The right ventricle in Fabry disease.
Identifieur interne : 003C88 ( PubMed/Curation ); précédent : 003C87; suivant : 003C89The right ventricle in Fabry disease.
Auteurs : C. Kampmann [Allemagne] ; F A Baehner ; C. Whybra ; M. Bajbouj ; K. Baron ; M. Knuf ; C M Wiethoff ; H. Trübel ; M. BeckSource :
- Acta paediatrica (Oslo, Norway : 1992). Supplement [ 0803-5326 ] ; 2005.
Descripteurs français
- KwdFr :
- Adulte, Cardiomyopathies (), Cardiomyopathies (diagnostic), Cardiomyopathies (physiopathologie), Diagnostic différentiel, Dysfonction ventriculaire droite (), Dysfonction ventriculaire droite (diagnostic), Dysfonction ventriculaire droite (physiopathologie), Défaillance cardiaque (), Défaillance cardiaque (diagnostic), Défaillance cardiaque (physiopathologie), Femelle, Humains, Hypertrophie ventriculaire droite (), Hypertrophie ventriculaire droite (anatomopathologie), Hypertrophie ventriculaire droite (physiopathologie), Hypertrophie ventriculaire gauche (), Hypertrophie ventriculaire gauche (anatomopathologie), Hypertrophie ventriculaire gauche (physiopathologie), Indice de masse corporelle, Maladie de Fabry (), Maladie de Fabry (physiopathologie), Mâle, Pression sanguine (physiologie), Électrocardiographie.
- MESH :
- anatomopathologie : Hypertrophie ventriculaire droite, Hypertrophie ventriculaire gauche.
- diagnostic : Cardiomyopathies, Dysfonction ventriculaire droite, Défaillance cardiaque.
- physiologie : Pression sanguine.
- physiopathologie : Cardiomyopathies, Dysfonction ventriculaire droite, Défaillance cardiaque, Hypertrophie ventriculaire droite, Hypertrophie ventriculaire gauche, Maladie de Fabry.
- Adulte, Cardiomyopathies, Diagnostic différentiel, Dysfonction ventriculaire droite, Défaillance cardiaque, Femelle, Humains, Hypertrophie ventriculaire droite, Hypertrophie ventriculaire gauche, Indice de masse corporelle, Maladie de Fabry, Mâle, Électrocardiographie.
English descriptors
- KwdEn :
- Adult, Blood Pressure (physiology), Body Mass Index, Cardiomyopathies (complications), Cardiomyopathies (diagnosis), Cardiomyopathies (physiopathology), Diagnosis, Differential, Electrocardiography, Fabry Disease (complications), Fabry Disease (physiopathology), Female, Heart Failure (complications), Heart Failure (diagnosis), Heart Failure (physiopathology), Humans, Hypertrophy, Left Ventricular (complications), Hypertrophy, Left Ventricular (pathology), Hypertrophy, Left Ventricular (physiopathology), Hypertrophy, Right Ventricular (complications), Hypertrophy, Right Ventricular (pathology), Hypertrophy, Right Ventricular (physiopathology), Male, Ventricular Dysfunction, Right (complications), Ventricular Dysfunction, Right (diagnosis), Ventricular Dysfunction, Right (physiopathology).
- MESH :
- complications : Cardiomyopathies, Fabry Disease, Heart Failure, Hypertrophy, Left Ventricular, Hypertrophy, Right Ventricular, Ventricular Dysfunction, Right.
- diagnosis : Cardiomyopathies, Heart Failure, Ventricular Dysfunction, Right.
- pathology : Hypertrophy, Left Ventricular, Hypertrophy, Right Ventricular.
- physiology : Blood Pressure.
- physiopathology : Cardiomyopathies, Fabry Disease, Heart Failure, Hypertrophy, Left Ventricular, Hypertrophy, Right Ventricular, Ventricular Dysfunction, Right.
- Adult, Body Mass Index, Diagnosis, Differential, Electrocardiography, Female, Humans, Male.
Abstract
Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease.
