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Bicuspid aortic valve with right-left cusp fusion

MedGen UID:
1387510
•Concept ID:
C4476979
•
Congenital Abnormality
HPO: HP:0031119

Definition

A type of bicuspid aortic valve (BAV) characterized by a single raphe between the right and left cusps (RL fusion pattern). This results in two leaflefts with an anterior-posterior leaflet orientation (also called the typical pattern). There is thus one completely developed noncoronary cusp, two completely developed commissures, and one raphe between the underdeveloped left and right coronary cusps extending to the corresponding malformed commissure. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBicuspid aortic valve with right-left cusp fusion

Professional guidelines

PubMed

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):e383-e414. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.06.019. PMID: 34304896
International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
Ann Thorac Surg 2021 Sep;112(3):e203-e235. Epub 2021 Jul 22 doi: 10.1016/j.athoracsur.2020.08.119. PMID: 34304860
International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ; Endorsed by the Heart Valve Society (HVS), European Association of Cardiovascular Imaging (EACVI), Society of Thoracic Surgeons (STS), American Association for Thoracic Surgery (AATS), Society for Cardiovascular Magnetic Resonance (SCMR), Society of Cardiovascular Computed Tomography (SCCT), North American Society for Cardiovascular Imaging (NASCI) and the International Bicuspid Aortic Valve Consortium (BAVCon)
Eur J Cardiothorac Surg 2021 Sep 11;60(3):448-476. doi: 10.1093/ejcts/ezab038. PMID: 34293102

Recent clinical studies

Etiology

Unlocking insights in bicuspid aortic valve management in adult patients: the vital role of cardiac imaging.
Di Michele S, Parato VM, Di Giannuario G, Kholaif N, Al Admawi M, Aljheish S, Arbili L, Alshammari A, Parato AG, Al Sergani H
Monaldi Arch Chest Dis 2023 Sep 15;94(1) doi: 10.4081/monaldi.2023.2761. PMID: 37721026
Serial echocardiography for valve dysfunction and aortic dilation in bicuspid aortic valves.
Parker MW, Muchnik RD, Ogunsua A, Kundu A, Lakshmanan S, Shah N, Dickey JB, Gentile BA, Pape LA
Echocardiography 2021 Mar;38(3):394-401. Epub 2021 Feb 14 doi: 10.1111/echo.15000. PMID: 33586263
Distribution Patterns of Valvular and Vascular Complications in Bicuspid Aortic Valve.
Wang Y, Wu B, Li J, Liu H, Shu X
Int Heart J 2020 Mar 28;61(2):273-280. Epub 2020 Mar 14 doi: 10.1536/ihj.19-467. PMID: 32173708
Bicuspid aortic valve morphology and its impact on aortic diameters-A systematic review with meta-analysis and meta-regression.
Miśkowiec D, Lipiec P, Szymczyk E, Wejner-Mik P, Michalski B, Kupczyńska K, Wierzbowska-Drabik K, Kasprzak JD
Echocardiography 2018 May;35(5):667-677. Epub 2018 Feb 4 doi: 10.1111/echo.13818. PMID: 29399873
Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes.
Niaz T, Poterucha JT, Olson TM, Johnson JN, Craviari C, Nienaber T, Palfreeman J, Cetta F, Hagler DJ
J Am Soc Echocardiogr 2018 Feb;31(2):194-200. Epub 2017 Nov 28 doi: 10.1016/j.echo.2017.10.008. PMID: 29191731

Diagnosis

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):e383-e414. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.06.019. PMID: 34304896
Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, Khoury GE, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):781-797. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.05.008. PMID: 34304894
Bicuspid aortic valve morphology and its impact on aortic diameters-A systematic review with meta-analysis and meta-regression.
Miśkowiec D, Lipiec P, Szymczyk E, Wejner-Mik P, Michalski B, Kupczyńska K, Wierzbowska-Drabik K, Kasprzak JD
Echocardiography 2018 May;35(5):667-677. Epub 2018 Feb 4 doi: 10.1111/echo.13818. PMID: 29399873
Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes.
Niaz T, Poterucha JT, Olson TM, Johnson JN, Craviari C, Nienaber T, Palfreeman J, Cetta F, Hagler DJ
J Am Soc Echocardiogr 2018 Feb;31(2):194-200. Epub 2017 Nov 28 doi: 10.1016/j.echo.2017.10.008. PMID: 29191731
Prevalence and predictors of intracranial aneurysms in patients with bicuspid aortic valve.
Egbe AC, Padang R, Brown RD, Khan AR, Luis SA, Huston J 3rd, Akintoye E, Connolly HM
Heart 2017 Oct;103(19):1508-1514. Epub 2017 Jun 8 doi: 10.1136/heartjnl-2016-311076. PMID: 28596303

