Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) presenting as congestive heart failure, ventricular tachycardia, and right atrial mass in a young male: role of echocardiography in connecting the missing link.

摘要

Arrhythmogenic right ventricular displa-sia/cardiomyopathy (ARVD/C) is predominantly a genetically determined cardiac muscle disorder characterized by fibrofatty replacement of the right ventricular (RV) myocardium. Though a definitive diagnosis of ARVD/C is based on his-tological demonstration of transmural fibrofatty replacement of RV myocardium at biopsy, two-dimensional echocardiography, by virtue of its widespread availability, low cost, and ease of performance and interpretation, remains one of the major tools for establishing the diagnosis of ARVC.Despite presence of RV dilatation, dysfunction, and even aneurysms in patients with ARVD/C, and the consequent milieu for formation of thrombi, reports of RV mural thrombi in ARVC are rare Even rarer is development of RA thrombi in patients with ARVC especially in sinus rhythm.