular dysplasia/cardiomyopathy shows a high degree of association with the PVC at baseline.

Methods And Results: The study population included 16 consecutive arrhythmogenic right ventricular dysplasia/cardiomyopathy patients with recurrent VT who underwent catheter ablation.

Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Background: Prior studies evaluating the efficacy of catheter ablation of ventricular tachycardia (VT) among patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) have reported varied outcomes. More recently, studies have suggested that an epicardial ablation is necessary for improved outcomes after catheter ablation of VT. The overall objective of the present study was to assess the efficacy of radiofrequency catheter ablation (RFA) of VT in ARVD/C, with particular focus on newer ablation strategies, including epicardial catheter ablation.