Cardiovascular Manifestation of a Family with Marfan's Syndrome

Cardiovascular Manifestation of a Family with Marfan's Syndrome

Marfan's syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, thre...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:The journal of Tehran Heart Center 2011, Vol.6 (1), p.37-40
Hauptverfasser: Davari, Mohammad Hossein, Kazemi, Toba, Alimirzaei, Hossein, Rezvani, Mohamad Reza
Format: Artikel
Sprache:eng
Schlagworte:
Case Report
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
container_end_page 40
container_issue 1
container_start_page 37
container_title The journal of Tehran Heart Center
container_volume 6
creator Davari, Mohammad Hossein
Kazemi, Toba
Alimirzaei, Hossein
Rezvani, Mohamad Reza
description Marfan's syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, three sons, and five grandchildren). The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mitral regurgitation. At ten years' follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive.
format Article
fullrecord <record><control><sourceid>pubmed</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_3466859</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>23074603</sourcerecordid><originalsourceid>FETCH-LOGICAL-p789-fb2fa60ef5ceda5f4434235d0f8c836561177aad046a68d5c8bd7c41d0ddbe063</originalsourceid><addsrcrecordid>eNpVkM1Kw0AYRQdRbKy-gszOVeDL_HejSLBVqLiw-_AlM2NHkkmYpJW-vQV_0NVdHO5ZnBOSMQCTM66LU5IVmstccg0zcjGO7wCqYEKekxnjoIUCnpG7EpMN_R7HZtdios8Yg3fjhFPoI-09RbrELrQH-hGm7REnj_FmpK-HaFPfuUty5rEd3dX3zslm-bApH_P1y-qpvF_ngzaL3NfMowLnZeMsSi8EF4xLC940hiupikJrRAtCoTJWNqa2uhGFBWtrB4rPye2XdtjVnbONi1PCthpS6DAdqh5D9Z_EsK3e-n3FhVJGLo6C67-C3-dPCf4JyNRdBA</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype></control><display><type>article</type><title>Cardiovascular Manifestation of a Family with Marfan's Syndrome</title><source>DOAJ Directory of Open Access Journals</source><source>PubMed Central Open Access</source><source>EZB-FREE-00999 freely available EZB journals</source><source>PubMed Central</source><creator>Davari, Mohammad Hossein ; Kazemi, Toba ; Alimirzaei, Hossein ; Rezvani, Mohamad Reza</creator><creatorcontrib>Davari, Mohammad Hossein ; Kazemi, Toba ; Alimirzaei, Hossein ; Rezvani, Mohamad Reza</creatorcontrib><description>Marfan's syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, three sons, and five grandchildren). The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mitral regurgitation. At ten years' follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive.</description><identifier>ISSN: 1735-5370</identifier><identifier>EISSN: 2008-2371</identifier><identifier>PMID: 23074603</identifier><language>eng</language><publisher>Iran: Tehran University of Medical Sciences</publisher><subject>Case Report</subject><ispartof>The journal of Tehran Heart Center, 2011, Vol.6 (1), p.37-40</ispartof><rights>Copyright © Tehran Heart Center, Tehran University of Medical Sciences 2011</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3466859/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3466859/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,885,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23074603$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Davari, Mohammad Hossein</creatorcontrib><creatorcontrib>Kazemi, Toba</creatorcontrib><creatorcontrib>Alimirzaei, Hossein</creatorcontrib><creatorcontrib>Rezvani, Mohamad Reza</creatorcontrib><title>Cardiovascular Manifestation of a Family with Marfan's Syndrome</title><title>The journal of Tehran Heart Center</title><addtitle>J Tehran Heart Cent</addtitle><description>Marfan's syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, three sons, and five grandchildren). The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mitral regurgitation. At ten years' follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive.</description><subject>Case Report</subject><issn>1735-5370</issn><issn>2008-2371</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><recordid>eNpVkM1Kw0AYRQdRbKy-gszOVeDL_HejSLBVqLiw-_AlM2NHkkmYpJW-vQV_0NVdHO5ZnBOSMQCTM66LU5IVmstccg0zcjGO7wCqYEKekxnjoIUCnpG7EpMN_R7HZtdios8Yg3fjhFPoI-09RbrELrQH-hGm7REnj_FmpK-HaFPfuUty5rEd3dX3zslm-bApH_P1y-qpvF_ngzaL3NfMowLnZeMsSi8EF4xLC940hiupikJrRAtCoTJWNqa2uhGFBWtrB4rPye2XdtjVnbONi1PCthpS6DAdqh5D9Z_EsK3e-n3FhVJGLo6C67-C3-dPCf4JyNRdBA</recordid><startdate>2011</startdate><enddate>2011</enddate><creator>Davari, Mohammad Hossein</creator><creator>Kazemi, Toba</creator><creator>Alimirzaei, Hossein</creator><creator>Rezvani, Mohamad Reza</creator><general>Tehran University of Medical Sciences</general><scope>NPM</scope><scope>5PM</scope></search><sort><creationdate>2011</creationdate><title>Cardiovascular Manifestation of a Family with Marfan's Syndrome</title><author>Davari, Mohammad Hossein ; Kazemi, Toba ; Alimirzaei, Hossein ; Rezvani, Mohamad Reza</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p789-fb2fa60ef5ceda5f4434235d0f8c836561177aad046a68d5c8bd7c41d0ddbe063</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Case Report</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Davari, Mohammad Hossein</creatorcontrib><creatorcontrib>Kazemi, Toba</creatorcontrib><creatorcontrib>Alimirzaei, Hossein</creatorcontrib><creatorcontrib>Rezvani, Mohamad Reza</creatorcontrib><collection>PubMed</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>The journal of Tehran Heart Center</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Davari, Mohammad Hossein</au><au>Kazemi, Toba</au><au>Alimirzaei, Hossein</au><au>Rezvani, Mohamad Reza</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cardiovascular Manifestation of a Family with Marfan's Syndrome</atitle><jtitle>The journal of Tehran Heart Center</jtitle><addtitle>J Tehran Heart Cent</addtitle><date>2011</date><risdate>2011</risdate><volume>6</volume><issue>1</issue><spage>37</spage><epage>40</epage><pages>37-40</pages><issn>1735-5370</issn><eissn>2008-2371</eissn><abstract>Marfan's syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, three sons, and five grandchildren). The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mitral regurgitation. At ten years' follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive.</abstract><cop>Iran</cop><pub>Tehran University of Medical Sciences</pub><pmid>23074603</pmid><tpages>4</tpages><oa>free_for_read</oa></addata></record>
fulltext fulltext
identifier ISSN: 1735-5370
ispartof The journal of Tehran Heart Center, 2011, Vol.6 (1), p.37-40
issn 1735-5370
2008-2371
language eng
recordid cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_3466859
source DOAJ Directory of Open Access Journals; PubMed Central Open Access; EZB-FREE-00999 freely available EZB journals; PubMed Central
subjects Case Report
title Cardiovascular Manifestation of a Family with Marfan's Syndrome
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-06T04%3A34%3A59IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-pubmed&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Cardiovascular%20Manifestation%20of%20a%20Family%20with%20Marfan's%20Syndrome&rft.jtitle=The%20journal%20of%20Tehran%20Heart%20Center&rft.au=Davari,%20Mohammad%20Hossein&rft.date=2011&rft.volume=6&rft.issue=1&rft.spage=37&rft.epage=40&rft.pages=37-40&rft.issn=1735-5370&rft.eissn=2008-2371&rft_id=info:doi/&rft_dat=%3Cpubmed%3E23074603%3C/pubmed%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_id=info:pmid/23074603&rfr_iscdi=true