MR Evaluation of Arrhythmogenic Right Ventricular Cardiomyopathy in Pediatric Patients
Galit Aviram1,2,
Joel E. Fishman1,
Ming-Lon Young3,
Esmail Redha3,
Gurur Biliciler-Denktas3,4 and
Maria M. Rodriguez5
1 Department of Radiology, University of Miami School of Medicine, Jackson
Memorial Hospital, WW279, 1611 N.W. 12th Ave., Miami, FL 33136.
2 Present address: Department of Radiology, Tel-Aviv Medical Center, 6 Weizman
St., Tel Aviv, Israel 64239.
3 Department of Pediatrics, University of Miami School of Medicine, Miami, FL
33136.
4 Present address: Department of Pediatric and Adolescent Medicine, Section of
Pediatric Cardiology, Mayo Clinic, Rochester, MN 55905.
5 Department of Pathology, University of Miami School of Medicine, Miami, FL
33136.
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Fig. 1A. —8-year-old girl with biopsy-proven arrhythmogenic right
ventricular cardiomyopathy. Non–fat-suppressed MR image shows locations
chosen to measure suspected fat (single white arrowhead), normal
muscle (double white arrowheads), and epicardial fat (black
arrowhead). Note heavy trabeculation near right ventricular apex
(arrow).
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Fig. 1B. —8-year-old girl with biopsy-proven arrhythmogenic right
ventricular cardiomyopathy. Fat-suppressed MR image obtained at same location
as A shows no evidence of low signal compatible with fatty
infiltration. Heavy trabeculation is again seen.
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Fig. 2. —Graph shows linear regression of MR imaging findings on
combined non–fat-suppressed, fat-suppressed, and cine sequences with
clinical diagnostic criteria for arrhythmogenic right ventricular
cardiomyopathy (y = 1.58 x –0.16, r = 0.58).
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Fig. 3A. —MR images of right ventricle in 13-year-old boy without
arrhythmogenic right ventricular cardiomyopathy. Both images were obtained at
identical location and gating delay time. Non–fat-suppressed MR image
shows high-signal stripe of epicardial fat (arrows) between
epicardial surface (white arrowhead) and pericardium (black
arrowheads).
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Fig. 3B. —MR images of right ventricle in 13-year-old boy without
arrhythmogenic right ventricular cardiomyopathy. Both images were obtained at
identical location and gating delay time. Fat-suppressed MR image shows lower
contrast between epicardial fat and epicardial surface. Fatty infiltration of
myocardium may be more difficult to assess on fat-suppressed sequence because
of this loss of contrast between tissues.
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Fig. 4. —Arrhythmogenic right ventricular cardiomyopathy in
14-year-old boy with syncope. High intensity signal compatible with fat is
seen in region of anterior right ventricular wall (arrows). High
signal intensity may represent fatty replacement of right ventricular
myocardium or epicardial fat outlining significantly thinned right ventricular
wall.
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Copyright © 2003 by the American Roentgen Ray Society.
