Sudden Cardiac Death in Arrhythmogenic Right Ventricular Dysplasia
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.
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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