Electrophysiologic Characteristics of Accessory Atrioventricular Connections in an Inherited Form of Wolff‐Parkinson‐White Syndrome
Identifieur interne : 000457 ( Main/Corpus ); précédent : 000456; suivant : 000458Electrophysiologic Characteristics of Accessory Atrioventricular Connections in an Inherited Form of Wolff‐Parkinson‐White Syndrome
Auteurs : Ali A. Mehdirad ; Diane Fatkin ; John P. Dimarco ; Calum A. Macrae ; Abdul Wase ; J. G. Seidman ; Christine E. Seidman ; D. Woodrow BensonSource :
- Journal of Cardiovascular Electrophysiology [ 1045-3873 ] ; 1999-05.
English descriptors
Abstract
EP Characteristics in Familial WPW. Introduction: A familial form of Wolff‐Parkinson‐White syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction ahnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome. Methods and Results: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy, Genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, θ= 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC. Conclusion: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.(J Cardiovasc Electrophysiol. Vol. 10, pp. 629‐635, May 1999)
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DOI: 10.1111/j.1540-8167.1999.tb00239.x
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Mehdirad</name><affiliation><mods:affiliation>Ohio State University School of Medicine, Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, The Ohio State University Medical Center, Columbus, Ohio</mods:affiliation></affiliation></author><author><name sortKey="Fatkin, Diane" sort="Fatkin, Diane" uniqKey="Fatkin D" first="Diane" last="Fatkin">Diane Fatkin</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Dimarco, John P" sort="Dimarco, John P" uniqKey="Dimarco J" first="John P." last="Dimarco">John P. Dimarco</name><affiliation><mods:affiliation>Department of Medicine, University of Virginia Charlottesville, Virginia</mods:affiliation></affiliation></author><author><name sortKey="Macrae, Calum A" sort="Macrae, Calum A" uniqKey="Macrae C" first="Calum A." last="Macrae">Calum A. Macrae</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Wase, Abdul" sort="Wase, Abdul" uniqKey="Wase A" first="Abdul" last="Wase">Abdul Wase</name><affiliation><mods:affiliation>Wright State University School of Medicine, Daylon, Ohio</mods:affiliation></affiliation></author><author><name sortKey="Seidman, J G" sort="Seidman, J G" uniqKey="Seidman J" first="J. G." last="Seidman">J. G. Seidman</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Seidman, Christine E" sort="Seidman, Christine E" uniqKey="Seidman C" first="Christine E." last="Seidman">Christine E. Seidman</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Benson, D Woodrow" sort="Benson, D Woodrow" uniqKey="Benson D" first="D. Woodrow" last="Benson">D. Woodrow Benson</name><affiliation><mods:affiliation>the Medical University of South Carolina. Charleston, South Carolina</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:1BC7B98639A601C4503D31A7BA19182844B236A6</idno><date when="1999" year="1999">1999</date><idno type="doi">10.1111/j.1540-8167.1999.tb00239.x</idno><idno type="url">https://api.istex.fr/document/1BC7B98639A601C4503D31A7BA19182844B236A6/fulltext/pdf</idno><idno type="wicri:Area/Main/Corpus">000457</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Electrophysiologic Characteristics of Accessory Atrioventricular Connections in an Inherited Form of Wolff‐Parkinson‐White Syndrome</title><author><name sortKey="Mehdirad, Ali A" sort="Mehdirad, Ali A" uniqKey="Mehdirad A" first="Ali A." last="Mehdirad">Ali A. Mehdirad</name><affiliation><mods:affiliation>Ohio State University School of Medicine, Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, The Ohio State University Medical Center, Columbus, Ohio</mods:affiliation></affiliation></author><author><name sortKey="Fatkin, Diane" sort="Fatkin, Diane" uniqKey="Fatkin D" first="Diane" last="Fatkin">Diane Fatkin</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Dimarco, John P" sort="Dimarco, John P" uniqKey="Dimarco J" first="John P." last="Dimarco">John P. Dimarco</name><affiliation><mods:affiliation>Department of Medicine, University of Virginia Charlottesville, Virginia</mods:affiliation></affiliation></author><author><name sortKey="Macrae, Calum A" sort="Macrae, Calum A" uniqKey="Macrae C" first="Calum A." last="Macrae">Calum