Abstract
A 32 year old man with no previous medical history suffered a sudden cardiac death. Post mortem examination revealed circumferential fibro-fatty infiltration of the left ventricular myocardium. Histological appearance was characteristic of arrhythmogenic right ventricular dysplasia but unusual for its localisation only to the left ventricle. As a result of this sudden cardiac death the family of the deceased was screened for cardiac disease. A brother of the index case was 36 years old and free of cardiac history and symptoms. Cardiac investigations revealed a functionally and electrically abnormal left ventricle with apparent sparing of the right ventricle. The brothers may have a left sided form of arrhythmogenic ventricular dysplasia and illustrate the importance of screening family members of young victims of sudden cardiac death. Keywords: left sided arrhythmogenic ventricular dysplasia
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Figure 1 .
Case 1. (A) Left and right ventricles (trichrome stain). Cut surface showing a circumferential band of fibrosis (cyan green) (arrowheads) extending between the middle and outer third of the left ventricular myocardium (dark green) and normal appearance of the right ventricular myocardium. (B) Left ventricle (trichrome stain). Portion of left ventricle showing replacement of the outer third of the myocardium with collagenous fibrous tissue (cyan green) (large arrowhead) and adipose tissue (filigree outlining cell borders) (tailed arrow) with scattered islands of residual myocardial fibres (dark green) (small arrowhead).
Figure 2 .
Case 2. Contrast ventriculography. Diastolic (A) and systolic (B) frame of left ventricular contraction showing anterior wall hypokinesis and focal dyskinetic area on inferior surface (arrowhead).