(Circulation. 1999;99:E8.)
© 1999 American Heart Association, Inc.
Circulation Electronic Pages |
Arrhythmogenic Right Ventricular Dysplasia
Sai Satish Oruganti, MD, DM;
Raghu Cherukupalli, MD, DM;
Kapardhi Lakshmi Narasimha Pannala, MD, DM;
Seshagiri Rao Damera, MD, DM
From the Division of Cardiology, Nizam's Institute of Medical
Sciences, Hyderabad, India.
Correspondence to Dr O. Sai Satish, Assistant Professor, Division of Cardiology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad 500 082, India.
A36-year-old woman was
admitted to a regional cardiac care center elsewhere with a history of
intermittent palpitations and giddiness. On evaluation, the patient was
found to have intermittent ventricular
tachycardia (VT) with hemodynamic
compromise. She required cardioversion several times for termination of
VT. Amiodarone was started for the control of VT but did not
show much benefit. A diagnosis of restrictive
cardiomyopathy involving the right heart was made
on the basis of echocardiographic findings. The patient
was referred to this university hospital for the management of
uncontrolled VT.
At admission to the intensive cardiac care unit, the patient was found
to be hypotensive (blood pressure, 80/60 mm Hg). A 12-lead ECG
showed VT (180 bpm) of left bundle-branch block morphology with
right-axis deviation (Figure 1
). The
patient was cardioverted immediately with 200 J. Subsequent episodes of
VT were controlled with overdrive pacing. The ECG during sinus rhythm
showed a discrete wave (epsilon wave) just beyond the QRS complex and
inverted T waves in the right precordial leads (Figure 2). Transthoracic
echocardiography showed a dilated right atrium (RA)
and right ventricle (RV) with sacculations at the RV apex and a
prominent hyperechoic moderator band. Apical and inferobasal
segments of the RV were hypokinetic. Left ventricular
morphology and contractility were normal (Figure 3). On the basis of the ECG and
echocardiographic findings, a diagnosis of
arrhythmogenic RV dysplasia was considered. A detailed family history
revealed a pattern suggestive of sudden cardiac death at a young age in
2 elder siblings of the patient. Transesophageal
echocardiography with a multiplane probe showed
sacculations in the RV free wall and enlargement of the RV outflow
tract (Figure 4). MRI of the heart showed
a thinned-out RV free wall with sacculations. Electrocardiographically
gated short-TE spin-echo axial MR images showed a signal enhancement in
the RV free wall suggestive of fat deposition in the
myocardium (Figure 5). This
feature is considered to be pathognomonic of arrhythmogenic RV
dysplasia. Endomyocardial biopsy was deferred in
view of the high risk of cardiac perforation in a dilated, thinned-out
RV.
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Figure 2. ECG during sinus rhythm shows inverted T waves in
leads V1 through V5. Arrowheads point to late
RV activation, called an epsilon wave.
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Figure 3. Transthoracic echocardiogram: apical
4-chamber view. Global RA and RV dilatation; thickening and
hyperreflectivity of the moderator band (white arrow); sacculations at
RV apex (black arrows). LA indicates left atrium; LV, left
ventricle.
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Figure 4. Transesophageal echocardiogram:
frontal long-axis 4-chamber view. RV free wall sacculations (black
arrows); prominent moderator band (white arrow). LA indicates left
atrium; LV, left ventricle.
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Figure 5. Spin-echo axial MR image: thinned-out and
sacculated RV free wall with transmural fat deposition (arrows).
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The patient was started on oral sotalol at a dose of 40 mg BID along
with mexiletene 150 mg TID. The dose of sotalol was stepped up to 120
mg BID with tapering of mexiletene. The patient did not experience any
further episodes of VT during the
follow-up.
Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
