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(Circulation. 2002;105:1394.)
© 2002 American Heart Association, Inc.

Images in Cardiovascular Medicine

Left Ventricular Involvement in Arrhythmogenic Right Ventricular Cardiomyopathy

J.A. McCrohon, MD, PhD; A.S. John, MD; C.H. Lorenz, PhD; S.W. Davies, MD; D.J. Pennell, MD

From the Departments of Cardiovascular Magnetic Resonance (J.A.M., A.S.J., C.H.L., D.J.P.) and Cardiology (S.W.D.), Royal Brompton Hospital, London, United Kingdom.

Correspondence to Dr J.A. McCrohon, Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, Sydney Sreet, London, UK SW3 6NP. E-mail jane_mccrohon{at}hotmail.com

A 55-year-old woman presented with an 18-month history of progressive dyspnoea, exertional chest tightness, and palpitations associated with light-headedness. Resting electrocardiogram showed sinus rhythm and incomplete right bundle branch block with nonsustained ventricular tachycardia of left bundle branch morphology on 24-hour recording. Thallium scintigraphy reported partial thickness infarction of the inferior wall and reversible ischemia in the anterior wall. Cardiac catheterization showed "disorganized" function of the left ventricle (LV) and severe hypokinesis of the inferior wall and mid-anterior wall. The left coronary system was normal, and the right coronary showed a 50% distal stenosis. Cardiovascular magnetic resonance (CMR) assessment confirmed an enlarged LV with severe hypokinesis of the inferior wall and hypokinesis and focal thinning of the mid anterior wall. The right ventricle (RV) was also abnormal with reduced systolic function, elevated end-systolic volume, dyscoordinate free wall contraction, and an area of prominent dyskinesis in the basal to mid inferolateral RV (Figure 1). T1-weighted spin-echo images showed fat deposits within the myocardium of the anterior and inferior LV walls and throughout the RV free wall with marked thinning in the areas of dyskinesis (Figure 2). There was no LV or RV myocardial infarction or macroscopic fibrosis on late enhancement imaging after gadolinium. These features are consistent with arrhythmogenic right ventricular cardiomyopathy with florid LV involvement. Fatty subepicardial deposits of the LV have been documented at autopsy in 64% of arrhythmogenic right ventricular cardiomyopathy cases but are rarely so clearly seen in life. The patient has been referred for electrophysiological testing and investigation of first-degree relatives.

View larger version (107K): [in this window] [in a new window]   Figure 1. TrueFISP cine images in the horizontal and vertical long-axis (A and B) and short-axis planes (C) showing a dilated left ventricle with arrows demonstrating mid-anterior and inferior wall hypokinesis and RV free wall dyskinesis and associated wall thinning. S indicates systole; D, diastole.

View larger version (91K): [in this window] [in a new window]   Figure 2. T1-weighted turbo spin-echo images before (upper panel) and after (lower panel) the application of a fat saturation pulse over the region of interest. The arrows (upper panel) point to areas of fatty infiltration of the anterior and inferior myocardium from the epicardial surface. Fatty infiltration is confirmed by the nulling of these areas (black regions) using a fat saturation prepulse in the same plane. The RV free wall shows diffuse high signal consistent with fat (C, top), which is nulled using a fat suppression sequence (C, bottom) to reveal a remaining thin layer of RV myocardium. FS indicates fat saturation.

Acknowledgments

Dr McCrohon is supported by the Marian Silcock Cardiac Fellowship, CORDA, UK

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 the Circulation Editorial Office, St Luke's Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

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This Article Extract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by McCrohon, J.A. Articles by Pennell, D.J. Search for Related Content PubMed PubMed Citation Articles by McCrohon, J.A. Articles by Pennell, D.J. Related Collections Structure Imaging Myocardial cardiomyopathy disease CT and MRI