Evaluation and course of an unusual case of arrhythmogenic right ventricular
Jan Fritz, Harikrishna Tandri, E. Rene Rodriguez, Hugh Calkins & David A. Bluemke
Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of
Medicine, Baltimore, MD, USA
Received 30 June 2005; accepted in revised form 16 July 2005
thy, magnetic resonance imaging, right ventricle
arrhythmogenic right ventricular dysplasia, arrhythmogenic right ventricular cardiomyopa-
We report a case of a 42-year-old Caucasian man who presented with isolated right ventricular failure and
atrial fibrillation without ventricular arrhythmia. In this report, we describe accurate evaluation by MR
imaging confirmed by histopathologic findings as well as imaging progression of this unusual case of
arrhythmogenic right ventricular dysplasia.
(ARVD) is a cardiomyopathy that is histopatho-
logically characterized by fatty and fibrofatty
replacement of the right ventricular myocardium
. Clinical features of arrhythmogenic right ven-
tricular dysplasia are progressive right heart fail-
Diagnosis of ARVD at early stages remains a
clinical challenge . In the diagnostic follow-up
MR imaging may be used to characterize the
morphologic and functional changes in this dis-
order. Fat infiltration of the right ventricle may be
identified by black blood MR imaging [3, 4] but
the reliability of this finding has not been verified
[5, 6]. Right ventricular enlargement or focal
aneurysms may be identified on MR cine images.
In addition fibrosis of the myocardium may
In this report, we describe the diagnostic work-
up as well as MR imaging findings in a patient
presenting with an atypical form of ARVD, con-
sisting of progressive right sided failure with late
onset of non-sustained ventricular tachycardia.
A 42-year-old Caucasian male patient with no
prior history of cardiac symptoms presented with
shortness of breath, nocturnal dyspnea, pedal
edema and a deterioration of his functional class
over a period of 2 years. His medical history was
unremarkable, except for a borderline high blood
The International Journal of Cardiovascular Imaging (2005)
? Springer 2005
Physical examination was notable for a blood
pressure of 128/92 mm of Hg. His heart rate was
irregular at 40–50 beats per minute (bpm). The
jugular venous pressure was approximately 20 cm
of H2O with absent ‘a’ waves. Carotids were nor-
mal without bruits. Cardiac examination revealed
a prominent right ventricular heave. Cardiac aus-
cultation revealed a normal S1 and a widely split
S2 which varied with respiration without any
significant murmurs. The lung fields were clear to
auscultation. The abdomen was soft and non-
tender with pulsatile hepatomegaly. The extremi-
ties were notable for a for 1+ pitting edema with
normal peripheral pulses.
Chest X-ray showed enlargement of cardiac
silhouette with clear lung fields. ECG revealed
atrial fibrillation with a ventricular rate of 40 bpm,
normal axis and a wide QRS consistent with right
bundle branch block. Echocardiography demon-
strated normal left ventricular function, massive
enlargement of the right atrium, moderate right
ventricular enlargement and dysfunction, mild to
moderate tricuspid regurgitation. The right ven-
tricular systolic pressure was within normal limits.
A VQ scan showed low probability of pulmo-
nary thrombo-embolism and a chest CT scan
showed no evidence of parenchymal or interstitial
lung disease. Cardiac MR images were obtained
on a 1.5 T scanner (CV/i, General Electric Medical
Systems, Waukesha, WI). On ECG-gated cine MR
images (steady state free precession SSFP pulse
sequence, FIESTA?, General Electric Medical
Systems, Waukesha, WI) there was diffuse global
hypokinesis of the right heart. There was no
evidence of regional contraction abnormalities.
The right ventricular ejection fraction was 25%.
The right atrial diameter was 9.3 cm and right
ventricle diameter was 6.5 cm. There was also
enlargement of the right ventricular outflow tract
and moderate tricuspid regurgitation. The left
heart chambers were normal in size with left atrial
diameter of 3.6 cm and left ventricular diameter of
4.3 cm with ejection fraction of 55%. There was
paradoxical motion of the interventricular septum
consistent with increased right heart pressures or
right bundle branch block. On T1-weighted images
(fast spin echo sequence, TR/TE 845/5.5) there
was no evidence of abnormal increased signal
intensity that would otherwise correspond to fat in
the anterior wall of the right ventricle (Figure 1a).
Myocardial delayed enhancement images (inver-
sion recovery prepared fast gradient echo blood
obtained approximately 15 min following the
administration of0.2 mmol/kg
gadodiamide contrast (General Electric Health
Systems, Princeton, NJ). Delayed myocardial
images indicated enhancement of the anterior and
inferior right ventricular wall compatible with
fibrosis or inflammation (Figure 1b).
