? Arrhythmogenic Right Ventricular Dysplasia /Cardiomyopathy (ARVD/C) is a disease characterized by a presence of ventricular arrhythmias originating from the right ventricle including ventricular premature beats, ventricular tachycardia or arrhythmic death. As the disease progresses, right and left heart failure can develop. When patients have typical clinical features including ECG changes and morphological abnormalities limited to the right ventricle, the diagnosis is readily and correctly diagnosed. Since the right ventricular structure and contractile pattern is asymmetrical it can be difficult to differentiate normal from mild abnormalities of this complex ventricular chamber. To address these uncertainties a Task Force was assembled in 1993 to propose diagnostic criteria for ARVD/C. When published in 1994, the Task Force agreed that there was no single test sufficiently specific and sensitive to establish the diagnosis. Therefore, major and minor criteria were selected and it was proposed that the diagnosis of ARVD/C should be made using combinations of these criteria. This working framework was a major advance to standardizing the diagnosis, but it has become evident that it has significant limitations, due to the fact that criteria are vague or based on qualitative rather than quantitative information. For example, there are no quantitative limits to separate mild from moderate or more severe impairment of global right ventricular function. It is now necessary to eliminate subjective assessment and to define quantitative aspects for the Task Force Criteria to better determine the presence of this disease. We propose a two day conference to advance clinical management and scientific progress in the diagnosis of this disease by bringing together several members from the original Task Force on ARVD/C, and current experts in the imaging modalities, electrocardiography and pathology who have a major interest in ARVD/C. The objectives of the proposed conference are: 1) The modification and revision of the Task Force Criteria for the diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), 2) Incorporation of quantitative standards and 3) To eliminate the subjective and qualitative description of qualifying abnormalities. A consensus will be sought on the normal values to be referenced and the parameters or cutoffs as standard deviations from the normal values for mild, moderate and severe abnormalities to be used within the various imaging modalities of echocardiography, angiography and MR I and incorporated into the Task Force Criteria. This standardization will aid the diagnosis of ARVD/C, provide a more defined phenotype and will incorporate the newer technologies for assessing the structure and function of the right ventricle. The revised Task Force Criteria will be published in a peer reviewed journal. This conference to update and modify the Task Force Criteria will enhance future research, and the ability to correctly diagnose the disease and prevent misdiagnosis of an inheritable genetic arrhythmia and cardiomyopathy. ? ? ? ?
| Marcus, Frank; Basso, Cristina; Gear, Kathleen et al. (2010) Pitfalls in the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. Am J Cardiol 105:1036-9 |
| Marcus, Frank I; McKenna, William J; Sherrill, Duane et al. (2010) Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation 121:1533-41 |