PubMed: 15895706
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Bajbouj</name></author><author><name sortKey="Baron, K" sort="Baron, K" uniqKey="Baron K" first="K" last="Baron">K. Baron</name></author><author><name sortKey="Knuf, M" sort="Knuf, M" uniqKey="Knuf M" first="M" last="Knuf">M. Knuf</name></author><author><name sortKey="Wiethoff, C M" sort="Wiethoff, C M" uniqKey="Wiethoff C" first="C M" last="Wiethoff">C M Wiethoff</name></author><author><name sortKey="Trubel, H" sort="Trubel, H" uniqKey="Trubel H" first="H" last="Trübel">H. Trübel</name></author><author><name sortKey="Beck, M" sort="Beck, M" uniqKey="Beck M" first="M" last="Beck">M. Beck</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2005">2005</date><idno type="RBID">pubmed:15895706</idno><idno type="pmid">15895706</idno><idno type="wicri:Area/PubMed/Corpus">003C88</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">003C88</idno><idno type="wicri:Area/PubMed/Curation">003C88</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">003C88</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">The right ventricle in Fabry disease.</title><author><name sortKey="Kampmann, C" sort="Kampmann, C" uniqKey="Kampmann C" first="C" last="Kampmann">C. Kampmann</name><affiliation wicri:level="1"><nlm:affiliation>Department of Cardiology, University Children's Hospital, Mainz, Germany. christoph.kampmann@uni-mainz.de</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Cardiology, University Children's Hospital, Mainz</wicri:regionArea></affiliation></author><author><name sortKey="Baehner, F A" sort="Baehner, F A" uniqKey="Baehner F" first="F A" last="Baehner">F A Baehner</name></author><author><name sortKey="Whybra, C" sort="Whybra, C" uniqKey="Whybra C" first="C" last="Whybra">C. Whybra</name></author><author><name sortKey="Bajbouj, M" sort="Bajbouj, M" uniqKey="Bajbouj M" first="M" last="Bajbouj">M. Bajbouj</name></author><author><name sortKey="Baron, K" sort="Baron, K" uniqKey="Baron K" first="K" last="Baron">K. Baron</name></author><author><name sortKey="Knuf, M" sort="Knuf, M" uniqKey="Knuf M" first="M" last="Knuf">M. Knuf</name></author><author><name sortKey="Wiethoff, C M" sort="Wiethoff, C M" uniqKey="Wiethoff C" first="C M" last="Wiethoff">C M Wiethoff</name></author><author><name sortKey="Trubel, H" sort="Trubel, H" uniqKey="Trubel H" first="H" last="Trübel">H. Trübel</name></author><author><name sortKey="Beck, M" sort="Beck, M" uniqKey="Beck M" first="M" last="Beck">M. Beck</name></author></analytic><series><title level="j">Acta paediatrica (Oslo, Norway : 1992). 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(complications)</term><term>Hypertrophy, Right Ventricular (pathology)</term><term>Hypertrophy, Right Ventricular (physiopathology)</term><term>Male</term><term>Ventricular Dysfunction, Right (complications)</term><term>Ventricular Dysfunction, Right (diagnosis)</term><term>Ventricular Dysfunction, Right (physiopathology)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Cardiomyopathies ()</term><term>Cardiomyopathies (diagnostic)</term><term>Cardiomyopathies (physiopathologie)</term><term>Diagnostic différentiel</term><term>Dysfonction ventriculaire droite ()</term><term>Dysfonction ventriculaire droite (diagnostic)</term><term>Dysfonction ventriculaire droite (physiopathologie)</term><term>Défaillance cardiaque ()</term><term>Défaillance cardiaque (diagnostic)</term><term>Défaillance cardiaque (physiopathologie)</term><term>Femelle</term><term>Humains</term><term>Hypertrophie ventriculaire droite ()</term><term>Hypertrophie ventriculaire droite (anatomopathologie)</term><term>Hypertrophie ventriculaire droite (physiopathologie)</term><term>Hypertrophie ventriculaire gauche ()</term><term>Hypertrophie ventriculaire gauche (anatomopathologie)</term><term>Hypertrophie ventriculaire gauche (physiopathologie)</term><term>Indice de masse corporelle</term><term>Maladie de Fabry ()</term><term>Maladie de Fabry (physiopathologie)</term><term>Mâle</term><term>Pression sanguine (physiologie)</term><term>Électrocardiographie</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Hypertrophie ventriculaire droite</term><term>Hypertrophie ventriculaire gauche</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Cardiomyopathies</term><term>Fabry Disease</term><term>Heart Failure</term><term>Hypertrophy, Left Ventricular</term><term>Hypertrophy, Right Ventricular</term><term>Ventricular Dysfunction, Right</term></keywords><keywords scheme="MESH" qualifier="diagnosis" 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qualifier="physiopathology" xml:lang="en"><term>Cardiomyopathies</term><term>Fabry Disease</term><term>Heart Failure</term><term>Hypertrophy, Left Ventricular</term><term>Hypertrophy, Right Ventricular</term><term>Ventricular Dysfunction, Right</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Body Mass Index</term><term>Diagnosis, Differential</term><term>Electrocardiography</term><term>Female</term><term>Humans</term><term>Male</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Cardiomyopathies</term><term>Diagnostic