Update and modification of Task Force Criteria for ARVD/C

描述（由申请人提供）： 致心律失常性右心室发育不良/心肌病（ARVD/C）是一种以存在源自右心室的室性心律失常为特征的疾病，包括室性早搏、室性心动过速或猝死。随着疾病的进展，右心衰竭和左心衰竭可能会发展。 当患者具有典型的临床特征，包括ECG改变和仅限于右心室的形态异常时，诊断容易且正确。由于右心室结构和收缩模式是不对称的，因此很难区分这种复杂心室腔的正常和轻度异常。为了解决这些不确定性，1993年成立了一个工作组，提出ARVD/C的诊断标准。在1994年发表的报告中，工作组一致认为，没有一种单一的测试方法具有足够的特异性和敏感性来确定诊断。因此，选择了主要和次要标准，并建议应使用这些标准的组合来诊断ARVD/C。这一工作框架是标准化诊断的一个重大进步，但由于标准模糊或基于定性而非定量信息，它显然具有重大局限性。例如，没有定量限制来区分轻度与中度或更严重的整体右心室功能损害。现在有必要消除主观评估，并确定工作队标准的量化方面，以更好地确定这种疾病的存在。我们建议召开一次为期两天的会议，通过召集ARVD/C原始工作组的几名成员以及对ARVD/C有重大兴趣的成像方式、心电图和病理学方面的现有专家，来推进这种疾病诊断的临床管理和科学进展。拟议会议的目的是：1）修改和修订工作组诊断致心律失常性右心室发育不良/心肌病（ARVD/C）的标准，2）纳入定量标准，3）消除定性异常的主观和定性描述。 将寻求对参考的正常值和参数或临界值达成共识，作为在超声心动图、血管造影和MR I的各种成像模式中使用的轻度、中度和重度异常的正常值的标准差，并纳入工作组标准。 这一标准化将有助于ARVD/C的诊断，提供更明确的表型，并将纳入评估右心室结构和功能的新技术。修订后的工作队标准将在同行评审期刊上发表。本次会议更新和修改工作组标准将加强未来的研究，以及正确诊断疾病和防止遗传性遗传性心律失常和心肌病误诊的能力。 null

DESCRIPTION (provided by applicant): 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. null