Misdiagnosis of Arrhythmogenic right ventricular Dysplasia/Cardiomyopathy
Misdiagnosis of Arrhythmogenic right ventricular Dysplasia/Cardiomyopathy
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DOI：
10.1046/j.1540-8167.2004.03429.x
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发表时间：
2004-03-01
影响因子：
2.7
通讯作者：
Calkins, H
中科院分区：
文献类型：
--
作者：
Bomma, C;Rutberg, J;Calkins, H
ARVD/C误诊。简介：致心律失常性右心室发育不良/心肌病（ARVD/C）的诊断对患者及其一级亲属的管理具有重要意义。诊断是基于国际心肌病工作组提出的一套标准。我们报告我们的经验，在提供一个重新评估的患者谁以前已被诊断为ARVD/C。方法和结果：我们研究了89例患者谁要求重新评估诊断ARVD/C在我们的中心。这些患者在初始评估时均被诊断为ARVD/C。每例患者在我们的中心进行了临床病史、体格检查和无创检测的重新评估。当有临床指征时，进行侵入性检查，包括电生理检查、右心室血管造影和肌内膜活检。在外部机构接受磁共振成像（MRI）的65例患者中，有60例（92%）的MRI异常与ARVD/C一致。在这些患者中，唯一发现的异常是46例患者的心肌内脂肪/壁变薄的定性结果。在重新评价时，这些定性结果未得到证实。这46例患者最终均未被诊断为ARVD/C。在整个患者组中，89例患者中只有24例（27%）符合ARVD/C的工作组标准。结论：本研究表明，ARVD/C的高“误诊”频率是由于过度依赖MRI上心肌内脂肪/壁变薄的存在、诊断测试不完整以及缺乏对工作组标准的认识。ARVD/C的诊断不能仅依赖MRI的定性特征。
ARVD/C Misdiagnosis. Introduction: Diagnosis of arrhythmogenic right ventricular dysplasia/ cardiomyopathy (ARVD/C) has major implications for the management of patients and their first-degree relatives. Diagnosis is based on a set of criteria proposed by the International Task Force for Cardiomyopathies. We report our experience in providing a re-evaluation for patients who previously have been diagnosed with ARVD/C.Methods and Results: We studied 89 patients who requested a re-evaluation for diagnosis of ARVD/C at our center. Each of these patients had been diagnosed with ARVD/C at their initial evaluation. Each patient was re-evaluated with clinical history, physical examination, and noninvasive testing at our center. Invasive testing, which included electrophysiologic testing, right ventricular angiography, and endomyocardial biopsy, was performed when clinically indicated. Sixty (92%) of the 65 patients who had undergone magnetic resonance imaging (MRI) at an outside institution were reported to have an abnormal MRI consistent with ARVD/C. Among these patients, the only abnormality identified was the qualitative finding of intramyocardial fat/wall thinning in 46 patients. On re-evaluation, these qualitative findings were not confirmed. None of these 46 patients ultimately were diagnosed with ARVD/C. Among the entire patient group, only 24 (27 %) of the 89 patients met the Task Force criteria for ARVD/C.Conclusion: This study demonstrates that the high frequency of "misdiagnosis" of ARVD/C is due to over-reliance on the presence of intramyocardial fat/wall thinning on MRI, incomplete diagnostic testing, and lack of awareness of the Task Force criteria. Diagnosis of ARVD/C cannot rely solely upon qualitative features on MRI.