BACKGROUND The Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC) was updated in 2010 to improve specificity. There was concern however that the revised cardiovascular magnetic resonance (CMR) criteria was too restrictive and not sensitive enough to detect early forms of the condition. We previously described patients with clinically suspected ARVC who satisfied criteria from non-imaging TFC categories and fulfilled parameters from the original but not the revised CMR criteria; as a result, these patients were not confirmed as definite ARVC but may represent an early phenotype. METHODS Patients scanned between 2008 and 2015 who had either right ventricular (RV) dilatation or regional dyskinesia satisfying at least minor imaging parameters from the original criteria and without contra-indication underwent serial CMR scanning using a 1.5 T scanner. The aims were to assess the risk of progressive RV abnormalities, evaluate the accuracy of the revised CMR criteria and the need for guideline directed CMR surveillance in at-risk individuals. RESULTS Overall, 48 patients were re-scanned; 24 had a first-degree relative diagnosed with ARVC using the revised TFC or a first-degree relative with premature sudden death from suspected ARVC and 24 patients had either left bundle branch morphology ventricular tachycardia or > 500 ventricular extra-systoles in 24-h. Mean follow up was 69+/- 25 months. The indexed RV end-diastolic, end-systolic volumes and ejection fraction were calculated for both scans. There was significant reduction in RV volumes and improvement in RV ejection fraction (EF) irrespective of changes to body surface area; - 11.7+/- 15.2 mls/m2, - 6.4+/- 10.5 mls/m2 and + 3.3 +/- 7.9% (p = 0.01, 0.01 and 0.04). Applying the RV parameters to the revised CMR criteria, two patients from the family history group (one with confirmed ARVC and one with a premature death) had progressive RV abnormalities satisfying major criteria. The remaining patients (n = 46) did not satisfy the criteria and either had normal RV parameters with regression of structural abnormalities (27,56.3%) or stable abnormalities (19,43.7%). CONCLUSION The revised CMR criteria represents a robust tool in the evaluation of patients with clinical suspicion of ARVC, especially for those with ventricular arrhythmias without a family history for ARVC. For patients with RV abnormalities that do not fulfill the revised criteria but have a family history of ARVC or an ARVC associated gene mutation, a surveillance CMR scan should be considered as part of the clinical follow up protocol.

中文翻译：

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对右心室异常和临床怀疑为心律失常的右心室心肌病（ARVC）的患者进行长期CMR随访。

背景技术心律失常性右室心肌病（ARVC）的工作组标准（TFC）在2010年进行了更新，以提高特异性。然而，有人担心修订后的心血管磁共振（CMR）标准过于严格，不够灵敏，无法检测出早期症状。我们先前曾描述过具有临床可疑ARVC的患者，这些患者满足非成像TFC类别的标准，并且满足原始但未修订的CMR标准的参数；结果，这些患者未被确认为明确的ARVC，但可能代表早期表型。方法在2008年至2015年之间扫描的患者，其右心室（RV）扩张或区域性运动障碍至少满足原始标准中的次要成像参数，并且没有禁忌症，使用1.5 T扫描仪进行了连续CMR扫描。目的是评估进行性RV异常的风险，评估修订的CMR标准的准确性以及对高危人群进行指导性CMR监测的必要性。结果共有48例患者被重新扫描; 24例使用修订后的TFC诊断为一级亲属，或疑似ARVC猝死的一级亲属； 24例患者在24小时内左束支形态室性心动过速或室性舒张期> 500。平均随访时间为69 +/- 25个月。带索引的RV舒张末期，两次扫描均计算了收缩末期容积和射血分数。无论体表面积如何变化，RV体积均显着减少，RV射血分数（EF）明显改善。-11.7 +/- 15.2 mls / m2，-6.4 +/- 10.5 mls / m2和+ 3.3 +/- 7.9％（p = 0.01、0.01和0.04）。将RV参数应用到修订的CMR标准中，家族史组的2例患者（1例确诊为ARVC，1例过早死亡）的进行性RV异常符合主要标准。其余患者（n = 46）不符合标准，并且RV参数正常，伴有结构异常的退变（27,56.3％）或稳定的异常（19,43.7％）。结论修订后的CMR标准是评估ARVC临床怀疑患者的有力工具，特别是对于那些没有ARVC家族史的室性心律失常者。对于不符合修订标准但具有ARVC家族史或ARVC相关基因突变的RV异常患者，应将监视CMR扫描视为临床随访方案的一部分。