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<meta name="keywords" content="C4476982, bicuspid aortic valve, two raphe, bicuspid aortic valve, two-raphe, congenital abnormality, double raphe bicuspid aortic valve, two raphe bicuspid aortic valve, two-raphe bicuspid aortic valve, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="A type of bicuspid aortic valvue (BAV) characterized by the presence of two raphes that each extend from the commissure to the free edge of the two underdeveloped, conjoint cusps. This type of BAV has developmental anlagen of three cusps, commissures, and sinuses, but two commissures are more or less malformed and obliterated, giving rise to a raphe, a fibrous ridge, which extends from the commissure to the free edge of the two underdeveloped, conjoint cusps. This type of BAV is typically associated with a high degree of aortic stenosis." /><meta name="robots" content="index,nofollow,noarchive" />
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<title>Two-raphe bicuspid aortic valve (Concept Id: C4476982)
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<!--
UID=1376892
ConceptID=C4476982
-->
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Two-raphe bicuspid aortic valve</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1376892</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information."><span class="highlight" style="background-color:">C4476982</span></a></dd><dt><span class="dotprefix"></span></dt><dd>Congenital Abnormality</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
<td>Bicuspid Aortic Valve, Two Raphe; Bicuspid Aortic Valve, Two-Raphe; Double Raphe Bicuspid Aortic Valve; Two Raphe Bicuspid Aortic Valve; Two-Raphe Bicuspid Aortic Valve</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0031122">HP:0031122</a></td></tr>
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<div class="portlet mgSection" id="ID_100">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">A type of bicuspid aortic valvue (BAV) characterized by the presence of two raphes that each extend from the commissure to the free edge of the two underdeveloped, conjoint cusps. This type of BAV has developmental anlagen of three cusps, commissures, and sinuses, but two commissures are more or less malformed and obliterated, giving rise to a raphe, a fibrous ridge, which extends from the commissure to the free edge of the two underdeveloped, conjoint cusps. This type of BAV is typically associated with a high degree of aortic stenosis. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
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<div class="portlet mgSection" id="ID_118">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test,  </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test,  </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM,  </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>,  </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar  </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="TLline">Two-raphe bicuspid aortic valve</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/892473" ref="tree=MeSH" title="MedGen record for Abnormal cardiovascular system morphology">Abnormal cardiovascular system morphology</a></span><ul><li><span class="TLline"><a href="/medgen/6748" ref="tree=MeSH" title="MedGen record for Abnormal heart morphology">Abnormal heart morphology</a></span><ul><li><span class="TLline"><a href="/medgen/892837" ref="tree=MeSH" title="MedGen record for Abnormal heart valve morphology">Abnormal heart valve morphology</a></span><ul><li><span class="TLline"><a href="/medgen/488999" ref="tree=MeSH" title="MedGen record for Abnormal aortic valve morphology">Abnormal aortic valve morphology</a></span><ul><li><span class="TLline"><a href="/medgen/1623463" ref="tree=MeSH" title="MedGen record for Abnormal aortic valve cusp morphology">Abnormal aortic valve cusp morphology</a></span><ul><li><span class="TLline"><a href="/medgen/57436" ref="tree=MeSH" title="MedGen record for Bicuspid aortic valve">Bicuspid aortic valve</a></span><ul><li><span class="matched_ds">Two-raphe bicuspid aortic valve</span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div></div></div>
</div>
<div class="portlet mgSection" id="ID_105">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
<div class="nl"><a target="_blank" href="/pubmed/36322642">2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Isselbacher EM,
Preventza O,
Hamilton Black J 3rd,
Augoustides JG,
Beck AW,
Bolen MA,
Braverman AC,
Bray BE,
Brown-Zimmerman MM,
Chen EP,
Collins TJ,
DeAnda A Jr,
Fanola CL,
Girardi LN,
Hicks CW,
Hui DS,
Schuyler Jones W,
Kalahasti V,
Kim KM,
Milewicz DM,
Oderich GS,
Ogbechie L,
Promes SB,
Gyang Ross E,
Schermerhorn ML,
Singleton Times S,
Tseng EE,
Wang GJ,
Woo YJ;
Peer Review Committee Members</span><br />
<span class="medgenPMjournal">Circulation</span>
2022 Dec 13;146(24):e334-e482.
Epub 2022 Nov 2
doi: 10.1161/CIR.0000000000001106.
