Cardiac Magnetic Resonance
Noninvasive Detection of Myocardial Fibrosis in
Arrhythmogenic Right Ventricular Cardiomyopathy
Using Delayed-Enhancement Magnetic Resonance Imaging
Harikrishna Tandri, MD,* Manoj Saranathan, PHD,† E. Rene Rodriguez, MD,* Claudia Martinez, MD,*
Chandra Bomma, MD,* Khurram Nasir, MBBS,* Boas Rosen, MD,* João A. C. Lima, MD,*
Hugh Calkins, MD,* David A. Bluemke, MD, PHD*†
We evaluated the role of myocardial delayed-enhancement (MDE) magnetic resonance
imaging (MRI) for noninvasive detection of fibrosis in Arrhythmogenic right ventricular
Arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by fibro-fatty
replacement of the right ventricle (RV) leading to arrhythmias and RV failure. Endomyo-
cardial biopsy can demonstrate fibro-fatty replacement of the RV myocardium; however, the
test is invasive and carries a risk of perforation.
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.
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.
Cardiol 2005;45:98–103) © 2005 by the American College of Cardiology Foundation
(J Am Coll
Arrhythmogenic right ventricular dysplasia/cardiomyopathy
(ARVD/C) is characterized by structural and functional
abnormalities of the right ventricular (RV) leading to
ventricular arrhythmias and progressive RV failure. The
most striking morphological feature of ARVD/C is diffuse
or segmental replacement of myocardium in the RV free
wall by fibro-fatty tissue (1–3). Diagnosis of ARVD/C at its
early stages remains a clinical challenge (4,5), more so in
patients with minimal RV abnormalities at echocardio-
graphic or angiographic examination. Endomyocardial bi-
opsy has the potential for in vivo demonstration of typical
fibro-fatty replacement of the RV myocardium. However,
sensitivity of this test is low because, for reasons of safety,
biopsy samples are usually taken from the septum, a region
uncommonly involved by the disease (6).
Magnetic resonance imaging (MRI) is a promising tech-
nique for delineation of RV anatomy and function as well as
for characterizing the composition of the RV wall, especially
with regard to the presence of fatty tissue (7,8). However,
fat visualization on MRI has not been found to be specific
for ARVD (9,10), and there is poor inter-reader agreement
in reporting of fat (11). Recently, myocardial delayed
enhancement (MDE) after intravenous administration of a
gadolinium-based contrast agent has been shown in dys-
functional areas of the left ventricle in patients with prior
myocardial infarction and fibrous scar (12,13). We hypoth-
esized that the RV in ARVD/C patients will demonstrate
increased signal on MDE-MRI because of the fibrotic
nature of the disease process.
The study population included 30 patients who were pro-
spectively evaluated for possible ARVD/C because of either
a family history or left bundle branch block (LBBB)
morphology ventricular arrhythmia. Each of these patients
From the *Division of Cardiology and †Department of Radiology, The Johns
Hopkins University, Baltimore, Maryland. 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.
Manuscript received April 10, 2004; revised manuscript received September 17,
2004, accepted September 21, 2004.
Journal of the American College of Cardiology
© 2005 by the American College of Cardiology Foundation
Published by Elsevier Inc.
Vol. 45, No. 1, 2005
ARVD/C. Consistent with their findings, each of the six
ARVD/C patients who had inducible monomorphic VT on
EP testing demonstrated myocardial delayed enhancement
of the RV. Myocardial delayed enhancement correlated
better than abnormal signal-averaged ECG (5 of 6) or RV
dysfunction (4 of 6) in predicting VT on EP testing. None
of the ARVD/C patients without delayed enhancement was
inducible. The above finding highlights the potential of
MDE-MRI not only to noninvasively visualize scar tissue,
but also to predict the results of EP testing.
In our study, only two-thirds of the ARVD/C patients
showed delayed enhancement. The reason for the lack of
delayed enhancement in the remainder may be the presence
of a pure fatty form of ARVD/C, or may reflect the
insensitivity of current MRI techniques to detect a small
amount of fibrosis in early disease.
Study limitations. For reasons of safety, biopsy specimens
were obtained from an endovascular approach. As such, the
biopsy specimens are representative of subendocardial his-
tology only. The location of the biopsies could not be
systematically correlated with the MRI findings on a one-
to-one basis. The low yield of biopsy (44%) in our study may
in fact reflect sampling error or may also be due to the focal
nature of the disease process. Another important limitation of
our study is the small sample size. Further studies with a large
sample size are needed to confirm our results.
Clinical implications. This study suggests for the first time
that fibrosis of the RV in ARVD/C can be noninvasively
visualized using MRI. The presence of delayed enhancement
and may also be useful in risk stratification. Abnormal RV
enhancement on MRI may help improve the specificity of
MRI for ARVD/C diagnosis. The absence of delayed en-
hancement in each of the patients with idiopathic VT is
reassuring, and consistent with the pathophysiology of ventric-
ular arrhythmias in this disease.
Reprint requests and correspondence: Dr. David A. Bluemke,
MRI Building, Room 143, Department of Radiology, The Johns
Hopkins Hospital, 600 N. Wolfe Street, Baltimore, Maryland
21287. E-mail: firstname.lastname@example.org.
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Figure 4. (A) Correlation between the extent of delayed enhancement and
right ventricular ejection fraction. (B) Correlation between the extent of
delayed enhancement and right ventricular end diastolic volume.
JACC Vol. 45, No. 1, 2005
January 4, 2005:98–103
Tandri et al.
Noninvasive Imaging of Fibrosis in ARVD