A. Macrae</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Wase, Abdul" sort="Wase, Abdul" uniqKey="Wase A" first="Abdul" last="Wase">Abdul Wase</name><affiliation><mods:affiliation>Wright State University School of Medicine, Daylon, Ohio</mods:affiliation></affiliation></author><author><name sortKey="Seidman, J G" sort="Seidman, J G" uniqKey="Seidman J" first="J. G." last="Seidman">J. G. Seidman</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Seidman, Christine E" sort="Seidman, Christine E" uniqKey="Seidman C" first="Christine E." last="Seidman">Christine E. Seidman</name><affiliation><mods:affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</mods:affiliation></affiliation></author><author><name sortKey="Benson, D Woodrow" sort="Benson, D Woodrow" uniqKey="Benson D" first="D. Woodrow" last="Benson">D. Woodrow Benson</name><affiliation><mods:affiliation>the Medical University of South Carolina. Charleston, South Carolina</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Journal of Cardiovascular Electrophysiology</title><idno type="ISSN">1045-3873</idno><idno type="eISSN">1540-8167</idno><imprint><publisher>Blackwell Publishing Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="1999-05">1999-05</date><biblScope unit="volume">10</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="629">629</biblScope><biblScope unit="page" to="635">635</biblScope></imprint><idno type="ISSN">1045-3873</idno></series><idno type="istex">1BC7B98639A601C4503D31A7BA19182844B236A6</idno><idno type="DOI">10.1111/j.1540-8167.1999.tb00239.x</idno><idno type="ArticleID">JCE629</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">1045-3873</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Wolff‐Parkinson‐White syndrome</term><term>cardiomyopathy</term><term>genetics</term><term>heart block</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">EP Characteristics in Familial WPW. Introduction: A familial form of Wolff‐Parkinson‐White syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction ahnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome. Methods and Results: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy, Genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, θ= 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC. Conclusion: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.(J Cardiovasc Electrophysiol. Vol. 10, pp. 629‐635, May 1999)</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>ALI A. MEHDIRAD M.D.</name><affiliations><json:string>Ohio State University School of Medicine, Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, The Ohio State University Medical Center, Columbus, Ohio</json:string></affiliations></json:item><json:item><name>DIANE FATKIN M.D.</name><affiliations><json:string>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</json:string></affiliations></json:item><json:item><name>JOHN P. DiMARCO M.D.</name><affiliations><json:string>Department of Medicine, University of Virginia Charlottesville, Virginia</json:string></affiliations></json:item><json:item><name>CALUM A. MacRAE M.D.</name><affiliations><json:string>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</json:string></affiliations></json:item><json:item><name>ABDUL WASE M.D.</name><affiliations><json:string>Wright State University School of Medicine, Daylon, Ohio</json:string></affiliations></json:item><json:item><name>J.G. SEIDMAN Ph.D.</name><affiliations><json:string>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</json:string></affiliations></json:item><json:item><name>CHRISTINE E. SEIDMAN M.D.</name><affiliations><json:string>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</json:string></affiliations></json:item><json:item><name>D. WOODROW BENSON M.D., Ph.D.</name><affiliations><json:string>the Medical University of South Carolina. Charleston, South Carolina</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>Wolff‐Parkinson‐White syndrome</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>cardiomyopathy</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>heart block</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>genetics</value></json:item></subject><articleId><json:string>JCE629</json:string></articleId><language><json:string>eng</json:string></language><abstract>EP Characteristics in Familial WPW. Introduction: A familial form of Wolff‐Parkinson‐White syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction ahnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome. Methods and Results: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy, Genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, θ= 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC. Conclusion: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.