Invasive testing was undertaken to confirm the
results of non-invasive testing. Cardiac catheteri-
zation demonstrated no left to right shunt, normal
left sided filling pressures with elevated right heart
filling pressures. Right ventriculography demon-
strated globally hypokinetic dilated right ventricle
with no focal aneurysms or regional dysfunction.
Endomyocardial biopsy taken from the myocar-
dial border of the interventricular septum revealed
mild to moderate fibrosis with only few focal areas
of adipose tissue infiltration (Figure 1c) consistent
with the diagnosis of ARVD.
Electrophysiologic testing revealed no induc-
stimulation even with Isoproterenol. The patient
was treated conservatively with diuretics and
digoxin for right sided heart failure and ICD
therapy was deferred due to the lack of ventric-
One year later, a 24-h Holter recording revealed
bardycardia with a mean heart rate of 53 bpm
varying from 37 to 151 bpm. There were occa-
sional multiform isolated ventricular complexes
and runs of ventricular tachycardia. The longest
and fastest run was 9 beats at a heart rate of
134 bpm (Figure 1d).
progression of enlargement of the right atrium to
9.5 cm and enlargement of the right ventricle to
7.3 cm. The left heart chambers were unremark-
able. In view of the progression of right ventricular
disease and the non-sustained ventricular tachy-
cardia an implantable defibrillator was placed to
prevent sudden death .
Diagnosis of arrhythmogenic right ventricular
dysplasia is made according to major and minor
criteria proposed by the Task Force of the
Working Group on Cardiomyopathies . Criteria
most commonly observed in ARVD are ventricu-
lar arrhythmia, ECG repolarization changes and
global or regional right ventricular dysfunction as
well as morphologic abnormalities of the right
ventricular free wall . However, patients may
be asymptomatic or symptoms may be limited to
infrequent palpitations and occasional syncope.
MR imaging has evolved to a promising tool for
evaluation of ARVD [11, 12]. In addition to highly
accurate functional information about the right
ventricle it provides unique information about
myocardial characteristics, especially in differenti-
ation of fatty and muscular tissue  that has been
reported to be strongly associated with ARVD .
On T1-weighted MR-images fat appears with high
signal intensity, whereas myocardium appears with
intermediate signal intensity. However, these MR
imaging findings were not considered as part of the
Task Force criteria for ARVD  because of
limited experience with MR imaging .
Figure 1. 42-year-old Caucasian man with atypical clinical presentation of ARVD: (a) T1-weighted MR image (fast spin echo se-
quence, TR/TE 845.1/5.5) showing no evidence of increased signal intensity (fat) in the anterior right ventricular wall. (b) Myocardial
delayed enhancement images (inversion recovery prepared fast gradient echo blood suppression sequence, TR/TE 5.3/1.3) revealed
enhancement of the anterior and inferior right ventricular wall consistent with myocardial fibrosis. The small image outlines the
enhancement. (c) Light micrograph stained for collagen (Masson trichrome, Calibration bar=100 lm) showing hypertrophied
myocardium with abundant loose (arrowheads) and dense (arrows) collagen fiber bundles. These bundles are visible in the interstitial
space, separating the myocytes. Note the absence of adipose tissue cells. (d) 24 h Holter monitor indicating a run of ventricular
tachcardia of 9 beats.
Following the initial evaluation, in our patient
two major criteria of the Task Force criteria for
ARVD were met: severe
dilatation in combination with reduced ejection
fractionas well as
fibrofatty infiltration of the right ventricular
myocardium. However, our patient is unique in
that common clinical manifestations seen in
patients presenting with ARVD were initially not
present . There was no spontaneous or inducible
ventricular tachycardia, absence of fat signal on
T1-weighted MR images, as well as no evidence of
right ventricular regional contraction abnormality.
The histopathologic finding of only few fatty
infiltrates in the right ventricular myocardium
were consistent with the absence of fat signal on
T1-weighted MR images. In addition, findings on
delayed enhancement MR images correlated with
the diffuse mild to moderate fibrosis of the right
delayed enhancement may be seen in other
conditions of the right ventricle such as in
inflammation , it is a promising tool for the non-
Recent reports suggest not only a roll in the
diagnostic work-up but also in risk stratification
due to the association of delayed enhancement and
inducibility during electrophysiologic testing [14,
In this case, MR imaging findings in delayed
myocardial enhancement and T1-weighted MR
images were consistent with the diagnosis of
fibrofatty variant of ARVD confirmed by patho-
tachyarrhythmias on initial presentation a multi-
disciplinary diagnostic approach including cardiac
MR imaging, electrophysiology and histopathol-
ogy revealed the diagnosis of ARVD.
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Address for correspondence: David A. Bluemke, Russell H.
Morgan Department of Radiology and Radiological Science,
Johns Hopkins University School of Medicine, 600 N. Wolfe
street MRI Rm 143, Baltimore, MD, 21287, USA
Tel.: +1-410-955-4062; Fax: +1-410-955-9799