<span class="bold">PMID: </span><a href="/pubmed/36322642" target="_blank">36322642</a><a href="/pmc/articles/PMC9876736" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33332150">2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Otto CM,
Nishimura RA,
Bonow RO,
Carabello BA,
Erwin JP 3rd,
Gentile F,
Jneid H,
Krieger EV,
Mack M,
McLeod C,
O'Gara PT,
Rigolin VH,
Sundt TM 3rd,
Thompson A,
Toly C</span><br />
<span class="medgenPMjournal">Circulation</span>
2021 Feb 2;143(5):e72-e227.
Epub 2020 Dec 17
doi: 10.1161/CIR.0000000000000923.
<span class="bold">PMID: </span><a href="/pubmed/33332150" target="_blank">33332150</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33332149">2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Otto CM,
Nishimura RA,
Bonow RO,
Carabello BA,
Erwin JP 3rd,
Gentile F,
Jneid H,
Krieger EV,
Mack M,
McLeod C,
O'Gara PT,
Rigolin VH,
Sundt TM 3rd,
Thompson A,
Toly C</span><br />
<span class="medgenPMjournal">Circulation</span>
2021 Feb 2;143(5):e35-e71.
Epub 2020 Dec 17
doi: 10.1161/CIR.0000000000000932.
<span class="bold">PMID: </span><a href="/pubmed/33332149" target="_blank">33332149</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(two-raphe%20bicuspid%20aortic%20valve)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (93)</a></div></div>
</div>
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
<div class="portlet mgSection" id="ID_103">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
<div class="nl"><a target="_blank" href="/pubmed/38747246">Transcatheter aortic valve implantation in low-risk tricuspid or bicuspid aortic stenosis: the NOTION-2 trial.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Jørgensen TH,
Thyregod HGH,
Savontaus M,
Willemen Y,
Bleie Ø,
Tang M,
Niemela M,
Angerås O,
Gudmundsdóttir IJ,
Sartipy U,
Dagnegaard H,
Laine M,
Rück A,
Piuhola J,
Petursson P,
Christiansen EH,
Malmberg M,
Olsen PS,
Haaverstad R,
Sondergaard L,
De Backer O;
NOTION-2 investigators</span><br />
<span class="medgenPMjournal">Eur Heart J</span>
2024 Oct 5;45(37):3804-3814.
doi: 10.1093/eurheartj/ehae331.
<span class="bold">PMID: </span><a href="/pubmed/38747246" target="_blank">38747246</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36718616">Bicuspid aortic valve associated aortopathy: 2022 guideline update.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Verma R,
Cohen G,
Colbert J,
Fedak PWM</span><br />
<span class="medgenPMjournal">Curr Opin Cardiol</span>
2023 Mar 1;38(2):61-67.
Epub 2022 Dec 29
doi: 10.1097/HCO.0000000000001020.
<span class="bold">PMID: </span><a href="/pubmed/36718616" target="_blank">36718616</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36400067">Valvular Heart Disease: New Concepts in Pathophysiology and Therapeutic Approaches.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Eleid MF,
Nkomo VT,
Pislaru SV,
Gersh BJ</span><br />
<span class="medgenPMjournal">Annu Rev Med</span>
2023 Jan 27;74:155-170.
Epub 2022 Nov 18
doi: 10.1146/annurev-med-042921-122533.
<span class="bold">PMID: </span><a href="/pubmed/36400067" target="_blank">36400067</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34686263">Thoracic Aortic Aneurysm: A Clinical Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Senser EM,
Misra S,
Henkin S</span><br />
<span class="medgenPMjournal">Cardiol Clin</span>
2021 Nov;39(4):505-515.
doi: 10.1016/j.ccl.2021.06.003.
<span class="bold">PMID: </span><a href="/pubmed/34686263" target="_blank">34686263</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32599026">Genetics in bicuspid aortic valve disease: Where are we?</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bravo-Jaimes K,
Prakash SK</span><br />
<span class="medgenPMjournal">Prog Cardiovasc Dis</span>
2020 Jul-Aug;63(4):398-406.
Epub 2020 Jun 27
doi: 10.1016/j.pcad.2020.06.005.
