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Bicuspid aortic valve with right-noncoronary cusp fusion

MedGen UID:
1385812
•Concept ID:
C4476980
•
Congenital Abnormality
HPO: HP:0031120

Definition

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

Term Hierarchy

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

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
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
Machine-learning phenotypic classification of bicuspid aortopathy.
Wojnarski CM, Roselli EE, Idrees JJ, Zhu Y, Carnes TA, Lowry AM, Collier PH, Griffin B, Ehrlinger J, Blackstone EH, Svensson LG, Lytle BW
J Thorac Cardiovasc Surg 2018 Feb;155(2):461-469.e4. Epub 2017 Sep 14 doi: 10.1016/j.jtcvs.2017.08.123. PMID: 29042101

Diagnosis

The Impact of Bicuspid Aortic Valve Leaflet Fusion Morphology on the Ascending Aorta and on Outcomes of Aortic Valve Replacement.
Bibevski S, Ruzmetov M, Plate JF, Scholl FG
Tex Heart Inst J 2023 Mar 1;50(2) doi: 10.14503/THIJ-21-7831. PMID: 36972539Free PMC Article
Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology.
Benvenga RM, Tribouilloy C, Michelena HI, Silverio A, Arregle F, Martel H, Denev S, Bohbot Y, Hubert S, Renard S, Camoin L, Casalta AC, Casalta JP, Gouriet F, Riberi A, Lepidi H, Collart F, Raoult D, Drancourt M, Galasso G, DeSimone DC, Citro R, Habib G
J Am Soc Echocardiogr 2023 Jul;36(7):760-768. Epub 2023 Jan 20 doi: 10.1016/j.echo.2023.01.010. PMID: 36682434
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
Machine-learning phenotypic classification of bicuspid aortopathy.
Wojnarski CM, Roselli EE, Idrees JJ, Zhu Y, Carnes TA, Lowry AM, Collier PH, Griffin B, Ehrlinger J, Blackstone EH, Svensson LG, Lytle BW
J Thorac Cardiovasc Surg 2018 Feb;155(2):461-469.e4. Epub 2017 Sep 14 doi: 10.1016/j.jtcvs.2017.08.123. PMID: 29042101

Therapy

Machine-learning phenotypic classification of bicuspid aortopathy.
Wojnarski CM, Roselli EE, Idrees JJ, Zhu Y, Carnes TA, Lowry AM, Collier PH, Griffin B, Ehrlinger J, Blackstone EH, Svensson LG, Lytle BW
J Thorac Cardiovasc Surg 2018 Feb;155(2):461-469.e4. Epub 2017 Sep 14 doi: 10.1016/j.jtcvs.2017.08.123. PMID: 29042101
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
A Regulated Trial of Bicuspid Aortic Valve Repair Supported by Geometric Ring Annuloplasty.
Mazzitelli D, Pfeiffer S, Rankin JS, Fischlein T, Choi YH, Wahlers T, Nöbauer C, Schreiber C, Lange R
Ann Thorac Surg 2015 Jun;99(6):2010-6. Epub 2015 Apr 10 doi: 10.1016/j.athoracsur.2015.01.050. PMID: 25865762

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
Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology.
Benvenga RM, Tribouilloy C, Michelena HI, Silverio A, Arregle F, Martel H, