MR imaging of arrhythmogenic right ventricular cardiomyopathy: Morphologic findings and interobserver reliability

Uppsala University, Uppsala, Uppsala, Sweden
Cardiology (Impact Factor: 2.04). 01/2003; 99(3):153-62. DOI: 10.1159/000070672
Source: PubMed

ABSTRACT 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.
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 T(1) signal (fat) in the myocardium, and (e) location of high T(1) signal (fat) on a Likert scale with formatted responses.
Readers indicated that the Task Force ARVC/D cases had significantly more (chi(2) = 119.93, d.f. = 10, p < 0.0001) RV chamber size enlargement (58%) than either the suspected ARVC/D (12%) or no ARVC/D (14%) cases. When readers reported the RV chamber size as enlarged they were significantly more likely to report the case as ARVC/D present (chi(2)(= )33.98, d.f. = 1, p < 0.0001). When readers reported the morphology as abnormal they were more likely to diagnose the case as ARVC/D present (chi(2) = 78.4, d.f. = 1, p < 0.0001), and the Task Force ARVC/D (47%) cases received significantly more abnormal reports than either suspected ARVC/D (20%) or non-ARVC/D (15%) cases. There was no significant difference between patient groups in the reported presence of high signal intensity (fat) in the RV (chi(2) = 0.9, d.f. = 2, p > 0.05).
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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    • "Cardiac MRI is a non invasive imaging modality which can be perfectly customized for each patient; furthermore with the increased time and spatial resolutions, it provides good images for a complete overview of the right ventricle (RV) [1]. In facts it allows an anatomic, functional and morphologic approach, so that it is possible to suspect several disorders despite the complex crescent shape of the RV. "
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    ABSTRACT: The aim of this study was to gain a wide perspective of the arrhythmogenic right ventricular dysplasia (ARVD) by developing algorithms for Cardiac Magnetic Resonance Imaging. We developed a semi-automatic procedure to assess the Right Ventricle (RV) volumes and to quantify RV wall motion; moreover, with the increased visible details in a single MR image, a manual method to evaluate the trabeculae mass was performed. All the algorithms used were based on the level set theory which allows detecting both endocardial and wall surfaces, as well as the black parts characterizing the trabeculae. 6 normal subjects and 6 subjects with ARVD have been investigated. Our method and the standard manual method for volume estimation were significantly correlated (y=0.92×+6.56), (r=0.92 p
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    • "The MR imaging protocol in right ventricular cardiomyopathy is aimed at recognizing two important aspects of the disease process: 1) global and regional right ventricular morphology, 2) global and regional right ventricular dysfunction, and 3) fibrofatty infiltration of the right ventricle. Hence the protocol includes 1) bright-blood cine imaging to visualize right ventricular global and regional function, and 2) black-blood imaging to identify intramyocardial fatty infiltration (Bluemke et al., 2003; Castillo et al., in press). The optimal protocol for ARVC/D is directly proportional to the scan time (duration of the scan), and in our experience it is around 45 minutes to 1 hour. "
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    ABSTRACT: Magnetic Resonance Imaging (MRI) is currently considered as the noninvasive modality of choice for evaluation of patients with suspected Arrhythmogenic Right Ventricular Dysplasia (i.e., right ventricular dysplasia). As arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) it is included in the WHO classification of cardiomyopathies. It has the unique ability to provide tissue characterization in addition to providing functional information. This article presents the basic techniques for the evaluation of right ventricular cardiomyopathy.
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    ABSTRACT: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV). In ARVD/C there is progressive replacement of right ventricular myocardium with fatty and fibrous tissue. The precise prevalence of ARVD/C has been estimated to vary between 1 in 1000 and 1 in 5000 of the general population. The purpose of this chapter is to review the current understanding of ARVD/C and its management. Particular attention will be focused on some of the recent advances in the understanding of the genetic basis of ARVD/C and how this information provides insight into the mechanism of ventricular arrhythmias that can cause sudden death.
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