Sen-Chowdhry Srijita, Prasad Sanjay K, Syrris Petros, Wage Ricardo, Ward Deirdre, Merrifield Robert, Smith Gillian C, Firmin David N, Pennell Dudley J, McKenna William J
Cardiology In The Young, The Heart Hospital, University College London, London, United Kingdom.
J Am Coll Cardiol. 2006 Nov 21;48(10):2132-40. doi: 10.1016/j.jacc.2006.07.045. Epub 2006 Oct 31.
We sought to assess the utility of cardiovascular magnetic resonance (CMR) in the evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) in relation to diagnostic criteria and genotype.
Timely diagnosis of ARVC is difficult as clinical findings may be subtle and nonspecific in early disease. The role of CMR is controversial owing to the absence of a standardized protocol, insufficient experience with the modality, and inherent difficulties in imaging the right ventricle.
Comprehensive CMR examination was performed in 232 patients undergoing evaluation for suspected ARVC. CMR outcomes were compared with: 1) prospective clinical diagnosis using Task Force guidelines, with and without the proposed modifications for familial ARVC; and 2) gene-carrier status in 35 individuals from genotyped families.
CMR studies were positive in all 64 patients who prospectively fulfilled Task Force criteria, resulting in 100% sensitivity. Specificity in relation to Task Force criteria was low (29%). Of the 119 apparent false positives detected by CMR, however, 63 fulfilled modified diagnostic criteria for familial ARVC and 7 were obligate gene carriers, suggesting that CMR frequently identifies individuals with early disease, in whom Task Force criteria are relatively insensitive. This was borne out by evaluation of genotyped individuals (26 gene-positive and 9 gene-negative), in whom CMR had a sensitivity of 96% and a specificity of 78%.
CMR is a valuable component of the diagnostic workup for ARVC when performed with a dedicated protocol by specialists with experience in analysis of volumes, right ventricular wall motion, and delayed-enhancement imaging.
我们试图评估心血管磁共振成像（CMR）在根据诊断标准和基因型评估致心律失常性右室心肌病（ARVC）方面的效用。
ARVC的早期诊断较为困难，因为在疾病早期临床症状可能不明显且缺乏特异性。由于缺乏标准化方案、对该检查方式经验不足以及对右心室成像存在固有困难，CMR的作用存在争议。
对232例疑似ARVC患者进行了全面的CMR检查。将CMR检查结果与以下两项进行比较：1）使用工作组指南进行的前瞻性临床诊断，包括有无针对家族性ARVC的拟议修改；2）来自基因分型家族的35名个体的基因携带者状态。
在所有64例前瞻性符合工作组标准的患者中，CMR检查均呈阳性，敏感性达100%。与工作组标准相关的特异性较低（29%）。然而，在CMR检测出的119例假阳性患者中，63例符合家族性ARVC的修改诊断标准，7例为确定的基因携带者，这表明CMR经常能识别出早期疾病患者，而工作组标准对这些患者相对不敏感。对基因分型个体（26例基因阳性和9例基因阴性）的评估证实了这一点，CMR对其敏感性为96%，特异性为78%。
当由在分析容积、右心室壁运动和延迟强化成像方面有经验的专家按照专门方案进行CMR检查时，它是ARVC诊断检查的重要组成部分。