<span class="bold">PMID: </span><a href="/pubmed/32599026" target="_blank">32599026</a><a href="/pmc/articles/PMC7530017" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Two-raphe%20bicuspid%20aortic%20valve%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (1829)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/36718616">Bicuspid aortic valve associated aortopathy: 2022 guideline update.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Verma R,
Cohen G,
Colbert J,
Fedak PWM</span><br />
<span class="medgenPMjournal">Curr Opin Cardiol</span>
2023 Mar 1;38(2):61-67.
Epub 2022 Dec 29
doi: 10.1097/HCO.0000000000001020.
<span class="bold">PMID: </span><a href="/pubmed/36718616" target="_blank">36718616</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36322642">2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Isselbacher EM,
Preventza O,
Hamilton Black J 3rd,
Augoustides JG,
Beck AW,
Bolen MA,
Braverman AC,
Bray BE,
Brown-Zimmerman MM,
Chen EP,
Collins TJ,
DeAnda A Jr,
Fanola CL,
Girardi LN,
Hicks CW,
Hui DS,
Schuyler Jones W,
Kalahasti V,
Kim KM,
Milewicz DM,
Oderich GS,
Ogbechie L,
Promes SB,
Gyang Ross E,
Schermerhorn ML,
Singleton Times S,
Tseng EE,
Wang GJ,
Woo YJ;
Peer Review Committee Members</span><br />
<span class="medgenPMjournal">Circulation</span>
2022 Dec 13;146(24):e334-e482.
Epub 2022 Nov 2
doi: 10.1161/CIR.0000000000001106.
<span class="bold">PMID: </span><a href="/pubmed/36322642" target="_blank">36322642</a><a href="/pmc/articles/PMC9876736" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34686263">Thoracic Aortic Aneurysm: A Clinical Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Senser EM,
Misra S,
Henkin S</span><br />
<span class="medgenPMjournal">Cardiol Clin</span>
2021 Nov;39(4):505-515.
doi: 10.1016/j.ccl.2021.06.003.
<span class="bold">PMID: </span><a href="/pubmed/34686263" target="_blank">34686263</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32380025">State-of-the art bicuspid aortic valve repair in 2020.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ehrlich T,
de Kerchove L,
Vojacek J,
Boodhwani M,
El-Hamamsy I,
De Paulis R,
Lansac E,
Bavaria JE,
El Khoury G,
Schäfers HJ</span><br />
<span class="medgenPMjournal">Prog Cardiovasc Dis</span>
2020 Jul-Aug;63(4):457-464.
Epub 2020 May 5
doi: 10.1016/j.pcad.2020.04.010.
<span class="bold">PMID: </span><a href="/pubmed/32380025" target="_blank">32380025</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28871571">Acute aortic dissection: pathogenesis, risk factors and diagnosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Gawinecka J,
Schönrath F,
von Eckardstein A</span><br />
<span class="medgenPMjournal">Swiss Med Wkly</span>
2017;147:w14489.
Epub 2017 Aug 25
doi: 10.4414/smw.2017.14489.
<span class="bold">PMID: </span><a href="/pubmed/28871571" target="_blank">28871571</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Two-raphe%20bicuspid%20aortic%20valve%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (1193)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/38747246">Transcatheter aortic valve implantation in low-risk tricuspid or bicuspid aortic stenosis: the NOTION-2 trial.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Jørgensen TH,
Thyregod HGH,
Savontaus M,
Willemen Y,
Bleie Ø,
Tang M,
Niemela M,
Angerås O,
Gudmundsdóttir IJ,
Sartipy U,
Dagnegaard H,
Laine M,
Rück A,
Piuhola J,
Petursson P,
Christiansen EH,
Malmberg M,
Olsen PS,
Haaverstad R,
Sondergaard L,
De Backer O;
NOTION-2 investigators</span><br />
<span class="medgenPMjournal">Eur Heart J</span>
2024 Oct 5;45(37):3804-3814.
doi: 10.1093/eurheartj/ehae331.