Therapy

Bicuspid aortic valve morphology and its association with aortic diameter: an echocardiographic study.
Miśkowiec DŁ, Lipiec P, Kasprzak JD
Kardiol Pol 2016;74(2):151-8. Epub 2015 Jul 23 doi: 10.5603/KP.a2015.0134. PMID: 26202528

Prognosis

Unlocking insights in bicuspid aortic valve management in adult patients: the vital role of cardiac imaging.
Di Michele S, Parato VM, Di Giannuario G, Kholaif N, Al Admawi M, Aljheish S, Arbili L, Alshammari A, Parato AG, Al Sergani H
Monaldi Arch Chest Dis 2023 Sep 15;94(1) doi: 10.4081/monaldi.2023.2761. PMID: 37721026
International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):e383-e414. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.06.019. PMID: 34304896
Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, Khoury GE, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):781-797. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.05.008. PMID: 34304894
Abnormal aortic stiffness in patients with bicuspid aortic valve: phenotypic variation determined by magnetic resonance imaging.
Boonyasirinant T, Rajiah P, Flamm SD
Int J Cardiovasc Imaging 2019 Jan;35(1):133-141. Epub 2018 Sep 5 doi: 10.1007/s10554-018-1433-y. PMID: 30187149
Prevalence and predictors of intracranial aneurysms in patients with bicuspid aortic valve.
Egbe AC, Padang R, Brown RD, Khan AR, Luis SA, Huston J 3rd, Akintoye E, Connolly HM
Heart 2017 Oct;103(19):1508-1514. Epub 2017 Jun 8 doi: 10.1136/heartjnl-2016-311076. PMID: 28596303

Clinical prediction guides

Unlocking insights in bicuspid aortic valve management in adult patients: the vital role of cardiac imaging.
Di Michele S, Parato VM, Di Giannuario G, Kholaif N, Al Admawi M, Aljheish S, Arbili L, Alshammari A, Parato AG, Al Sergani H
Monaldi Arch Chest Dis 2023 Sep 15;94(1) doi: 10.4081/monaldi.2023.2761. PMID: 37721026
International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):e383-e414. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.06.019. PMID: 34304896
Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, Khoury GE, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):781-797. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.05.008. PMID: 34304894
Bicuspid aortic valve morphology and its impact on aortic diameters-A systematic review with meta-analysis and meta-regression.
Miśkowiec D, Lipiec P, Szymczyk E, Wejner-Mik P, Michalski B, Kupczyńska K, Wierzbowska-Drabik K, Kasprzak JD
Echocardiography 2018 May;35(5):667-677. Epub 2018 Feb 4 doi: 10.1111/echo.13818. PMID: 29399873
Prevalence and predictors of intracranial aneurysms in patients with bicuspid aortic valve.
Egbe AC, Padang R, Brown RD, Khan AR, Luis SA, Huston J 3rd, Akintoye E, Connolly HM
Heart 2017 Oct;103(19):1508-1514. Epub 2017 Jun 8 doi: 10.1136/heartjnl-2016-311076. PMID: 28596303

Recent systematic reviews

Bicuspid aortic valve morphology and its impact on aortic diameters-A systematic review with meta-analysis and meta-regression.
Miśkowiec D, Lipiec P, Szymczyk E, Wejner-Mik P, Michalski B, Kupczyńska K, Wierzbowska-Drabik K, Kasprzak JD
Echocardiography 2018 May;35(5):667-677. Epub 2018 Feb 4 doi: 10.1111/echo.13818. PMID: 29399873

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