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Bicuspid Aortic Valve Disease A Comprehensive Review英文文献资料.doc

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Bicuspid Aortic Valve Disease A Comprehensive Review英文文献资料

HindawiPublishingCorporation CardiologyResearchandPractice Volume2012,ArticleID196037,7pages doi:10.1155/2012/196037 ReviewArticle BicuspidAorticValveDisease:AComprehensiveReview IfyMordiandNikolaosTzemos InstituteforCardiovascularResearch,BritishHeartFoundationGlasgowCardiovascularResearchCentre, UniversityofGlasgow,GlasgowG128TA,UK CorrespondenceshouldbeaddressedtoNikolaosTzemos,niko.tzemos@glasgow.ac.uk Received27January2012;Accepted26March2012 AcademicEditor:AniC.Anyanwu Copyright?2012I.MordiandN.Tzemos.ThisisanopenaccessarticledistributedundertheCreativeCommonsAttribution License,whichpermitsunrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalworkisproperly cited. Bicuspidaorticvalveisthecommonestcongenitalcardiacabnormalityinthegeneralpopulation.Thispaperarticlewilldiscuss ourcurrentknowledgeoftheanatomy,pathophysiology,genetics,andclinicalaspectsofbicuspidaorticvalvedisease. 1.Introduction The exact pathogenesis of the formation of bicuspid aorticvalvesisnotyetfullyunderstood.Itisthoughtthereis certainly a genetic component, especially given the associ- ation of BAV with other congenital abnormalities such as coarctation of the aorta. In summary however, the BAV is formedbyfusionoftheaorticcuspsduringvalvulogenesis. Thepulmonaryvalvecanalsobebicuspid,althoughthis ismuchrarerandismostcommonlyassociatedwithcon- genitalheartdiseasesuchasTetralogyofFallot.Therehave beenlessthan10casesreportedintheliteratureofanisolated bicuspidpulmonaryvalve[6]. Bicuspid aortic valve (BAV) is the commonest congenital cardiacabnormalitywithanestimatedprevalenceof1-2% [1].Itisalmost3timesmorecommoninmalesthanfemales [2].AdversecardiovascularoutcomesinpatientswithBAV are more common than previously thought [3], therefore givenitshighprevalenceitpresentspotentiallyalargeburden oncardiovascularcare. This paper will discuss our current knowledge of the anatomy, pathophysiology, genetics, and clinical aspects of BAV disease using echocardiographic literature. On