<span class="bold">PMID: </span><a href="/pubmed/38747246" target="_blank">38747246</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33972115">2020 ACC/AHA guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Writing Committee Members,
Otto CM,
Nishimura RA,
Bonow RO,
Carabello BA,
Erwin JP 3rd,
Gentile F,
Jneid H,
Krieger EV,
Mack M,
McLeod C,
O'Gara PT,
Rigolin VH,
Sundt TM 3rd,
Thompson A,
Toly C;
ACC/AHA Joint Committee Members,
O'Gara PT,
Beckman JA,
Levine GN,
Al-Khatib SM,
Armbruster A,
Birtcher KK,
Ciggaroa J,
Deswal A,
Dixon DL,
Fleisher LA,
de las Fuentes L,
Gentile F,
Goldberger ZD,
Gorenek B,
Haynes N,
Hernandez AF,
Hlatky MA,
Joglar JA,
Jones WS,
Marine JE,
Mark D,
Palaniappan L,
Piano MR,
Spatz ES,
Tamis-Holland J,
Wijeysundera DN,
Woo YJ</span><br />
<span class="medgenPMjournal">J Thorac Cardiovasc Surg</span>
2021 Aug;162(2):e183-e353.
Epub 2021 May 8
doi: 10.1016/j.jtcvs.2021.04.002.
<span class="bold">PMID: </span><a href="/pubmed/33972115" target="_blank">33972115</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33683945">Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Stenosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Vincent F,
Ternacle J,
Denimal T,
Shen M,
Redfors B,
Delhaye C,
Simonato M,
Debry N,
Verdier B,
Shahim B,
Pamart T,
Spillemaeker H,
Schurtz G,
Pontana F,
Thourani VH,
Pibarot P,
Van Belle E</span><br />
<span class="medgenPMjournal">Circulation</span>
2021 Mar 9;143(10):1043-1061.
Epub 2021 Mar 8
doi: 10.1161/CIRCULATIONAHA.120.048048.
<span class="bold">PMID: </span><a href="/pubmed/33683945" target="_blank">33683945</a></div>
<div class="nl"><a target="_blank" href="/pubmed/29622096">Management of Patients With Aortic Valve Stenosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kanwar A,
Thaden JJ,
Nkomo VT</span><br />
<span class="medgenPMjournal">Mayo Clin Proc</span>
2018 Apr;93(4):488-508.
doi: 10.1016/j.mayocp.2018.01.020.
<span class="bold">PMID: </span><a href="/pubmed/29622096" target="_blank">29622096</a></div>
<div class="nl"><a target="_blank" href="/pubmed/25173340">2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC).</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Erbel R,
Aboyans V,
Boileau C,
Bossone E,
Bartolomeo RD,
Eggebrecht H,
Evangelista A,
Falk V,
Frank H,
Gaemperli O,
Grabenwöger M,
Haverich A,
Iung B,
Manolis AJ,
Meijboom F,
Nienaber CA,
Roffi M,
Rousseau H,
Sechtem U,
Sirnes PA,
Allmen RS,
Vrints CJ;
ESC Committee for Practice Guidelines</span><br />
<span class="medgenPMjournal">Eur Heart J</span>
2014 Nov 1;35(41):2873-926.
Epub 2014 Aug 29
doi: 10.1093/eurheartj/ehu281.
<span class="bold">PMID: </span><a href="/pubmed/25173340" target="_blank">25173340</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Two-raphe%20bicuspid%20aortic%20valve%22%20AND%20Therapy%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (615)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/38253300">Bicuspid aortic valve: The most frequent and not so benign congenital heart disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Thiene G,
Rizzo S,
Basso C</span><br />
<span class="medgenPMjournal">Cardiovasc Pathol</span>
2024 May-Jun;70:107604.
Epub 2024 Jan 20
doi: 10.1016/j.carpath.2024.107604.
<span class="bold">PMID: </span><a href="/pubmed/38253300" target="_blank">38253300</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34686263">Thoracic Aortic Aneurysm: A Clinical Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Senser EM,
Misra S,
Henkin S</span><br />
<span class="medgenPMjournal">Cardiol Clin</span>
2021 Nov;39(4):505-515.
doi: 10.1016/j.ccl.2021.06.003.
<span class="bold">PMID: </span><a href="/pubmed/34686263" target="_blank">34686263</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32854836">Bicuspid Aortic Valve Morphology and Outcomes After Transcatheter Aortic Valve Replacement.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Yoon SH,
Kim WK,
Dhoble A,
Milhorini Pio S,
Babaliaros V,
Jilaihawi H,
Pilgrim T,
De Backer O,
Bleiziffer S,
Vincent F,
Shmidt T,
Butter C,
Kamioka N,
Eschenbach L,
Renker M,
Asami M,
Lazkani M,
Fujita B,
Birs A,
Barbanti M,
Pershad A,
Landes U,
Oldemeyer B,
Kitamura M,
Oakley L,
Ochiai T,
Chakravarty T,
Nakamura M,
Ruile P,
Deuschl F,
Berman D,
Modine T,
Ensminger S,
Kornowski R,
Lange R,
McCabe JM,
Williams MR,
Whisenant B,
Delgado V,
Windecker S,
Van Belle E,
Sondergaard L,
Chevalier B,
Mack M,
Bax JJ,
Leon MB,
Makkar RR;
Bicuspid Aortic Valve Stenosis Transcatheter Aortic Valve Replacement Registry Investigators</span><br />
