(Circulation. 1999;99:E8.)
© 1999 American Heart Association, Inc.
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Arrhythmogenic Right Ventricular Dysplasia
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