(Circulation. 2000;101:825.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Sudden Cardiac Death in Arrhythmogenic Right Ventricular Dysplasia
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 1
)
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 2) revealed onset of
ventricular ectopy and ventricular
tachycardia shortly after midnight, degenerating to
ventricular fibrillation 3 minutes later. Pathological
examination (Figure 3) 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 4) 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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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.