Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease
Identifieur interne : 001361 ( Main/Corpus ); précédent : 001360; suivant : 001362Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease
Auteurs : Jawahar Mehta ; Naip Tuna ; James H. Moller ; Robert J. DesnickSource :
- American Heart Journal [ 0002-8703 ] ; 1976.
Abstract
Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abnormal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chamber enlargement, and other abnormalities. Although Fabry's disease is rare, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.
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DOI: 10.1016/S0002-8703(77)80064-1
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<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease</title><author><name sortKey="Mehta, Jawahar" sort="Mehta, Jawahar" uniqKey="Mehta J" first="Jawahar" last="Mehta">Jawahar Mehta</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author><author><name sortKey="Tuna, Naip" sort="Tuna, Naip" uniqKey="Tuna N" first="Naip" last="Tuna">Naip Tuna</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author><author><name sortKey="Moller, James H" sort="Moller, James H" uniqKey="Moller J" first="James H." last="Moller">James H. Moller</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author><author><name sortKey="Desnick, Robert J" sort="Desnick, Robert J" uniqKey="Desnick R" first="Robert J." last="Desnick">Robert J. Desnick</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:A112CD775363665A5DF533374ABEAC4DFD9016D6</idno><date when="1977" year="1977">1977</date><idno type="doi">10.1016/S0002-8703(77)80064-1</idno><idno type="url">https://api.istex.fr/document/A112CD775363665A5DF533374ABEAC4DFD9016D6/fulltext/pdf</idno><idno type="wicri:Area/Main/Corpus">001361</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease</title><author><name sortKey="Mehta, Jawahar" sort="Mehta, Jawahar" uniqKey="Mehta J" first="Jawahar" last="Mehta">Jawahar Mehta</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author><author><name sortKey="Tuna, Naip" sort="Tuna, Naip" uniqKey="Tuna N" first="Naip" last="Tuna">Naip Tuna</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author><author><name sortKey="Moller, James H" sort="Moller, James H" uniqKey="Moller J" first="James H." last="Moller">James H. Moller</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author><author><name sortKey="Desnick, Robert J" sort="Desnick, Robert J" uniqKey="Desnick R" first="Robert J." last="Desnick">Robert J. Desnick</name><affiliation><mods:affiliation>Minneapolis, Minn., USA</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">American Heart Journal</title><title level="j" type="abbrev">YMHJ</title><idno type="ISSN">0002-8703</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1976">1976</date><biblScope unit="volume">93</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="699">699</biblScope><biblScope unit="page" to="705">705</biblScope></imprint><idno type="ISSN">0002-8703</idno></series><idno type="istex">A112CD775363665A5DF533374ABEAC4DFD9016D6</idno><idno type="DOI">10.1016/S0002-8703(77)80064-1</idno><idno type="PII">S0002-8703(77)80064-1</idno><idno type="ArticleID">77800641</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0002-8703</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abnormal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chamber enlargement, and other abnormalities. Although Fabry's disease is rare, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.</div></front></TEI><istex><corpusName>elsevier</corpusName><author><json:item><name>Jawahar Mehta M.D.</name><affiliations><json:string>Minneapolis, Minn., USA</json:string></affiliations></json:item><json:item><name>Naip Tuna Ph.D., M.D.</name><affiliations><json:string>Minneapolis, Minn., USA</json:string></affiliations></json:item><json:item><name>James H. Moller M.D.</name><affiliations><json:string>Minneapolis, Minn., USA</json:string></affiliations></json:item><json:item><name>Robert J. Desnick Ph.D., M.D.</name><affiliations><json:string>Minneapolis, Minn., USA</json:string></affiliations></json:item></author><articleId><json:string>77800641</json:string></articleId><language><json:string>eng</json:string></language><abstract>Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abnormal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chamber enlargement, and other abnormalities. Although Fabry's disease is rare, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.