Clinical research
Cardiac magnetic resonance
Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging

https://doi.org/10.1016/j.jacc.2004.09.053Get rights and content
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Objectives

We evaluated the role of myocardial delayed-enhancement (MDE) magnetic resonance imaging (MRI) for noninvasive detection of fibrosis in Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).

Background

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by fibro-fatty replacement of the right ventricle (RV) leading to arrhythmias and RV failure. Endomyocardial biopsy can demonstrate fibro-fatty replacement of the RV myocardium; however, the test is invasive and carries a risk of perforation.

Methods

Thirty consecutive patients were prospectively evaluated for ARVD/C. Magnetic resonance imaging was performed on a 1.5-T scanner. Ten minutes after intravenous administration of 0.2 mmol/kg of gadodiamide, MDE-MRI was obtained. Diagnosis of ARVD/C was based upon the Task Force criteria and did not include MRI findings.

Results

Twelve (40%) of 30 patients met the Task Force criteria for ARVD/C. Eight (67%) of the 12 ARVD/C patients demonstrated increased signal on MDE-MRI in the RV compared with none (0%) of the 18 patients without ARVD/C (p < 0.001). Endomyocardial biopsy was performed in 9 of the 12 ARVD/C patients. Of the nine patients, four had fibro-fatty changes consistent with the diagnosis of ARVD/C. Each of these patients had increased RV signal on MDE-MRI. None of the patients without ARVD/C had any abnormalities either on histopathology or on MDE-MRI. Electrophysiologic testing revealed inducible sustained ventricular tachycardia (VT) in six of the eight ARVD/C patients with delayed enhancement, compared with none of the ARVD/C patients without delayed enhancement (p = 0.01).

Conclusions

Noninvasive detection of RV myocardial fibro-fatty changes in ARVD/C is possible by MDE-MRI. Magnetic resonance imaging findings had an excellent correlation with histopathology and predicted inducible VT on programmed electrical stimulation, suggesting a possible role in evaluation and diagnosis of patients with suspected ARVD/C.

Abbreviations and acronyms

ARVD/C
arrhythmogenic right ventricular dysplasia/cardiomyopathy
CNR
contrast-to-noise ratio
ECG
electrocardiogram
EP
electrophysiologic
FOV
field of view
LBBB
left bundle branch block
MDE
myocardial delayed enhancement
MRI
magnetic resonance imaging
ROI
region of interest
RV
right ventricle
RVEDV
right ventricular end diastolic volume
RVEF
right ventricular ejection fraction
RVOT
right ventricular outflow tract
VT
ventricular tachycardia

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The Johns Hopkins ARVD program is funded by a private grant from the Bogle Foundation and the National Institutes of Health Research Grant 1 UO1 HL65594-01A1.