Arrhythmogenic Right Ventricular Dysplasia
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.
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.
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.
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