Dilated cardiomyopathy in Noonan's syndrome

CM Yu, LTC Chow, JE Sanderson - International journal of cardiology, 1996 - Elsevier
CM Yu, LTC Chow, JE Sanderson
International journal of cardiology, 1996Elsevier
Hypertrophic cardiomyopathy, with myocardial fibre disarray, may occur in 20–25% of cases
with Noonan's syndrome. However, dilated cardiomyopathy has not previously been
reported. We describe a patient with Noonan's syndrome who presented with typical
features of dilated cardiomyopathy with biventricular enlargement and left ventricular
ejection fraction of 0.16. Endomyocardial biopsy showed focal interstitial fibrosis and fibre
hypertrophy but no disarray. This is the first report in the world literature of an association …
Hypertrophic cardiomyopathy, with myocardial fibre disarray, may occur in 20–25% of cases with Noonan's syndrome. However, dilated cardiomyopathy has not previously been reported. We describe a patient with Noonan's syndrome who presented with typical features of dilated cardiomyopathy with biventricular enlargement and left ventricular ejection fraction of 0.16. Endomyocardial biopsy showed focal interstitial fibrosis and fibre hypertrophy but no disarray. This is the first report in the world literature of an association between Noonan's syndrome and dilated cardiomyopathy. It is possible that this linkage with dilated rather than hypertrophic cardiomyopathy is more common in the Chinese.
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