Dilated cardiomyopathy in Noonan's syndrome

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 wit...

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Veröffentlicht in:International journal of cardiology 1996-09, Vol.56 (1), p.83-85
Hauptverfasser: Yu, C.M., Chow, L.T.C., Sanderson, J.E.
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container_title International journal of cardiology
container_volume 56
creator Yu, C.M.
Chow, L.T.C.
Sanderson, J.E.
description 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.
doi_str_mv 10.1016/0167-5273(96)02713-1
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Joint deformations</topic><topic>Medical sciences</topic><topic>Noonan Syndrome - complications</topic><topic>Noonan Syndrome - diagnosis</topic><topic>Noonan Syndrome - physiopathology</topic><topic>Noonan's syndrome</topic><topic>Ventricular Dysfunction, Left - complications</topic><topic>Ventricular Dysfunction, Left - diagnosis</topic><topic>Ventricular Dysfunction, Left - physiopathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Yu, C.M.</creatorcontrib><creatorcontrib>Chow, L.T.C.</creatorcontrib><creatorcontrib>Sanderson, J.E.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>International journal of cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Yu, C.M.</au><au>Chow, L.T.C.</au><au>Sanderson, J.E.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Dilated cardiomyopathy in Noonan's syndrome</atitle><jtitle>International journal of cardiology</jtitle><addtitle>Int J Cardiol</addtitle><date>1996-09-01</date><risdate>1996</risdate><volume>56</volume><issue>1</issue><spage>83</spage><epage>85</epage><pages>83-85</pages><issn>0167-5273</issn><eissn>1874-1754</eissn><coden>IJCDD5</coden><abstract>Hypertrophic cardiomyopathy, with myocardial fibre disarray, may occur in 20–25% of cases with Noonan's syndrome. 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subjects Adult
Biological and medical sciences
Biopsy
Cardiomyopathy, Dilated - complications
Cardiomyopathy, Dilated - diagnosis
Cardiomyopathy, Dilated - physiopathology
Dilated cardiomyopathy
Diseases of the osteoarticular system
Echocardiography, Doppler, Pulsed
Hemodynamics
Humans
Male
Malformations and congenital and or hereditary diseases involving bones. Joint deformations
Medical sciences
Noonan Syndrome - complications
Noonan Syndrome - diagnosis
Noonan Syndrome - physiopathology
Noonan's syndrome
Ventricular Dysfunction, Left - complications
Ventricular Dysfunction, Left - diagnosis
Ventricular Dysfunction, Left - physiopathology
title Dilated cardiomyopathy in Noonan's syndrome
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