</abstract><qualityIndicators><score>7.916</score><pdfVersion>1.3</pdfVersion><pdfPageSize>576 x 792 pts</pdfPageSize><refBibsNative>true</refBibsNative><keywordCount>0</keywordCount><abstractCharCount>1640</abstractCharCount><pdfWordCount>6596</pdfWordCount><pdfCharCount>22345</pdfCharCount><pdfPageCount>7</pdfPageCount><abstractWordCount>243</abstractWordCount></qualityIndicators><title>Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease</title><pii><json:string>S0002-8703(77)80064-1</json:string></pii><genre><json:string>research-article</json:string></genre><host><volume>93</volume><pii><json:string>S0002-8703(77)X8061-0</json:string></pii><pages><last>705</last><first>699</first></pages><issn><json:string>0002-8703</json:string></issn><issue>6</issue><genre><json:string>Journal</json:string></genre><language><json:string>unknown</json:string></language><title>American Heart Journal</title><publicationDate>1977</publicationDate></host><categories><wos><json:string>CARDIAC & CARDIOVASCULAR SYSTEMS</json:string></wos></categories><publicationDate>1976</publicationDate><copyrightDate>1977</copyrightDate><doi><json:string>10.1016/S0002-8703(77)80064-1</json:string></doi><id>A112CD775363665A5DF533374ABEAC4DFD9016D6</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/A112CD775363665A5DF533374ABEAC4DFD9016D6/fulltext/pdf</uri></json:item><json:item><original>true</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/A112CD775363665A5DF533374ABEAC4DFD9016D6/fulltext/txt</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/A112CD775363665A5DF533374ABEAC4DFD9016D6/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/A112CD775363665A5DF533374ABEAC4DFD9016D6/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>ELSEVIER</publisher><availability><p>ELSEVIER</p></availability><date>1977</date></publicationStmt><notesStmt><note>This work was supported in part by a grant (74-915) from the American Heart Association; a grant (1-273) from the National Foundation-March of Dimes; a grant (AM 15174) from the National Institutes of Health; and a grant (RR-400) from the General Clinical Research Center, Program of the Division of Research Resources, National Institutes of Health; Dr. Desnick is a recipient of a National Institutes of Health Career Development Award (1K04-AM00042).</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease</title><author><persName><forename type="first">Jawahar</forename><surname>Mehta</surname></persName><roleName type="degree">M.D.</roleName><note type="biography">Reprint requests: J. Mehta, M.D., Box J-277, JHM Health Center, University of Florida, Gainsville, Fla. 32610.</note><note type="biography">From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</note><affiliation>Reprint requests: J. Mehta, M.D., Box J-277, JHM Health Center, University of Florida, Gainsville, Fla. 32610.</affiliation><affiliation>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</affiliation><affiliation>Minneapolis, Minn., USA</affiliation></author><author><persName><forename type="first">Naip</forename><surname>Tuna</surname></persName><roleName type="degree">Ph.D., M.D.</roleName><note type="biography">From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</note><affiliation>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</affiliation><affiliation>Minneapolis, Minn., USA</affiliation></author><author><persName><forename type="first">James H.</forename><surname>Moller</surname></persName><roleName type="degree">M.D.</roleName><note type="biography">From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</note><affiliation>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</affiliation><affiliation>Minneapolis, Minn., USA</affiliation></author><author><persName><forename type="first">Robert J.</forename><surname>Desnick</surname></persName><roleName type="degree">Ph.D., M.D.</roleName><note type="biography">From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</note><affiliation>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</affiliation><affiliation>Minneapolis, Minn., USA</affiliation></author></analytic><monogr><title level="j">American Heart Journal</title><title level="j" type="abbrev">YMHJ</title><idno type="pISSN">0002-8703</idno><idno type="PII">S0002-8703(77)X8061-0</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1976"></date><biblScope unit="volume">93</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="699">699</biblScope><biblScope unit="page" to="705">705</biblScope></imprint></monogr><idno type="istex">A112CD775363665A5DF533374ABEAC4DFD9016D6</idno><idno type="DOI">10.1016/S0002-8703(77)80064-1</idno><idno type="PII">S0002-8703(77)80064-1</idno><idno type="ArticleID">77800641</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1977</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abnormal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chamber enlargement, and other abnormalities. Although Fabry's disease is rare, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.