Spectrum of cardiovascular anomalies in Williams-Beuren syndrome
Identifieur interne : 000792 ( Istex/Corpus ); précédent : 000791; suivant : 000793Spectrum of cardiovascular anomalies in Williams-Beuren syndrome
Auteurs : E. Zalzstein ; C. A. F. Moes ; N. N. Musewe ; R. M. FreedomSource :
- Pediatric Cardiology [ 0172-0643 ] ; 1991-12-01.
Abstract
Summary: This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2∶1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticsts. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10–40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. The role of coronary artery abnormalities has yet to be defined.
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DOI: 10.1007/BF02310569
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ISTEX:8CF920A63F9CE8956B8EC847FF8C7F31045DD509Le document en format XML
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Zalzstein</name><affiliation><mods:affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</mods:affiliation></affiliation></author><author><name sortKey="Moes, C A F" sort="Moes, C A F" uniqKey="Moes C" first="C. A. F." last="Moes">C. A. F. Moes</name><affiliation><mods:affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</mods:affiliation></affiliation></author><author><name sortKey="Musewe, N N" sort="Musewe, N N" uniqKey="Musewe N" first="N. N." last="Musewe">N. N. Musewe</name><affiliation><mods:affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</mods:affiliation></affiliation></author><author><name sortKey="Freedom, R M" sort="Freedom, R M" uniqKey="Freedom R" first="R. M." last="Freedom">R. M. Freedom</name><affiliation><mods:affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Pediatric Cardiology</title><title level="j" type="abbrev">Pediatr Cardiol</title><idno type="ISSN">0172-0643</idno><idno type="eISSN">1432-1971</idno><imprint><publisher>Springer-Verlag</publisher><pubPlace>New York</pubPlace><date type="published" when="1991-12-01">1991-12-01</date><biblScope unit="volume">12</biblScope><biblScope unit="issue">4</biblScope><biblScope unit="page" from="219">219</biblScope><biblScope unit="page" to="223">223</biblScope></imprint><idno type="ISSN">0172-0643</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0172-0643</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Summary: This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2∶1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticsts. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10–40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. 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Zalzstein</name><affiliations><json:string>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>C. A. F. Moes</name><affiliations><json:string>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>N. N. Musewe</name><affiliations><json:string>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>Dr. R. M. 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Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10–40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. 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xml:id="author-0002"><persName><forename type="first">N.</forename><surname>Musewe</surname></persName><affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</affiliation></author><author xml:id="author-0003" corresp="yes"><persName><forename type="first">R.</forename><surname>Freedom</surname></persName><roleName type="degree">Dr.</roleName><affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</affiliation></author><idno type="istex">8CF920A63F9CE8956B8EC847FF8C7F31045DD509</idno><idno type="DOI">10.1007/BF02310569</idno><idno type="ArticleID">BF02310569</idno><idno type="ArticleID">Art5</idno></analytic><monogr><title level="j">Pediatric Cardiology</title><title level="j" type="abbrev">Pediatr Cardiol</title><idno type="pISSN">0172-0643</idno><idno type="eISSN">1432-1971</idno><idno type="journal-ID">true</idno><idno type="issue-article-count">14</idno><idno type="volume-issue-count">4</idno><imprint><publisher>Springer-Verlag</publisher><pubPlace>New York</pubPlace><date type="published" when="1991-12-01"></date><biblScope unit="volume">12</biblScope><biblScope unit="issue">4</biblScope><biblScope unit="page" from="219">219</biblScope><biblScope unit="page" to="223">223</biblScope></imprint></monogr></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1991</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Summary: This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2∶1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticsts. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10–40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. The role of coronary artery abnormalities has yet to be defined.</p></abstract><textClass><keywords scheme="Journal Subject"><list><head>Medicine & Public Health</head><item><term>Cardiology</term></item><item><term>Cardiac Surgery</term></item><item><term>Vascular Surgery</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1991-12-01">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api-v5.istex.fr/document/8CF920A63F9CE8956B8EC847FF8C7F31045DD509/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Springer, Publisher found" wicri:toSee="no header"><istex:xmlDeclaration>version="1.0" encoding="UTF-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//Springer-Verlag//DTD A++ V2.4//EN" URI="http://devel.springer.de/A++/V2.4/DTD/A++V2.4.dtd" 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DisplayOrder="Western"><GivenName>E.