différentiel</term><term>Dysfonction ventriculaire droite</term><term>Défaillance cardiaque</term><term>Femelle</term><term>Humains</term><term>Hypertrophie ventriculaire droite</term><term>Hypertrophie ventriculaire gauche</term><term>Indice de masse corporelle</term><term>Maladie de Fabry</term><term>Mâle</term><term>Électrocardiographie</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">15895706</PMID><DateCreated><Year>2005</Year><Month>05</Month><Day>17</Day></DateCreated><DateCompleted><Year>2005</Year><Month>08</Month><Day>25</Day></DateCompleted><DateRevised><Year>2008</Year><Month>05</Month><Day>12</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0803-5326</ISSN><JournalIssue CitedMedium="Print"><Volume>94</Volume><Issue>447</Issue><PubDate><Year>2005</Year><Month>Mar</Month></PubDate></JournalIssue><Title>Acta paediatrica (Oslo, Norway : 1992). Supplement</Title><ISOAbbreviation>Acta Paediatr Suppl</ISOAbbreviation></Journal><ArticleTitle>The right ventricle in Fabry disease.</ArticleTitle><Pagination><MedlinePgn>15-8; discussion 9-10</MedlinePgn></Pagination><Abstract><AbstractText Label="AIM" NlmCategory="OBJECTIVE">Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease.</AbstractText><AbstractText Label="METHODS" NlmCategory="METHODS">Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease.</AbstractText><AbstractText Label="RESULTS" NlmCategory="RESULTS">RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement < 10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling pattems). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92 +/- 11.7 g/m(2.7)).</AbstractText><AbstractText Label="CONCLUSION" NlmCategory="CONCLUSIONS">RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Kampmann</LastName><ForeName>C</ForeName><Initials>C</Initials><AffiliationInfo><Affiliation>Department of Cardiology, University Children's Hospital, Mainz, Germany. christoph.kampmann@uni-mainz.de</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Baehner</LastName><ForeName>F A</ForeName><Initials>FA</Initials></Author><Author ValidYN="Y"><LastName>Whybra</LastName><ForeName>C</ForeName><Initials>C</Initials></Author><Author ValidYN="Y"><LastName>Bajbouj</LastName><ForeName>M</ForeName><Initials>M</Initials></Author><Author ValidYN="Y"><LastName>Baron</LastName><ForeName>K</ForeName><Initials>K</Initials></Author><Author ValidYN="Y"><LastName>Knuf</LastName><ForeName>M</ForeName><Initials>M</Initials></Author><Author ValidYN="Y"><LastName>Wiethoff</LastName><ForeName>C M</ForeName><Initials>CM</Initials></Author><Author ValidYN="Y"><LastName>Trübel</LastName><ForeName>H</ForeName><Initials>H</Initials></Author><Author ValidYN="Y"><LastName>Beck</LastName><ForeName>M</ForeName><Initials>M</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>Norway</Country><MedlineTA>Acta Paediatr Suppl</MedlineTA><NlmUniqueID>9315043</NlmUniqueID><ISSNLinking>0803-5326</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D001794" MajorTopicYN="N">Blood Pressure</DescriptorName><QualifierName UI="Q000502" MajorTopicYN="N">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D015992" MajorTopicYN="N">Body Mass Index</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D009202" MajorTopicYN="N">Cardiomyopathies</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000503" MajorTopicYN="N">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D003937" MajorTopicYN="N">Diagnosis, Differential</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D004562" MajorTopicYN="N">Electrocardiography</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000795" MajorTopicYN="N">Fabry Disease</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName><QualifierName UI="Q000503" MajorTopicYN="Y">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006333" MajorTopicYN="N">Heart Failure</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000503" MajorTopicYN="N">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D017379" MajorTopicYN="N">Hypertrophy, Left Ventricular</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName><QualifierName UI="Q000503" MajorTopicYN="N">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D017380" MajorTopicYN="N">Hypertrophy, Right Ventricular</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName><QualifierName UI="Q000503" MajorTopicYN="N">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D018497" MajorTopicYN="N">Ventricular Dysfunction, Right</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000503" MajorTopicYN="Y">physiopathology</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2005</Year><Month>5</Month><Day>18</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2005</Year><Month>8</Month><Day>27</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2005</Year><Month>5</Month><Day>18</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">15895706</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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