DILATED CARDIOMYOPATHY IN NOONANS-SYNDROME

Hypertrophic cardiomyopathy, with myocardial fibre disarray, may occur in 20-25% of cases with Noonan's syndrome. However, dilated cardiomyo pathy has not previously been reported. We describe a patient with Noo nan's syndrome who presented with typical features of dilated cardiomy opathy with biventricular enlargement and left ventricular ejection fr action of 0.16. Endomyocardial biopsy showed focal interstitial fibros is and fibre hypertrophy but no disarray. This is the first report in the world literature of an association between Noonan's syndrome and d ilated cardiomyopathy. It is possible that this linkage with dilated r ather than hypertrophic cardiomyopathy is more common in the Chinese.