(J Cardiovasc Electrophysiol. 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WOODROW</forename><surname>BENSON</surname></persName><roleName type="degree">M.D., Ph.D.</roleName><note type="correspondence"><p>Correspondence: D. Woodrow Benson, M.D., Ph.D., Director, Cardiovascular Genetics, Medical University of South Carolina, 171 Ashley Avenue, Charleston, SC 29425. Fax: 843‐792‐3284; E‐mail:</p></note><affiliation>the Medical University of South Carolina. Charleston, South Carolina</affiliation></author></analytic><monogr><title level="j">Journal of Cardiovascular Electrophysiology</title><idno type="pISSN">1045-3873</idno><idno type="eISSN">1540-8167</idno><idno type="DOI">10.1111/(ISSN)1540-8167</idno><imprint><publisher>Blackwell Publishing Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="1999-05"></date><biblScope unit="volume">10</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="629">629</biblScope><biblScope unit="page" to="635">635</biblScope></imprint></monogr><idno type="istex">1BC7B98639A601C4503D31A7BA19182844B236A6</idno><idno type="DOI">10.1111/j.1540-8167.1999.tb00239.x</idno><idno type="ArticleID">JCE629</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1999</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>EP Characteristics in Familial WPW. Introduction: A familial form of Wolff‐Parkinson‐White syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction ahnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome. Methods and Results: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy, Genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, θ= 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC. Conclusion: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.(J Cardiovasc Electrophysiol. Vol. 10, pp. 629‐635, May 1999)</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>Wolff‐Parkinson‐White syndrome</term></item><item><term>cardiomyopathy</term></item><item><term>heart block</term></item><item><term>genetics</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1999-05">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/1BC7B98639A601C4503D31A7BA19182844B236A6/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Blackwell Publishing Ltd</publisherName><publisherLoc>Oxford, UK</publisherLoc></publisherInfo><doi origin="wiley" registered="yes">10.1111/(ISSN)1540-8167</doi><issn type="print">1045-3873</issn><issn type="electronic">1540-8167</issn><idGroup><id type="product" value="JCE"></id><id type="publisherDivision" value="ST"></id></idGroup><titleGroup><title type="main" sort="JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY">Journal of Cardiovascular Electrophysiology</title></titleGroup></publicationMeta><publicationMeta level="part" position="05005"><doi origin="wiley">10.1111/jce.1999.10.issue-5</doi><numberingGroup><numbering type="journalVolume" number="10">10</numbering><numbering type="journalIssue" number="5">5</numbering></numberingGroup><coverDate startDate="1999-05">May 1999</coverDate></publicationMeta><publicationMeta level="unit" type="article" position="0062900" status="forIssue"><doi origin="wiley">10.1111/j.1540-8167.1999.tb00239.x</doi><idGroup><id type="unit" value="JCE629"></id></idGroup><countGroup><count type="pageTotal" number="7"></count></countGroup><titleGroup><title type="tocHeading1">ORIGINAL ARTICLES</title></titleGroup><eventGroup><event type="firstOnline" date="2007-04-20"></event><event type="publishedOnlineFinalForm" date="2007-04-20"></event><event type="xmlConverted" agent="Converter:BPG_TO_WML3G version:2.3.2 mode:FullText source:HeaderRef result:HeaderRef" date="2010-03-10"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-01-29"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-23"></event></eventGroup><numberingGroup><numbering type="pageFirst" number="629">629</numbering><numbering type="pageLast" number="635">635</numbering></numberingGroup><correspondenceTo>D. Woodrow Benson, M.D., Ph.D., Director, Cardiovascular Genetics, Medical University of South Carolina, 171 Ashley Avenue, Charleston, SC 29425. Fax: 843‐792‐3284; E‐mail: <email>bensondw@musc.edu</email></correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:JCE.JCE629.pdf"></link></linkGroup></publicationMeta><contentMeta><unparsedEditorialHistory>Manuscript received 30 October 1998; Accepted for publication 3 February 1999.