</p></abstract></profileDesc><revisionDesc><change when="1976-01-27">Received</change><change when="1976">Published</change></revisionDesc></teiHeader></istex:fulltextTEI></fulltext><metadata><istex:metadataXml wicri:clean="Elsevier doc found" wicri:toSee="Elsevier, no converted or simple article"><istex:xmlDeclaration>version="1.0" encoding="UTF-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//ES//DTD journal article DTD version 5.0.1//EN//XML" URI="art501.dtd" name="istex:docType"></istex:docType><istex:document><article version="5.0" xml:lang="en" docsubtype="fla"><item-info><jid>YMHJ</jid><aid>77800641</aid><ce:pii>S0002-8703(77)80064-1</ce:pii><ce:doi>10.1016/S0002-8703(77)80064-1</ce:doi><ce:copyright type="unknown" year="1977"></ce:copyright></item-info><head><ce:article-footnote><ce:label>*</ce:label><ce:note-para>This work was supported in part by a grant (74-915) from the American Heart Association; a grant (1-273) from the National Foundation-March of Dimes; a grant (AM 15174) from the National Institutes of Health; and a grant (RR-400) from the General Clinical Research Center, Program of the Division of Research Resources, National Institutes of Health; Dr. Desnick is a recipient of a National Institutes of Health Career Development Award (1K04-AM00042).</ce:note-para></ce:article-footnote><ce:title>Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease</ce:title><ce:author-group><ce:author><ce:degrees>M.D.</ce:degrees><ce:given-name>Jawahar</ce:given-name><ce:surname>Mehta</ce:surname><ce:cross-ref refid="cor1"><ce:sup loc="post">†</ce:sup></ce:cross-ref><ce:cross-ref refid="fn1"><ce:sup loc="post">1</ce:sup></ce:cross-ref></ce:author><ce:author><ce:degrees>Ph.D., M.D.</ce:degrees><ce:given-name>Naip</ce:given-name><ce:surname>Tuna</ce:surname><ce:cross-ref refid="fn1"><ce:sup loc="post">1</ce:sup></ce:cross-ref></ce:author><ce:author><ce:degrees>M.D.</ce:degrees><ce:given-name>James H.</ce:given-name><ce:surname>Moller</ce:surname><ce:cross-ref refid="fn1"><ce:sup loc="post">1</ce:sup></ce:cross-ref></ce:author><ce:author><ce:degrees>Ph.D., M.D.</ce:degrees><ce:given-name>Robert J.</ce:given-name><ce:surname>Desnick</ce:surname><ce:cross-ref refid="fn1"><ce:sup loc="post">1</ce:sup></ce:cross-ref></ce:author><ce:affiliation id="aff1"><ce:textfn>Minneapolis, Minn., USA</ce:textfn></ce:affiliation><ce:correspondence id="cor1"><ce:label>†</ce:label><ce:text>Reprint requests: J. Mehta, M.D., Box J-277, JHM Health Center, University of Florida, Gainsville, Fla. 32610.</ce:text></ce:correspondence><ce:footnote id="fn1"><ce:label>1</ce:label><ce:note-para>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</ce:note-para></ce:footnote></ce:author-group><ce:date-received day="27" month="1" year="1976"></ce:date-received><ce:abstract id="ab1" class="author" xml:lang="en"><ce:section-title>Summary</ce:section-title><ce:abstract-sec><ce:simple-para view="all" id="simple-para.0010">Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abnormal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chamber enlargement, and other abnormalities. Although Fabry's disease is rare, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.</ce:simple-para></ce:abstract-sec></ce:abstract></head><tail view="all"><ce:bibliography view="all" id="bibliography.0010"><ce:section-title>References</ce:section-title><ce:bibliography-sec id="bibliography-sec.0010"><ce:bib-reference id="bib1"><ce:label>1.</ce:label><sb:reference><sb:contribution langtype="en"><sb:authors><sb:author><ce:surname>Sweeley</ce:surname><ce:given-name>C.C.</ce:given-name></sb:author><sb:author><ce:surname>Klionsky</ce:surname><ce:given-name>B.</ce:given-name></sb:author><sb:author><ce:surname>Krivit</ce:surname><ce:given-name>W.</ce:given-name></sb:author><sb:et-al></sb:et-al></sb:authors><sb:title><sb:maintitle>Fabry's disease</sb:maintitle></sb:title></sb:contribution><sb:host><sb:edited-book><sb:editors><sb:editor><ce:surname>Stanbury</ce:surname><ce:given-name>J.B.</ce:given-name></sb:editor><sb:editor><ce:surname>Wyngaarden</ce:surname><ce:given-name>J.B.