Cardiovascular malformations in Turner's and Noonan's syndrome
The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan'...
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| Veröffentlicht in: | British Heart Journal 1978-05, Vol.40 (5), p.500-509 |
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| creator | Van der Hauwaert, L G Fryns, J P Dumoulin, M Logghe, N |
| description | The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal defect associated with mild supravalvar pulmonary stenosis and anomalous drainage of the right upper pulmonary veins. In the majority of patients the electrocardiogram was different from the pattern usually seen in pulmonary valve stenosis: the QRS axis in the frontal plane was superiorly oriented in 7 out of 9 cases and in 2 patients evidence of right ventricular hypertrophy was lacking in the right praecordial leads; in 5 patients an rS complex was seen in the left praecordial leads. Gross thickening of pulmonary valve cusps was found at operation in 4 of the 8 patients who were operated on. Although phenotypically related, Turner's and Noonan's syndromes are associated with different and distinct cardiovascular anomalies. |
| doi_str_mv | 10.1136/hrt.40.5.500 |
| format | Article |
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Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal defect associated with mild supravalvar pulmonary stenosis and anomalous drainage of the right upper pulmonary veins. In the majority of patients the electrocardiogram was different from the pattern usually seen in pulmonary valve stenosis: the QRS axis in the frontal plane was superiorly oriented in 7 out of 9 cases and in 2 patients evidence of right ventricular hypertrophy was lacking in the right praecordial leads; in 5 patients an rS complex was seen in the left praecordial leads. Gross thickening of pulmonary valve cusps was found at operation in 4 of the 8 patients who were operated on. 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Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal defect associated with mild supravalvar pulmonary stenosis and anomalous drainage of the right upper pulmonary veins. In the majority of patients the electrocardiogram was different from the pattern usually seen in pulmonary valve stenosis: the QRS axis in the frontal plane was superiorly oriented in 7 out of 9 cases and in 2 patients evidence of right ventricular hypertrophy was lacking in the right praecordial leads; in 5 patients an rS complex was seen in the left praecordial leads. Gross thickening of pulmonary valve cusps was found at operation in 4 of the 8 patients who were operated on. Although phenotypically related, Turner's and Noonan's syndromes are associated with different and distinct cardiovascular anomalies.</description><subject>Adolescent</subject><subject>Aortic Coarctation - complications</subject><subject>Aortic Valve Stenosis - complications</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Electrocardiography</subject><subject>Female</subject><subject>Heart Valve Diseases - complications</subject><subject>Humans</subject><subject>Karyotyping</subject><subject>Male</subject><subject>Phenotype</subject><subject>Phonocardiography</subject><subject>Pulmonary Valve Stenosis - complications</subject><subject>Turner Syndrome - complications</subject><issn>0007-0769</issn><issn>1355-6037</issn><issn>1468-201X</issn><issn>2053-5864</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1978</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNp9kUtv1DAUha2KtgwDuy5ZREKiGzL4_ViAhCL6QGXYTBFiYzmx02ZI7GInFf33uEo1almwsq7Od6_PvQeAIwRXCBH-_jqOKwpXbMUg3AMLRLksMUQ_noEFhFCUUHD1HLxIaZtLqiQ_BAeccYzYAnysTLRduDWpmXoTi8H0bYiDGbvgU9H5YjNF7-JxKoy3xToEb3wu0p23MQzuJdhvTZ_cq4d3CS5PPm-qs_Li2-l59emirAnGsCTcNKqVxErqEJeQYOacVaa2SHDeEueUs6alSFGMlBICM9zKWhKEiRWyJUvwYZ57M9WDs43zYzS9vondYOKdDqbTTxXfXeurcKupJJSw3P_2oT-G35NLox661Li-N96FKWlBIUQoG1uCN_-A25AvkHfTSAgIlcQEZerdTDUxpBRdu3OCoL7PROdMNIWa6ZxJxl8_dr-D5xCyXM5yl0b3Z6ea-EtzQQTT6--Vrr5-4WS94fpn5o9nvh62___4L_8Joy4</recordid><startdate>197805</startdate><enddate>197805</enddate><creator>Van der Hauwaert, L G</creator><creator>Fryns, J P</creator><creator>Dumoulin, M</creator><creator>Logghe, N</creator><general>BMJ Publishing Group Ltd and British Cardiovascular Society</general><general>BMJ Publishing Group LTD</general><scope>BSCLL</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>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88I</scope><scope>8AF</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>197805</creationdate><title>Cardiovascular malformations in Turner's and Noonan's syndrome</title><author>Van der Hauwaert, L G ; Fryns, J P ; Dumoulin, M ; Logghe, N</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b3220-36ac9f83d84e1680325eed9abd1766f3ee9edaf4194219977252f8b83123d78f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1978</creationdate><topic>Adolescent</topic><topic>Aortic Coarctation - complications</topic><topic>Aortic Valve Stenosis - complications</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Electrocardiography</topic><topic>Female</topic><topic>Heart Valve Diseases - complications</topic><topic>Humans</topic><topic>Karyotyping</topic><topic>Male</topic><topic>Phenotype</topic><topic>Phonocardiography</topic><topic>Pulmonary Valve Stenosis - complications</topic><topic>Turner Syndrome - complications</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Van der Hauwaert, L G</creatorcontrib><creatorcontrib>Fryns, J P</creatorcontrib><creatorcontrib>Dumoulin, M</creatorcontrib><creatorcontrib>Logghe, N</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Science Database (Alumni Edition)</collection><collection>STEM Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>BMJ Journals</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Science Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>British Heart Journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Van der Hauwaert, L G</au><au>Fryns, J P</au><au>Dumoulin, M</au><au>Logghe, N</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cardiovascular malformations in Turner's and Noonan's syndrome</atitle><jtitle>British Heart Journal</jtitle><addtitle>Br Heart J</addtitle><date>1978-05</date><risdate>1978</risdate><volume>40</volume><issue>5</issue><spage>500</spage><epage>509</epage><pages>500-509</pages><issn>0007-0769</issn><issn>1355-6037</issn><eissn>1468-201X</eissn><eissn>2053-5864</eissn><abstract>The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal defect associated with mild supravalvar pulmonary stenosis and anomalous drainage of the right upper pulmonary veins. In the majority of patients the electrocardiogram was different from the pattern usually seen in pulmonary valve stenosis: the QRS axis in the frontal plane was superiorly oriented in 7 out of 9 cases and in 2 patients evidence of right ventricular hypertrophy was lacking in the right praecordial leads; in 5 patients an rS complex was seen in the left praecordial leads. Gross thickening of pulmonary valve cusps was found at operation in 4 of the 8 patients who were operated on. Although phenotypically related, Turner's and Noonan's syndromes are associated with different and distinct cardiovascular anomalies.</abstract><cop>England</cop><pub>BMJ Publishing Group Ltd and British Cardiovascular Society</pub><pmid>656215</pmid><doi>10.1136/hrt.40.5.500</doi><tpages>10</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Adolescent Aortic Coarctation - complications Aortic Valve Stenosis - complications Child Child, Preschool Electrocardiography Female Heart Valve Diseases - complications Humans Karyotyping Male Phenotype Phonocardiography Pulmonary Valve Stenosis - complications Turner Syndrome - complications |
| title | Cardiovascular malformations in Turner's and Noonan's syndrome |
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