<span class="medgenPMjournal">J Am Coll Cardiol</span>
2020 Sep 1;76(9):1018-1030.
doi: 10.1016/j.jacc.2020.07.005.
<span class="bold">PMID: </span><a href="/pubmed/32854836" target="_blank">32854836</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32380025">State-of-the art bicuspid aortic valve repair in 2020.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ehrlich T,
de Kerchove L,
Vojacek J,
Boodhwani M,
El-Hamamsy I,
De Paulis R,
Lansac E,
Bavaria JE,
El Khoury G,
Schäfers HJ</span><br />
<span class="medgenPMjournal">Prog Cardiovasc Dis</span>
2020 Jul-Aug;63(4):457-464.
Epub 2020 May 5
doi: 10.1016/j.pcad.2020.04.010.
<span class="bold">PMID: </span><a href="/pubmed/32380025" target="_blank">32380025</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30366931">Heartbeat: Improving diagnosis and management of aortic valve disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Otto CM</span><br />
<span class="medgenPMjournal">Heart</span>
2018 Nov;104(22):1807-1809.
doi: 10.1136/heartjnl-2018-314232.
<span class="bold">PMID: </span><a href="/pubmed/30366931" target="_blank">30366931</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Two-raphe%20bicuspid%20aortic%20valve%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (1069)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/38747246">Transcatheter aortic valve implantation in low-risk tricuspid or bicuspid aortic stenosis: the NOTION-2 trial.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Jørgensen TH,
Thyregod HGH,
Savontaus M,
Willemen Y,
Bleie Ø,
Tang M,
Niemela M,
Angerås O,
Gudmundsdóttir IJ,
Sartipy U,
Dagnegaard H,
Laine M,
Rück A,
Piuhola J,
Petursson P,
Christiansen EH,
Malmberg M,
Olsen PS,
Haaverstad R,
Sondergaard L,
De Backer O;
NOTION-2 investigators</span><br />
<span class="medgenPMjournal">Eur Heart J</span>
2024 Oct 5;45(37):3804-3814.
doi: 10.1093/eurheartj/ehae331.
<span class="bold">PMID: </span><a href="/pubmed/38747246" target="_blank">38747246</a></div>
<div class="nl"><a target="_blank" href="/pubmed/37611071">Bicuspid aortic valve: long-term morbidity and mortality.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Yang LT,
Ye Z,
Wajih Ullah M,
Maleszewski JJ,
Scott CG,
Padang R,
Pislaru SV,
Nkomo VT,
Mankad SV,
Pellikka PA,
Oh JK,
Roger VL,
Enriquez-Sarano M,
Michelena HI</span><br />
<span class="medgenPMjournal">Eur Heart J</span>
2023 Nov 14;44(43):4549-4562.
doi: 10.1093/eurheartj/ehad477.
<span class="bold">PMID: </span><a href="/pubmed/37611071" target="_blank">37611071</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36718616">Bicuspid aortic valve associated aortopathy: 2022 guideline update.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Verma R,
Cohen G,
Colbert J,
Fedak PWM</span><br />
<span class="medgenPMjournal">Curr Opin Cardiol</span>
2023 Mar 1;38(2):61-67.
Epub 2022 Dec 29
doi: 10.1097/HCO.0000000000001020.
<span class="bold">PMID: </span><a href="/pubmed/36718616" target="_blank">36718616</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34686263">Thoracic Aortic Aneurysm: A Clinical Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Senser EM,
Misra S,
Henkin S</span><br />
<span class="medgenPMjournal">Cardiol Clin</span>
2021 Nov;39(4):505-515.
doi: 10.1016/j.ccl.2021.06.003.
