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Circulation. 2000;101:825-827

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(Circulation. 2000;101:825.)
© 2000 American Heart Association, Inc.


Images in Cardiovascular Medicine

Sudden Cardiac Death in Arrhythmogenic Right Ventricular Dysplasia

Shahid Aziz, MB; Ray F. T. McMahon, MD; Clifford J. Garratt, DM

From Manchester Heart Centre, Manchester Royal Infirmary (S.A., C.J.G.), and Department of Pathological Sciences, University of Manchester (R.F.T.M), Manchester, UK.

Correspondence to Clifford J. Garratt, DM, Manchester Heart Centre, Oxford Road, Manchester M13 9WL, UK.

A19-year-old male college student with a 2-month history of palpitations associated with exercise was seen in the clinic. Examination was normal. An ECG (Figure 1Down) showed sinus rhythm with widespread T-wave inversion and ventricular ectopic beats with a left bundle-branch block morphology. The patient was advised to stop sporting activity and underwent Holter monitoring. He was found dead outside his house at 4 AM the following day. Analysis of the ambulatory ECG (Figure 2Down) revealed onset of ventricular ectopy and ventricular tachycardia shortly after midnight, degenerating to ventricular fibrillation 3 minutes later. Pathological examination (Figure 3Down) showed thinning of the right ventricular wall (specimen opened along the tricuspid valve) and replacement by fatty tissue typical of arrhythmogenic right ventricular dysplasia. The photomicrograph (Figure 4Down) is of full-thickness right ventricular free wall with pericardium above and endocardium below. Extensive fatty replacement of muscle can be seen.



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Figure 1.



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Figure 2.



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Figure 3.



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Figure 4.

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.





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Right arrow Articles by Garratt, C. J.
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Right arrow Myocardial cardiomyopathy disease
Right arrow Other diagnostic testing
Right arrow Arrhythmias, clinical electrophysiology, drugs