MR Imaging of Arrhythmogenic Right Ventricular Cardiomyopathy: Morphologic Findings and Interobserver Reliability

Uppsala University, Uppsala, Uppsala, Sweden
Cardiology (Impact Factor: 2.18). 06/2003; 99(3):153-62. DOI: 10.1159/000070672
Source: PubMed


Background: Magnetic resonance (MR) imaging is frequently used to diagnose arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). However, the reliability of various MR imaging features for diagnosing ARVC/D is unknown. The purpose of this study was to determine which morphologic MR imaging features have the greatest interobserver reliability for diagnosing ARVC/D. Methods: Forty-five sets of films of cardiac MR images were sent to 8 radiologists and 5 cardiologists with experience in this field. There were 7 cases of definite ARVC/D as defined by the Task Force criteria. Six cases were controls. The remaining 32 cases had MR imaging because of clinical suspicion of ARVC/D. Readers evaluated the images for the presence of (a) right ventricle (RV) enlargement, (b) RV abnormal morphology, (c) left ventricle enlargement, (d) presence of high T1 signal (fat) in the myocardium, and (e) location of high T1 signal (fat) on a Likert scale with formatted responses. Results: Readers indicated that the Task Force ARVC/D cases had significantly more (χ2 = 119.93, d.f. = 10, p 2= 33.98, d.f. = 1, p 2 = 78.4, d.f. = 1, p 2 = 0.9, d.f. = 2, p > 0.05). Conclusions: Reviewers found that the size and shape of abnormalities in the RV are key MR imaging discriminates of ARVD. Subsequent protocol development and multicenter trials need to address these parameters. Essential steps in improving accuracy and reducing variability include a standardized acquisition protocol and standardized analysis with dynamic cine review of regional RV function and quantification of RV and left ventricle volumes.

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Available from: Richard D White, Aug 19, 2014
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    • "Tissue characterization is another advantage of CMR over echocardiography. In arrhythmogenic RV cardiomyopathy (AVRC), MRI can detect dysplastic areas of myocardial thinning, wall motion abnormalities, and fibrofatty replacement; however, fatty infiltration is seen in normal adults and can be obscured by epicardial fat, limiting the utility of MRI in this aspect of diagnosis.[76] Gadolinium enhancement of myocardial fibrosis may be more sensitive than Task Force Criteria for the detection of AVRC carrier-gene status, but is not currently a diagnostic criterion.[7778] "
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    ABSTRACT: Right ventricular (RV) function is a strong independent predictor of outcome in a number of distinct cardiopulmonary diseases. The RV has a remarkable ability to sustain damage and recover function which may be related to unique anatomic, physiologic, and genetic factors that differentiate it from the left ventricle. This capacity has been described in patients with RV myocardial infarction, pulmonary arterial hypertension, and chronic thromboembolic disease as well as post-lung transplant and post-left ventricular assist device implantation. Various echocardiographic and magnetic resonance imaging parameters of RV function contribute to the clinical assessment and predict outcomes in these patients; however, limitations remain with these techniques. Early diagnosis of RV function and better insight into the mechanisms of RV recovery could improve patient outcomes. Further refinement of established and emerging imaging techniques is necessary to aid subclinical diagnosis and inform treatment decisions.
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    • "The results of Fogel et al.43) and our study are in contrast to the results of Aviram et al.44) that demonstrated MRI signals compatible with fat in 70% of 26 patients aged 4-17 years. Considering that detection of intramyocardial fat is hampered by a high interobserver variability32) and fatty replacement of the myocardium is the latest pathological manifestation of loss of myocardium, the high incidence of intramyocardial fat in their pediatric population seems questionable. In our routine MRI protocol (Table 3), approximately one third of the scan time is used for imaging the fat and late gadolinium enhancement. "
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    ABSTRACT: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetically determined disease that progresses continuously from conception and throughout life. ARVC/D manifests predominantly in young adulthood. Early identification of the concealed cases in childhood is of utmost importance for the prevention of sudden cardiac death later in life. Magnetic resonance imaging (MRI) is routinely requested in patients with a confirmed or suspected diagnosis of ARVC/D and in family members of the patients with ARVC/D. Although the utility of MRI in the assessment of ARVC/D is well recognized in adults, MRI is a low-yield test in children as the anatomical, histological, and functional changes are frequently subtle or not present in the early phase of the disease. MRI findings of ARVC/D include morphologic changes such as right ventricular dilatation, wall thinning, and aneurismal outpouchings, as well as abnormal tissue characteristics such as myocardial fibrosis and fatty infiltration, and functional abnormalities such as global ventricular dysfunction and regional wall motion abnormalities. Among these findings, regional wall motion abnormalities are the most reliable MRI findings both in children and adults, while myocardial fibrosis and fat infiltration are rarely seen in children. Therefore, an MRI protocol should be tailored according to the patient's age and compliance, as well as the presence of other findings, instead of using the protocol that is used for adults. We propose that MRI in children with ARVC/D should focus on the detection of regional wall motion abnormalities and global ventricular function by using a cine imaging sequence and that the sequences for myocardial fat and late gadolinium enhancement of the myocardium are reserved for those who show abnormal findings at cine imaging. Importantly, MRI should be performed and interpreted by experienced examiners to reduce the number of false positive and false negative readings.
    Full-text · Article · Aug 2010 · Korean Circulation Journal
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    • "Fibrofatty infiltration in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has been established through both autopsy/biopsy as well as imaging studies [1, 10, 13, 17], yet the ARVD Taskforce guidelines for diagnosis by CMR do not include the presence of fat as criteria [48]. This is due in part to the subjectivity of interpreting fat based on conventional chemical fat suppression imaging [9, 48]. "
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    ABSTRACT: The presence of intramyocardial fat may form a substrate for arrhythmias, and fibrofatty infiltration of the myocardium has been shown to be associated with sudden death. Therefore, noninvasive detection could have high prognostic value. Fat-water-separated imaging in the heart by MRI is a sensitive means of detecting intramyocardial fat and characterizing fibrofatty infiltration. It is also useful in characterizing fatty tumors and delineating epicardial and/or pericardial fat. Multi-echo methods for fat and water separation provide a sensitive means of detecting small concentrations of fat with positive contrast and have a number of advantages over conventional chemical-shift fat suppression. Furthermore, fat and water-separated imaging is useful in resolving artifacts that may arise due to the presence of fat. Examples of fat-water-separated imaging of the heart are presented for patients with ischemic and nonischemic cardiomyopathies, as well as general tissue classification.
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