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

David A. Bluemke; Elizabeth A. Krupinski; Theron Ovitt; Kathleen Gear; Evan Unger; Leon Axel; Lawrence M. Boxt; Giancarlo Casolo; Victor A. Ferrari; Brian Funaki; Sebastian Globits; Charles B. Higgins; Paul Julsrud; Martin Lipton; John Mawson; Anders Nygren; Dudley J. Pennell; Arthur Stillman; Richard D. White; Thomas Wichter; Frank Marcus

doi:10.1159/000070672

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

David A. Bluemke, Elizabeth A. Krupinski, Theron Ovitt, Kathleen Gear, Evan Unger, Leon Axel, Lawrence M. Boxt, Giancarlo Casolo, Victor A. Ferrari, Brian Funaki, Sebastian Globits, Charles B. Higgins, Paul Julsrud, Martin Lipton, John Mawson, Anders Nygren, Dudley J. Pennell, Arthur Stillman, Richard D. White, Thomas WichterFrank Marcus

Research output: Contribution to journal › Article › peer-review

177 Scopus citations

Abstract

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 < 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 (χ² = 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 (χ² = 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 (χ² = 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.

Original language	English (US)
Pages (from-to)	153-162
Number of pages	10
Journal	Cardiology
Volume	99
Issue number	3
DOIs	https://doi.org/10.1159/000070672
State	Published - 2003

Keywords

Cardiomyopathy
Diagnosis
Magnetic resonance imaging
Right ventricle

ASJC Scopus subject areas

Cardiology and Cardiovascular Medicine
Pharmacology (medical)

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10.1159/000070672

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Bluemke, D. A., Krupinski, E. A., Ovitt, T., Gear, K., Unger, E., Axel, L., Boxt, L. M., Casolo, G., Ferrari, V. A., Funaki, B., Globits, S., Higgins, C. B., Julsrud, P., Lipton, M., Mawson, J., Nygren, A., Pennell, D. J., Stillman, A., White, R. D., ... Marcus, F. (2003). MR imaging of arrhythmogenic right ventricular cardiomyopathy: Morphologic findings and interobserver reliability. Cardiology, 99(3), 153-162. https://doi.org/10.1159/000070672

Bluemke, DA, Krupinski, EA, Ovitt, T, Gear, K, Unger, E, Axel, L, Boxt, LM, Casolo, G, Ferrari, VA, Funaki, B, Globits, S, Higgins, CB, Julsrud, P, Lipton, M, Mawson, J, Nygren, A, Pennell, DJ, Stillman, A, White, RD, Wichter, T & Marcus, F 2003, 'MR imaging of arrhythmogenic right ventricular cardiomyopathy: Morphologic findings and interobserver reliability', Cardiology, vol. 99, no. 3, pp. 153-162. https://doi.org/10.1159/000070672

@article{77858ab8ed12446cb1f19e80deeaa9b7,

title = "MR imaging of arrhythmogenic right ventricular cardiomyopathy: Morphologic findings and interobserver reliability",

abstract = "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 < 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 (χ2 = 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 (χ2 = 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 (χ2 = 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.",

keywords = "Cardiomyopathy, Diagnosis, Magnetic resonance imaging, Right ventricle",

author = "Bluemke, {David A.} and Krupinski, {Elizabeth A.} and Theron Ovitt and Kathleen Gear and Evan Unger and Leon Axel and Boxt, {Lawrence M.} and Giancarlo Casolo and Ferrari, {Victor A.} and Brian Funaki and Sebastian Globits and Higgins, {Charles B.} and Paul Julsrud and Martin Lipton and John Mawson and Anders Nygren and Pennell, {Dudley J.} and Arthur Stillman and White, {Richard D.} and Thomas Wichter and Frank Marcus",

year = "2003",

doi = "10.1159/000070672",

language = "English (US)",

volume = "99",

pages = "153--162",

journal = "Cardiology",

issn = "0008-6312",

publisher = "S. Karger AG",

number = "3",

}

TY - JOUR

T1 - MR imaging of arrhythmogenic right ventricular cardiomyopathy

T2 - Morphologic findings and interobserver reliability

AU - Bluemke, David A.

AU - Krupinski, Elizabeth A.

AU - Ovitt, Theron

AU - Gear, Kathleen

AU - Unger, Evan

AU - Axel, Leon

AU - Boxt, Lawrence M.

AU - Casolo, Giancarlo

AU - Ferrari, Victor A.

AU - Funaki, Brian

AU - Globits, Sebastian

AU - Higgins, Charles B.

AU - Julsrud, Paul

AU - Lipton, Martin

AU - Mawson, John

AU - Nygren, Anders

AU - Pennell, Dudley J.

AU - Stillman, Arthur

AU - White, Richard D.

AU - Wichter, Thomas

AU - Marcus, Frank

PY - 2003

Y1 - 2003

N2 - 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 < 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 (χ2 = 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 (χ2 = 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 (χ2 = 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.

AB - 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 < 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 (χ2 = 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 (χ2 = 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 (χ2 = 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.

KW - Cardiomyopathy

KW - Diagnosis

KW - Magnetic resonance imaging

KW - Right ventricle

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UR - http://www.scopus.com/inward/citedby.url?scp=0037631917&partnerID=8YFLogxK

U2 - 10.1159/000070672

DO - 10.1159/000070672

M3 - Article

C2 - 12824723

SN - 0008-6312

VL - 99

SP - 153

EP - 162

JO - Cardiology

JF - Cardiology

IS - 3

ER -

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

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