<span class="bold">PMID: </span><a href="/pubmed/34686263" target="_blank">34686263</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32380025">State-of-the art bicuspid aortic valve repair in 2020.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ehrlich T,
de Kerchove L,
Vojacek J,
Boodhwani M,
El-Hamamsy I,
De Paulis R,
Lansac E,
Bavaria JE,
El Khoury G,
Schäfers HJ</span><br />
<span class="medgenPMjournal">Prog Cardiovasc Dis</span>
2020 Jul-Aug;63(4):457-464.
Epub 2020 May 5
doi: 10.1016/j.pcad.2020.04.010.
<span class="bold">PMID: </span><a href="/pubmed/32380025" target="_blank">32380025</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Two-raphe%20bicuspid%20aortic%20valve%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (995)</a></div></div>
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<div class="nl"><a target="_blank" href="/pubmed/37487404">Transcatheter Aortic Valve Replacement in Bicuspid Versus Tricuspid Aortic Valve Stenosis: Meta-Analysis and Systemic Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Saeed Al-Asad K,
Martinez Salazar A,
Radwan Y,
Wang E,
Salam MF,
Sabanci R,
Saeed M,
Halboni A,
Al-Abcha A,
Abela G</span><br />
<span class="medgenPMjournal">Am J Cardiol</span>
2023 Sep 15;203:105-112.
Epub 2023 Jul 22
doi: 10.1016/j.amjcard.2023.06.120.
<span class="bold">PMID: </span><a href="/pubmed/37487404" target="_blank">37487404</a></div>
<div class="nl"><a target="_blank" href="/pubmed/37288540">Family screening for bicuspid aortic valve and aortic dilatation: a meta-analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bray JJH,
Freer R,
Pitcher A,
Kharbanda R</span><br />
<span class="medgenPMjournal">Eur Heart J</span>
2023 Sep 1;44(33):3152-3164.
doi: 10.1093/eurheartj/ehad320.
<span class="bold">PMID: </span><a href="/pubmed/37288540" target="_blank">37288540</a><a href="/pmc/articles/PMC10702468" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30239633">Haemodynamic assessment of bicuspid aortic valve aortopathy: a systematic review of the current literature.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Edlin J,
Youssefi P,
Bilkhu R,
Figueroa CA,
Morgan R,
Nowell J,
Jahangiri M</span><br />
<span class="medgenPMjournal">Eur J Cardiothorac Surg</span>
2019 Apr 1;55(4):610-617.
doi: 10.1093/ejcts/ezy312.
<span class="bold">PMID: </span><a href="/pubmed/30239633" target="_blank">30239633</a></div>
<div class="nl"><a target="_blank" href="/pubmed/29399873">Bicuspid aortic valve morphology and its impact on aortic diameters-A systematic review with meta-analysis and meta-regression.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Miśkowiec D,
Lipiec P,
Szymczyk E,
Wejner-Mik P,
Michalski B,
Kupczyńska K,
Wierzbowska-Drabik K,
Kasprzak JD</span><br />
<span class="medgenPMjournal">Echocardiography</span>
2018 May;35(5):667-677.
Epub 2018 Feb 4
doi: 10.1111/echo.13818.
<span class="bold">PMID: </span><a href="/pubmed/29399873" target="_blank">29399873</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28490615">Contemporary natural history of bicuspid aortic valve disease: a systematic review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Masri A,
Svensson LG,
Griffin BP,
Desai MY</span><br />
<span class="medgenPMjournal">Heart</span>
2017 Sep;103(17):1323-1330.
Epub 2017 May 10
doi: 10.1136/heartjnl-2016-309916.
<span class="bold">PMID: </span><a href="/pubmed/28490615" target="_blank">28490615</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Two-raphe%20bicuspid%20aortic%20valve%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (43)</a></div></div>
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<h2 class="offscreen_noflow">Supplemental Content</h2>
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<div class="portlet_content ln"><ul><li><a href="https://clinicaltrials.gov/search?cond=Two-raphe%20bicuspid%20aortic%20valve" target="_blank">ClinicalTrials.gov</a></li></ul></div>
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<div class="portlet_content ln"><ul class="a_poppers"><li><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(two-raphe%20bicuspid%20aortic%20valve)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">PubMed</a><div class="help-popup">See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li><li><a target="_blank" href="/books/?term=((%22clinical%20guidelines%22%5BResource%20Type%5D)%20OR%20%22practice%20guideline%22%5BPublication%20Type%5D)%20AND%20(%22Two-raphe%20bicuspid%20aortic%20valve%22)">Bookshelf</a><div class="help-popup">See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li></ul></div>
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