Sudden Death in Supravalvular Aortic Stenosis: Fusion of a Coronary Leaflet to the Sinus Ridge, Dysplasia and Stenosis of Aortic and Pulmonic Valves
Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinp...
Gespeichert in:
| Veröffentlicht in: | Fetal and pediatric pathology 1992, Vol.12 (5), p.751-759 |
|---|---|
| Hauptverfasser: | , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 759 |
|---|---|
| container_issue | 5 |
| container_start_page | 751 |
| container_title | Fetal and pediatric pathology |
| container_volume | 12 |
| creator | Sun, Chen-Chih J. Jacot, Jorge Brenner, Joel I. |
| description | Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency. |
| doi_str_mv | 10.3109/15513819209024230 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmed_primary_1437889</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>73333511</sourcerecordid><originalsourceid>FETCH-LOGICAL-c467t-4f6381251037a59b29ab32b9e37e1f910849b0962074a76a754bf73f2afa875a3</originalsourceid><addsrcrecordid>eNp9kUtv1DAQxyMEKqXwATgg-cSJLX4k6xi4VFsKSCuBWOAaTZIx68qxFz-K9nvwgetoV0UIgS-e1_9nz0xVPWX0XDCqXrKmYaJlilNFec0FvVedzrGFaLm4f2ez5mH1KMZrSoVsW3lSnbB6ttRp9WuTxxEduURIW2Ic2eRdgBuwN9lCIBc-JDOQTULno4mvyFWOxjviNQGy8sE7CHuyRtAWE0mepC2SjXE5ks9m_I4vyOU-7ixEAwTceAeaAUf2HP6U7eRdcb6VhzE-rh5osBGfHO-z6uvV2y-r94v1x3cfVhfrxVAvZVrUell64w0rbUGjeq6gF7xXKCQyrRhta9VTteRU1iCXIJu611JoDhpa2YA4q54fuLvgf2SMqZtMHNBacOhz7KQop2GsFLJD4RB8jAF1twtmKq13jHbzJrq_NlE0z47w3E84_lYcRl_ybw5547QPE_z0wY5dgr31QQdwg4kz-t_413_Itwg2bQcI2F37HFyZ238-dwuheKkH</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>73333511</pqid></control><display><type>article</type><title>Sudden Death in Supravalvular Aortic Stenosis: Fusion of a Coronary Leaflet to the Sinus Ridge, Dysplasia and Stenosis of Aortic and Pulmonic Valves</title><source>MEDLINE</source><source>Taylor & Francis:Master (3349 titles)</source><source>Taylor & Francis Medical Library - CRKN</source><creator>Sun, Chen-Chih J. ; Jacot, Jorge ; Brenner, Joel I.</creator><creatorcontrib>Sun, Chen-Chih J. ; Jacot, Jorge ; Brenner, Joel I.</creatorcontrib><description>Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency.</description><identifier>ISSN: 1551-3815</identifier><identifier>ISSN: 0277-0938</identifier><identifier>EISSN: 1551-3823</identifier><identifier>DOI: 10.3109/15513819209024230</identifier><identifier>PMID: 1437889</identifier><language>eng</language><publisher>United States: Informa UK Ltd</publisher><subject>Abnormalities, Multiple - pathology ; aortic valve ; Aortic Valve - abnormalities ; Aortic Valve Stenosis - pathology ; Child, Preschool ; coronary ostium ; Death, Sudden, Cardiac ; Humans ; Male ; Pulmonary Valve - abnormalities ; Pulmonary Valve Stenosis - pathology ; pulmonic valve ; Sinus of Valsalva - abnormalities ; stenosis ; supravalvular stenosis ; valvular dysplasia</subject><ispartof>Fetal and pediatric pathology, 1992, Vol.12 (5), p.751-759</ispartof><rights>1992 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted 1992</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c467t-4f6381251037a59b29ab32b9e37e1f910849b0962074a76a754bf73f2afa875a3</citedby><cites>FETCH-LOGICAL-c467t-4f6381251037a59b29ab32b9e37e1f910849b0962074a76a754bf73f2afa875a3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.tandfonline.com/doi/pdf/10.3109/15513819209024230$$EPDF$$P50$$Ginformahealthcare$$H</linktopdf><linktohtml>$$Uhttps://www.tandfonline.com/doi/full/10.3109/15513819209024230$$EHTML$$P50$$Ginformahealthcare$$H</linktohtml><link.rule.ids>314,780,784,4024,27923,27924,27925,59647,60436,61221,61256,61402,61437</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/1437889$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sun, Chen-Chih J.</creatorcontrib><creatorcontrib>Jacot, Jorge</creatorcontrib><creatorcontrib>Brenner, Joel I.</creatorcontrib><title>Sudden Death in Supravalvular Aortic Stenosis: Fusion of a Coronary Leaflet to the Sinus Ridge, Dysplasia and Stenosis of Aortic and Pulmonic Valves</title><title>Fetal and pediatric pathology</title><addtitle>Pediatr Pathol</addtitle><description>Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency.</description><subject>Abnormalities, Multiple - pathology</subject><subject>aortic valve</subject><subject>Aortic Valve - abnormalities</subject><subject>Aortic Valve Stenosis - pathology</subject><subject>Child, Preschool</subject><subject>coronary ostium</subject><subject>Death, Sudden, Cardiac</subject><subject>Humans</subject><subject>Male</subject><subject>Pulmonary Valve - abnormalities</subject><subject>Pulmonary Valve Stenosis - pathology</subject><subject>pulmonic valve</subject><subject>Sinus of Valsalva - abnormalities</subject><subject>stenosis</subject><subject>supravalvular stenosis</subject><subject>valvular dysplasia</subject><issn>1551-3815</issn><issn>0277-0938</issn><issn>1551-3823</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1992</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kUtv1DAQxyMEKqXwATgg-cSJLX4k6xi4VFsKSCuBWOAaTZIx68qxFz-K9nvwgetoV0UIgS-e1_9nz0xVPWX0XDCqXrKmYaJlilNFec0FvVedzrGFaLm4f2ez5mH1KMZrSoVsW3lSnbB6ttRp9WuTxxEduURIW2Ic2eRdgBuwN9lCIBc-JDOQTULno4mvyFWOxjviNQGy8sE7CHuyRtAWE0mepC2SjXE5ks9m_I4vyOU-7ixEAwTceAeaAUf2HP6U7eRdcb6VhzE-rh5osBGfHO-z6uvV2y-r94v1x3cfVhfrxVAvZVrUell64w0rbUGjeq6gF7xXKCQyrRhta9VTteRU1iCXIJu611JoDhpa2YA4q54fuLvgf2SMqZtMHNBacOhz7KQop2GsFLJD4RB8jAF1twtmKq13jHbzJrq_NlE0z47w3E84_lYcRl_ybw5547QPE_z0wY5dgr31QQdwg4kz-t_413_Itwg2bQcI2F37HFyZ238-dwuheKkH</recordid><startdate>1992</startdate><enddate>1992</enddate><creator>Sun, Chen-Chih J.</creator><creator>Jacot, Jorge</creator><creator>Brenner, Joel I.</creator><general>Informa UK Ltd</general><general>Taylor & Francis</general><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>1992</creationdate><title>Sudden Death in Supravalvular Aortic Stenosis: Fusion of a Coronary Leaflet to the Sinus Ridge, Dysplasia and Stenosis of Aortic and Pulmonic Valves</title><author>Sun, Chen-Chih J. ; Jacot, Jorge ; Brenner, Joel I.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c467t-4f6381251037a59b29ab32b9e37e1f910849b0962074a76a754bf73f2afa875a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1992</creationdate><topic>Abnormalities, Multiple - pathology</topic><topic>aortic valve</topic><topic>Aortic Valve - abnormalities</topic><topic>Aortic Valve Stenosis - pathology</topic><topic>Child, Preschool</topic><topic>coronary ostium</topic><topic>Death, Sudden, Cardiac</topic><topic>Humans</topic><topic>Male</topic><topic>Pulmonary Valve - abnormalities</topic><topic>Pulmonary Valve Stenosis - pathology</topic><topic>pulmonic valve</topic><topic>Sinus of Valsalva - abnormalities</topic><topic>stenosis</topic><topic>supravalvular stenosis</topic><topic>valvular dysplasia</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sun, Chen-Chih J.</creatorcontrib><creatorcontrib>Jacot, Jorge</creatorcontrib><creatorcontrib>Brenner, Joel I.</creatorcontrib><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>Fetal and pediatric pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sun, Chen-Chih J.</au><au>Jacot, Jorge</au><au>Brenner, Joel I.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sudden Death in Supravalvular Aortic Stenosis: Fusion of a Coronary Leaflet to the Sinus Ridge, Dysplasia and Stenosis of Aortic and Pulmonic Valves</atitle><jtitle>Fetal and pediatric pathology</jtitle><addtitle>Pediatr Pathol</addtitle><date>1992</date><risdate>1992</risdate><volume>12</volume><issue>5</issue><spage>751</spage><epage>759</epage><pages>751-759</pages><issn>1551-3815</issn><issn>0277-0938</issn><eissn>1551-3823</eissn><abstract>Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency.</abstract><cop>United States</cop><pub>Informa UK Ltd</pub><pmid>1437889</pmid><doi>10.3109/15513819209024230</doi><tpages>9</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1551-3815 |
| ispartof | Fetal and pediatric pathology, 1992, Vol.12 (5), p.751-759 |
| issn | 1551-3815 0277-0938 1551-3823 |
| language | eng |
| recordid | cdi_pubmed_primary_1437889 |
| source | MEDLINE; Taylor & Francis:Master (3349 titles); Taylor & Francis Medical Library - CRKN |
| subjects | Abnormalities, Multiple - pathology aortic valve Aortic Valve - abnormalities Aortic Valve Stenosis - pathology Child, Preschool coronary ostium Death, Sudden, Cardiac Humans Male Pulmonary Valve - abnormalities Pulmonary Valve Stenosis - pathology pulmonic valve Sinus of Valsalva - abnormalities stenosis supravalvular stenosis valvular dysplasia |
| title | Sudden Death in Supravalvular Aortic Stenosis: Fusion of a Coronary Leaflet to the Sinus Ridge, Dysplasia and Stenosis of Aortic and Pulmonic Valves |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-26T11%3A01%3A45IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Sudden%20Death%20in%20Supravalvular%20Aortic%20Stenosis:%20Fusion%20of%20a%20Coronary%20Leaflet%20to%20the%20Sinus%20Ridge,%20Dysplasia%20and%20Stenosis%20of%20Aortic%20and%20Pulmonic%20Valves&rft.jtitle=Fetal%20and%20pediatric%20pathology&rft.au=Sun,%20Chen-Chih%20J.&rft.date=1992&rft.volume=12&rft.issue=5&rft.spage=751&rft.epage=759&rft.pages=751-759&rft.issn=1551-3815&rft.eissn=1551-3823&rft_id=info:doi/10.3109/15513819209024230&rft_dat=%3Cproquest_pubme%3E73333511%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=73333511&rft_id=info:pmid/1437889&rfr_iscdi=true |