</GivenName><FamilyName>Zalzstein</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff1"><AuthorName DisplayOrder="Western"><GivenName>C.</GivenName><GivenName>A.</GivenName><GivenName>F.</GivenName><FamilyName>Moes</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff1"><AuthorName DisplayOrder="Western"><GivenName>N.</GivenName><GivenName>N.</GivenName><FamilyName>Musewe</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff1" CorrespondingAffiliationID="Aff1"><AuthorName DisplayOrder="Western"><Prefix>Dr.</Prefix><GivenName>R.</GivenName><GivenName>M.</GivenName><FamilyName>Freedom</FamilyName></AuthorName></Author><Affiliation ID="Aff1"><OrgDivision>Division of Cardiology, Department of Pediatrics</OrgDivision><OrgName>The Hospital for Sick Children</OrgName><OrgAddress><Postcode>M5G 1X8</Postcode><City>Toronto</City><State>Ontario</State><Country>Canada</Country></OrgAddress></Affiliation></AuthorGroup><Abstract ID="Abs1" Language="En"><Heading>Summary</Heading><Para>This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2∶1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticsts. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10–40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. The role of coronary artery abnormalities has yet to be defined.</Para></Abstract><KeywordGroup Language="En"><Heading>Key Words</Heading><Keyword>Williams-Beuren syndrome</Keyword><Keyword>Supravalvular aortic stenosis</Keyword><Keyword>Pulmonary artery branch stenosis</Keyword></KeywordGroup></ArticleHeader><NoBody></NoBody></Article></Issue></Volume></Journal></Publisher></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Spectrum of cardiovascular anomalies in Williams-Beuren syndrome</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Spectrum of cardiovascular anomalies in Williams-Beuren syndrome</title></titleInfo><name type="personal"><namePart type="given">E.</namePart><namePart type="family">Zalzstein</namePart><affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">C.</namePart><namePart type="given">A.</namePart><namePart type="given">F.</namePart><namePart type="family">Moes</namePart><affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">N.</namePart><namePart type="given">N.</namePart><namePart type="family">Musewe</namePart><affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal" displayLabel="corresp"><namePart type="termsOfAddress">Dr.</namePart><namePart type="given">R.</namePart><namePart type="given">M.</namePart><namePart type="family">Freedom</namePart><affiliation>Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="OriginalPaper"></genre><originInfo><publisher>Springer-Verlag</publisher><place><placeTerm type="text">New York</placeTerm></place><dateIssued encoding="w3cdtf">1991-12-01</dateIssued><copyrightDate encoding="w3cdtf">1991</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType></physicalDescription><abstract lang="en">Summary: This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2∶1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticsts. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10–40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. The role of coronary artery abnormalities has yet to be defined.</abstract><note>Original Articles</note><relatedItem type="host"><titleInfo><title>Pediatric Cardiology</title></titleInfo><titleInfo type="abbreviated"><title>Pediatr Cardiol</title></titleInfo><genre type="journal" displayLabel="Archive Journal"></genre><originInfo><dateIssued encoding="w3cdtf">1991-12-01</dateIssued><copyrightDate encoding="w3cdtf">1991</copyrightDate></originInfo><subject><genre>Medicine & Public Health</genre><topic>Cardiology</topic><topic>Cardiac Surgery</topic><topic>Vascular Surgery</topic></subject><identifier type="ISSN">0172-0643</identifier><identifier type="eISSN">1432-1971</identifier><identifier type="JournalID">246</identifier><identifier type="IssueArticleCount">14</identifier><identifier type="VolumeIssueCount">4</identifier><part><date>1991</date><detail type="volume"><number>12</number><caption>vol.</caption></detail><detail type="issue"><number>4</number><caption>no.</caption></detail><extent unit="pages"><start>219</start><end>223</end></extent></part><recordInfo><recordOrigin>Springer-Verlag New York Inc., 1991</recordOrigin></recordInfo></relatedItem><identifier type="istex">8CF920A63F9CE8956B8EC847FF8C7F31045DD509</identifier><identifier type="DOI">10.1007/BF02310569</identifier><identifier type="ArticleID">BF02310569</identifier><identifier type="ArticleID">Art5</identifier><accessCondition type="use and reproduction" contentType="copyright">Springer-Verlag New York Inc, 1991</accessCondition><recordInfo><recordContentSource>SPRINGER</recordContentSource><recordOrigin>Springer-Verlag New York Inc, 1991</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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