Hein W. M. Kayser, MD ; Ernst E. van der Wall, MD ; Mohan U. Sivananthan, MD ; Sven Plein, MD ; Timothy N. Bloomer, MD ; Albert de Roos, MD
1 From the Departments of Radiology (H.W.M.K., E.E.v.d.W., A.d.R.) and Cardiology (E.E.v.d.W.), Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht (H.W.M.K., A.d.R.); and Cardiac MRI Unit, General Infirmary at Leeds, England (M.U.S., S.P., T.N.B.). Received May 11, 2001; revision requested July 5 and received January 21, 2002; accepted January 21.
Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disorder of primarily the right ventricle, with unknown cause and prevalence and with a frequent familial occurrence. The typical clinical manifestation consists of ventricular arrhythmias with a left bundle branch block (LBBB) pattern that occur predominantly in young adults. ARVD may result in sudden death. Other manifestations are electrocardiographic repolarization and depolarization changes, structural abnormalities that range from subtle wall aneurysms within the so-called “triangle of dysplasia” to biventricular regional or global dysfunction, and localized or widespread fibrofatty infiltration of the right ventricular myocardium. The diagnosis of ARVD is based on the presence of major and minor criteria encompassing genetic, electrocardiographic, pathophysiologic, and histopathologic factors. The imaging modalities used to evaluate right ventricular abnormalities include conventional angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and magnetic resonance (MR) imaging. Among these techniques, MR imaging allows the clearest visualization of the heart. Because MR imaging depicts both functional and structural abnormalities, positive MR imaging findings should be used as important additional criteria in the clinical diagnosis of ARVD. MR imaging appears to be the optimal technique for detection and follow-up of clinically suspected ARVD.