</ce:given-name></sb:editor><sb:editor><ce:surname>Fredrickson</ce:surname><ce:given-name>O.S.</ce:given-name></sb:editor></sb:editors><sb:title><sb:maintitle>The metabolic basis of inherited disease</sb:maintitle></sb:title><sb:edition>ed. 3</sb:edition><sb:date>1972</sb:date><sb:publisher><sb:name>McGraw-Hill Book Company, Inc.</sb:name><sb:location>New York</sb:location></sb:publisher></sb:edited-book><sb:pages><sb:first-page>663</sb:first-page><sb:last-page>687</sb:last-page></sb:pages></sb:host></sb:reference></ce:bib-reference><ce:bib-reference id="bib2"><ce:label>2.</ce:label><sb:reference><sb:contribution langtype="en"><sb:authors><sb:author><ce:surname>Ferrans</ce:surname><ce:given-name>V.J.</ce:given-name></sb:author><sb:author><ce:surname>Hibbs</ce:surname><ce:given-name>R.G.</ce:given-name></sb:author><sb:author><ce:surname>Burda</ce:surname><ce:given-name>C.D.</ce:given-name></sb:author></sb:authors><sb:title><sb:maintitle>The heart in Fabry's disease. 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Mehta, M.D., Box J-277, JHM Health Center, University of Florida, Gainsville, Fla. 32610.</description><description>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Naip</namePart><namePart type="family">Tuna</namePart><namePart type="termsOfAddress">Ph.D., M.D.</namePart><affiliation>Minneapolis, Minn., USA</affiliation><description>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">James H.</namePart><namePart type="family">Moller</namePart><namePart type="termsOfAddress">M.D.</namePart><affiliation>Minneapolis, Minn., USA</affiliation><description>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Robert J.</namePart><namePart type="family">Desnick</namePart><namePart type="termsOfAddress">Ph.D., M.D.</namePart><affiliation>Minneapolis, Minn., USA</affiliation><description>From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455.</description><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="Full-length article"></genre><originInfo><publisher>ELSEVIER</publisher><dateIssued encoding="w3cdtf">1976</dateIssued><dateCaptured encoding="w3cdtf">1976-01-27</dateCaptured><copyrightDate encoding="w3cdtf">1977</copyrightDate></originInfo><language><languageTerm type="code" authority="iso639-2b">eng</languageTerm><languageTerm type="code" authority="rfc3066">en</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType></physicalDescription><abstract lang="en">Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abnormal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chamber enlargement, and other abnormalities. Although Fabry's disease is rare, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.</abstract><note>This work was supported in part by a grant (74-915) from the American Heart Association; a grant (1-273) from the National Foundation-March of Dimes; a grant (AM 15174) from the National Institutes of Health; and a grant (RR-400) from the General Clinical Research Center, Program of the Division of Research Resources, National Institutes of Health; Dr. Desnick is a recipient of a National Institutes of Health Career Development Award (1K04-AM00042).</note><relatedItem type="host"><titleInfo><title>American Heart Journal</title></titleInfo><titleInfo type="abbreviated"><title>YMHJ</title></titleInfo><genre type="Journal">journal</genre><originInfo><dateIssued encoding="w3cdtf">197706</dateIssued></originInfo><identifier type="ISSN">0002-8703</identifier><identifier type="PII">S0002-8703(77)X8061-0</identifier><part><detail type="volume"><number>93</number><caption>vol.</caption></detail><detail type="issue"><number>6</number><caption>no.</caption></detail><extent unit="issue pages"><start>679</start><end>812</end></extent><extent unit="pages"><start>699</start><end>705</end></extent></part></relatedItem><identifier type="istex">A112CD775363665A5DF533374ABEAC4DFD9016D6</identifier><identifier type="DOI">10.1016/S0002-8703(77)80064-1</identifier><identifier type="PII">S0002-8703(77)80064-1</identifier><identifier type="ArticleID">77800641</identifier><recordInfo><recordContentSource>ELSEVIER</recordContentSource></recordInfo></mods></metadata><enrichments><istex:catWosTEI uri="https://api.istex.fr/document/A112CD775363665A5DF533374ABEAC4DFD9016D6/enrichments/catWos"><teiHeader><profileDesc><textClass><classCode scheme="WOS">CARDIAC & CARDIOVASCULAR SYSTEMS</classCode></textClass></profileDesc></teiHeader></istex:catWosTEI></enrichments><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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