</unparsedEditorialHistory><countGroup><count type="referenceTotal" number="23"></count><count type="linksCrossRef" number="9"></count></countGroup><titleGroup><title type="main">Electrophysiologic Characteristics of Accessory Atrioventricular Connections in an Inherited Form of Wolff‐Parkinson‐White Syndrome</title></titleGroup><creators><creator creatorRole="author" xml:id="cr1" affiliationRef="#a1"><personName><givenNames>ALI A.</givenNames><familyName>MEHDIRAD</familyName><degrees>M.D.</degrees></personName></creator><creator creatorRole="author" xml:id="cr2" affiliationRef="#a2"><personName><givenNames>DIANE</givenNames><familyName>FATKIN</familyName><degrees>M.D.</degrees></personName></creator><creator creatorRole="author" xml:id="cr3" affiliationRef="#a3"><personName><givenNames>JOHN P.</givenNames><familyName>DiMARCO</familyName><degrees>M.D.</degrees></personName></creator><creator creatorRole="author" xml:id="cr4" affiliationRef="#a2"><personName><givenNames>CALUM A.</givenNames><familyName>MacRAE</familyName><degrees>M.D.</degrees></personName></creator><creator creatorRole="author" xml:id="cr5" affiliationRef="#a4"><personName><givenNames>ABDUL</givenNames><familyName>WASE</familyName><degrees>M.D.</degrees></personName></creator><creator creatorRole="author" xml:id="cr6" affiliationRef="#a2"><personName><givenNames>J.G.</givenNames><familyName>SEIDMAN</familyName><degrees>Ph.D.</degrees></personName></creator><creator creatorRole="author" xml:id="cr7" affiliationRef="#a2"><personName><givenNames>CHRISTINE E.</givenNames><familyName>SEIDMAN</familyName><degrees>M.D.</degrees></personName></creator><creator creatorRole="author" xml:id="cr8" affiliationRef="#a5" corresponding="yes"><personName><givenNames>D. WOODROW</givenNames><familyName>BENSON</familyName><degrees>M.D., Ph.D.</degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="a1" countryCode="US"><unparsedAffiliation>Ohio State University School of Medicine, Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, The Ohio State University Medical Center, Columbus, Ohio</unparsedAffiliation></affiliation><affiliation xml:id="a2" countryCode="US"><unparsedAffiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</unparsedAffiliation></affiliation><affiliation xml:id="a3" countryCode="US"><unparsedAffiliation>Department of Medicine, University of Virginia Charlottesville, Virginia</unparsedAffiliation></affiliation><affiliation xml:id="a4" countryCode="US"><unparsedAffiliation>Wright State University School of Medicine, Daylon, Ohio</unparsedAffiliation></affiliation><affiliation xml:id="a5" countryCode="US"><unparsedAffiliation>the Medical University of South Carolina. Charleston, South Carolina</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en"><keyword xml:id="k1">Wolff‐Parkinson‐White syndrome</keyword><keyword xml:id="k2">cardiomyopathy</keyword><keyword xml:id="k3">heart block</keyword><keyword xml:id="k4">genetics</keyword></keywordGroup><abstractGroup><abstract type="main" xml:lang="en"><p>EP Characteristics in Familial WPW. <i>Introduction</i>: A familial form of Wolff‐Parkinson‐White syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction ahnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome.</p><p> <i>Methods and Results</i>: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy, Genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, θ= 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC.</p><p> <i>Conclusion</i>: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.(J Cardiovasc Electrophysiol. Vol. 10, pp. 629‐635, May 1999)</p></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="n-fnt-1" numbered="no"><p>Dr. D. Woodrow Benson's study was supported in part by grants from the National Institutes of Health (HL 09461‐01), and Drs. Diane Fatkin, J.G. Seidman, and Christine E. Seidman were supported by the Howard Hughes Medical Institute.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Electrophysiologic Characteristics of Accessory Atrioventricular Connections in an Inherited Form of Wolff‐Parkinson‐White Syndrome</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Electrophysiologic Characteristics of Accessory Atrioventricular Connections in an Inherited Form of Wolff‐Parkinson‐White Syndrome</title></titleInfo><name type="personal"><namePart type="given">ALI A.