BACKGROUND Desmosomal changes, electric uncoupling, and surviving myocardial bundles in fibrofatty tissue characterize arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Resultant activation delay is pivotal for reentry and thereby ventricular tachycardia (VT). Current task force criteria (TFC) for diagnosis have limited sensitivity. The aim of this study was to assess the diag...

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 def...

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy associated with cardiac arrhythmias originating in the right ventricle, heart failure, and sudden cardiac death. Development of ARVD/C type 1 has been attributed to differential expression of transforming growth factor beta 3 (TGF β 3). Several mechanisms underlying the molecular basis of ARVD/C ty...

Patients with cardiac sarcoidosis may present with clinical and morphological features similar to arrhythmogenic right ventricular dysplasia (ARVD) or cardiomyopathy (ARVC). Three cases of cardiac sarcoidosis are presented that clinically mimicked ARVD or ARVC until a pathology diagnosis of sarcoidosis was made at biopsy or autopsy. A diagnostic distinction, while often difficult to make, is im...

pathology affecting multiple sites of the right ventricle. However the predictive value of this finding is not 100% accurate. Early in the disease of ARVD/C one may see a single form of VT. There has also been a report of multiple forms of VT seen in an idiopathic VT, but the diagnosis in these rare instances must be carefully scrutinized. Niroomand et al. (2002) performed electrophysiology stu...

OBJECTIVES The purpose of this study was to evaluate whether electrocardiographic characteristics of ventricular arrhythmias distinguish patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) from those with right ventricular outflow tract tachycardia (RVOT-VT). BACKGROUND Ventricular arrhythmias in RVOT-VT and ARVD/C-VT patients can share a left bundle branch block/...

AIMS Radiofrequency catheter ablation is considered first line treatment for symptomatic patients with right ventricular outflow tract tachycardia (RVOT). The role of ablation in arrhythmogenic right ventricular dysplasia (ARVD) is more limited. As such, differentiating between the two conditions is essential. METHODS AND RESULTS This study compared non-invasive findings, magnetic resonance i...

Arrhythmogenic right ventricular dysplasia (ARVD) is a recently described entity characterized by right ventricular myopathic changes and right ventricular tachycardia. The presence or extent of left ventricular dysfunction in ARVD is not known. We assessed right ventricular and left ventricular function and size in six patients with ARVD by echocardiography and radionuclide angiocardiography d...

• Atherosclerotic renovascular stenosis is a potentially progressive disease. • Risk factors for progressive stenosis and renal artery occlusion include: – uncontrolled systolic hypertension (>160 mmHg) – diabetes mellitus – high grade (>70%) ipsilateral and contralateral atherosclerotic renal vascular disease (ARVD), and – significant baseline proteinuria. • Risk factors for atrophy include: –...

Arrhythmogenic right ventricular dysplasia (ARVD) is a familial cardiomyopathy that causes fibro-fatty replacement of the right ventricle (RV), leading to sudden death due to ventricular arrhythmias. The disease is an important cause of sudden death in individuals younger than 35 years of age. Structural and functional abnormalities of the RV constitute an important diagnostic criterion for the...