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 |
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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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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.</description><identifier>ISSN: 0167-5273</identifier><identifier>EISSN: 1874-1754</identifier><identifier>DOI: 10.1016/0167-5273(96)02713-1</identifier><identifier>PMID: 8891809</identifier><identifier>CODEN: IJCDD5</identifier><language>eng</language><publisher>Shannon: Elsevier Ireland Ltd</publisher><subject>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</subject><ispartof>International journal of cardiology, 1996-09, Vol.56 (1), p.83-85</ispartof><rights>1996</rights><rights>1996 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c386t-573d7196f93de5449b070e3549b52e74f6c5defed52f66d47c96408b1cafcc7f3</citedby><cites>FETCH-LOGICAL-c386t-573d7196f93de5449b070e3549b52e74f6c5defed52f66d47c96408b1cafcc7f3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/0167-5273(96)02713-1$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=3226244$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/8891809$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Yu, C.M.</creatorcontrib><creatorcontrib>Chow, L.T.C.</creatorcontrib><creatorcontrib>Sanderson, J.E.</creatorcontrib><title>Dilated cardiomyopathy in Noonan's syndrome</title><title>International journal of cardiology</title><addtitle>Int J Cardiol</addtitle><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.</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Cardiomyopathy, Dilated - complications</subject><subject>Cardiomyopathy, Dilated - diagnosis</subject><subject>Cardiomyopathy, Dilated - physiopathology</subject><subject>Dilated cardiomyopathy</subject><subject>Diseases of the osteoarticular system</subject><subject>Echocardiography, Doppler, Pulsed</subject><subject>Hemodynamics</subject><subject>Humans</subject><subject>Male</subject><subject>Malformations and congenital and or hereditary diseases involving bones. Joint deformations</subject><subject>Medical sciences</subject><subject>Noonan Syndrome - complications</subject><subject>Noonan Syndrome - diagnosis</subject><subject>Noonan Syndrome - physiopathology</subject><subject>Noonan's syndrome</subject><subject>Ventricular Dysfunction, Left - complications</subject><subject>Ventricular Dysfunction, Left - diagnosis</subject><subject>Ventricular Dysfunction, Left - physiopathology</subject><issn>0167-5273</issn><issn>1874-1754</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1996</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kEtLxDAQgIMouq7-A4U9iA-kmrRpHhdB1icsetFzyCYTjLTNmnSF_ntbtuzRwzAzzDfD8CF0QvANwYTd9sGzMufFpWRXOOekyMgOmhDBaUZ4SXfRZIscoMOUvjHGVEqxj_aFkERgOUHXD77SLdiZ0dH6UHdhpduvbuab2VsIjW4u0ix1jY2hhiO053SV4HjMU_T59Pgxf8kW78-v8_tFZgrB2qzkheVEMicLCyWlcok5hqLsizIHTh0zpQUHtswdY5ZyIxnFYkmMdsZwV0zR-ebuKoafNaRW1T4ZqCrdQFgnxQWVOJeyB-kGNDGkFMGpVfS1jp0iWA2O1CBADQKU7JvBkSL92ul4f72swW6XRin9_Gyc62R05aJujE9brMhzllPaY3cbDHoXvx6iSsZDY8D6CKZVNvj___gDNTqBXQ</recordid><startdate>19960901</startdate><enddate>19960901</enddate><creator>Yu, C.M.</creator><creator>Chow, L.T.C.</creator><creator>Sanderson, J.E.</creator><general>Elsevier Ireland Ltd</general><general>Elsevier Science</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>19960901</creationdate><title>Dilated cardiomyopathy in Noonan's syndrome</title><author>Yu, C.M. ; Chow, L.T.C. ; Sanderson, J.E.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c386t-573d7196f93de5449b070e3549b52e74f6c5defed52f66d47c96408b1cafcc7f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1996</creationdate><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>Cardiomyopathy, Dilated - complications</topic><topic>Cardiomyopathy, Dilated - diagnosis</topic><topic>Cardiomyopathy, Dilated - physiopathology</topic><topic>Dilated cardiomyopathy</topic><topic>Diseases of the osteoarticular system</topic><topic>Echocardiography, Doppler, Pulsed</topic><topic>Hemodynamics</topic><topic>Humans</topic><topic>Male</topic><topic>Malformations and congenital and or hereditary diseases involving bones. 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. 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.</abstract><cop>Shannon</cop><pub>Elsevier Ireland Ltd</pub><pmid>8891809</pmid><doi>10.1016/0167-5273(96)02713-1</doi><tpages>3</tpages></addata></record> |
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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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