Relative Utility of Magnetic Resonance Imaging and Right Ventricular Angiography to Diagnose Arrhythmogenic Right Ventricular Cardiomyopathy

INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibrofatty replacement of the RV myocardium. Two imaging techniques used to assess patients suspected of having ARVC are magnetic resonance imaging (MRI) and right ventricular angiography (RVA). Traditionally, RVA has played a central role in the diagnosis of ARVC, but the non-invasive nature of MRI and its unique ability to detect fatty tissue infiltration has increased its popularity as a diagnostic tool. The objective of this study was to assess the relative diagnostic accuracy of MRI and RVA for ARVC. METHODS AND RESULTS: Seventeen patients (9 men, 8 women; ages 42 +/- 17 [range 16-78] years) with documented ventricular arrhythmias were investigated for ARVC. A positive diagnosis of ARVC was based on criteria set forth by the ISFC Working Group on Cardiomyopathies and Dysplasia. ECG-gated spin-echo and gradient-echo MR images in multiple planes and RAO/LAO RV angiograms were compared for diagnostic concordance. Based on working group criteria, 7 patients were diagnosed with ARVC. In ten patients, MRI suggested ARVC. The remaining 7 patients had no MRI findings suggestive of the disease. Four patients with MRI findings of ARVC were incorrectly diagnosed based on Task Force criteria. Conversely, 1 patient with a normal MRI met Task Force criteria for the diagnosis of ARVC. Based on RV angiograms, 7 patients had findings suggestive of ARVC. The 10 patients without AVRD (based on RVA) also did not meet the necessary criteria for diagnosis of ARVC using Task Force standards. RVA was 100% specific and 100% sensitive compared to MRI that was only 86% sensitive and 60% specific. MRI proved to be most reliable when the images demonstrated gross, lipomatous infiltration, evidenced by a large area of hyperintensity. When the results of MRI and RVA were congruent, the diagnosis was always accurate. CONCLUSION: RVA is more sensitive and specific to diagnose ARVC diagnosis than MRI, at least until MRI protocols are better developed. MRI results are most robust when indicators of ARVC are grossly apparent. False-positive diagnosis by MRI was primarily related to perceived motion abnormalities that were not seen by RVA. One of its greatest potential assets (fat detection) did not enhance diagnostic specificity.     

致心律失常性右室心肌病的诊断和治疗

致心律失常性右室心肌病(ARVC)是一种相对少见的原发性心肌疾病,主要临床表现为起源于右室的室性心律失常。由于疾病的异质性导致推荐的诊断标准有较大的局限性。现对该病的诊断和治疗做一简要综述。     

致心律失常性右心室心肌病的诊治与反思

随着近年来对致心律失常性右心室心肌病认识的提高,临床报道逐年增多,但仍有许多问题需要探讨和研究。现就其诊断及治疗方面的问题进行综述。     

Prediction of Life-Threatening Ventricular Arrhythmia in Patients With Arrhythmogenic Cardiomyopathy: A Primary Prevention Cohort Study

Objectives

This study aimed to identify clinical, electrocardiographic (ECG) and cardiac imaging predictors of first-time life-threatening ventricular arrhythmia in patients with arrhythmogenic cardiomyopathy (AC).

致心律失常性右室心肌病的研究进展

致心律失常性右室心肌病或致心律失常性右室发育不良/心肌病是一种主要累及右室的心肌疾病,表现为室性心动过速和猝死。新近研究证明这种疾病不是一种少见疾病,患病率约为1/1000,50%~80%有家族史,是一种常染色体遗传性疾病。1994年的诊断标准导致诊断率较低。目前对诊断指标进行了修改,如胸前导联QRS时限延长。通过改进ECG记录方式可以发现更多的epsilon波。应用新的诊断标准将发现更多的有症状和无症状致心律失常性右室心肌病的患者。基因筛查特别是plakophil-in-2突变筛查将成为疾病重要的早期诊断工具。致心律失常性右室心肌病的治疗应在改变生活方式(包括限制参加竞技运动)的基础上,根据病情应用β阻滞剂、胺碘酮、索他洛尔和/或ICD治疗预防猝死。     

右心室心肌病室性心动过速诊治的新进展

致心律失常右心室心肌病室性心动过速是青壮年猝死的常见疾病,传统的治疗方法是在药物基础上的植入埋藏式自动复律仪治疗;由于我国的经济欠发达状况,普通患者难以负担植入埋藏式自动复律仪,以及导管消融新设备的开发和消融技术的进步,导管消融技术已经是部分患者的一线治疗。现就致心律失常右心室心肌病诊治新进展进行综述。     

Correlation between the Parameters of Signal‐Averaged ECG and Two‐Dimensional Echocardiography in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy

Background: The correlation between parameters of two‐dimensional echocardiography and signal‐averaged ECG (SAECG) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is not known well. Methods: Thirty‐three patients (13 females, 40.3 ± 14.4 years old) were included in this study. Both the right and left ventricular dimensions and systolic function were assessed with two‐dimensional echocardiography. The SAECG was performed with high‐gain amplification and filtered using bidirectional Butterworth filters between 40 and 250 Hz. We evaluated the correlation between the parameters of the SAECG and two‐dimensional echocardiography. Results: The right ventricular (RV) outflow tract was the most frequently (n = 18, 54%) involved segment. Six (18%) patients had only mildly decreased RV systolic function. All the other patients had normal RV systolic function. Although localized left ventricular wall motion abnormalities were observed in 14 (42%) patients, the left ventricular ejection fraction was normal in most (n = 32, 97%). Late potentials were positive in 22 (63%) patients. There was no significant correlation between parameters of the SAECG and two‐dimensional echocardiography for the entire patient population. Conclusions: The SAECG parameters exhibited no correlation to any of two‐dimensional echocardiography parameters in the patients with ARVC. Fragmented electrical activity may develop with no significant relation to the anatomical changes in the patients with ARVC.     

刻不容缓:致心律失常性右室心肌病/发育不良的早期诊断与治疗

致心律失常性右室心肌病/发育不良是一种以纤维-脂肪组织进行性替代右室心肌细胞为特征的遗传性心肌疾病,是引起青少年心源性猝死的主要原因之一。欧洲心脏病协会颁布了最新的诊断标准,使它诊断的敏感性及特异性显著的提高。但心律失常性右室心肌病/发育不良的早期诊断与治疗对于心内科医生来说仍然是一个巨大的挑战。现综述近年来在新标准的基础上对早期诊断以及治疗心律失常性右室心肌病/发育不良的新进展。