</namePart><namePart type="family">MEHDIRAD</namePart><namePart type="termsOfAddress">M.D.</namePart><affiliation>Ohio State University School of Medicine, Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, The Ohio State University Medical Center, Columbus, Ohio</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">DIANE</namePart><namePart type="family">FATKIN</namePart><namePart type="termsOfAddress">M.D.</namePart><affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">JOHN P.</namePart><namePart type="family">DiMARCO</namePart><namePart type="termsOfAddress">M.D.</namePart><affiliation>Department of Medicine, University of Virginia Charlottesville, Virginia</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">CALUM A.</namePart><namePart type="family">MacRAE</namePart><namePart type="termsOfAddress">M.D.</namePart><affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">ABDUL</namePart><namePart type="family">WASE</namePart><namePart type="termsOfAddress">M.D.</namePart><affiliation>Wright State University School of Medicine, Daylon, Ohio</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">J.G.</namePart><namePart type="family">SEIDMAN</namePart><namePart type="termsOfAddress">Ph.D.</namePart><affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">CHRISTINE E.</namePart><namePart type="family">SEIDMAN</namePart><namePart type="termsOfAddress">M.D.</namePart><affiliation>Harvard Medical School, Howard Hughes Medical Institute, Brigham and Women's Hospital, Boston, Massachusetts</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">D. WOODROW</namePart><namePart type="family">BENSON</namePart><namePart type="termsOfAddress">M.D., Ph.D.</namePart><affiliation>the Medical University of South Carolina. Charleston, South Carolina</affiliation><description>Correspondence: D. Woodrow Benson, M.D., Ph.D., Director, Cardiovascular Genetics, Medical University of South Carolina, 171 Ashley Avenue, Charleston, SC 29425. Fax: 843‐792‐3284; E‐mail: </description><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="article" displayLabel="article"></genre><originInfo><publisher>Blackwell Publishing Ltd</publisher><place><placeTerm type="text">Oxford, UK</placeTerm></place><dateIssued encoding="w3cdtf">1999-05</dateIssued><edition>Manuscript received 30 October 1998; Accepted for publication 3 February 1999.</edition><copyrightDate encoding="w3cdtf">1999</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="references">23</extent></physicalDescription><abstract lang="en">EP Characteristics in Familial WPW. Introduction: A familial form of Wolff‐Parkinson‐White syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction ahnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome. Methods and Results: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy, Genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, θ= 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC. Conclusion: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.(J Cardiovasc Electrophysiol. Vol. 10, pp. 629‐635, May 1999)</abstract><subject lang="en"><genre>Keywords</genre><topic>Wolff‐Parkinson‐White syndrome</topic><topic>cardiomyopathy</topic><topic>heart block</topic><topic>genetics</topic></subject><relatedItem type="host"><titleInfo><title>Journal of Cardiovascular Electrophysiology</title></titleInfo><genre type="Journal">journal</genre><identifier type="ISSN">1045-3873</identifier><identifier type="eISSN">1540-8167</identifier><identifier type="DOI">10.1111/(ISSN)1540-8167</identifier><identifier type="PublisherID">JCE</identifier><part><date>1999</date><detail type="volume"><caption>vol.</caption><number>10</number></detail><detail type="issue"><caption>no.</caption><number>5</number></detail><extent unit="pages"><start>629</start><end>635</end><total>7</total></extent></part></relatedItem><identifier type="istex">1BC7B98639A601C4503D31A7BA19182844B236A6</identifier><identifier type="DOI">10.1111/j.1540-8167.1999.tb00239.x</identifier><identifier type="ArticleID">JCE629</identifier><recordInfo><recordContentSource>WILEY</recordContentSource><recordOrigin>Blackwell Publishing Ltd</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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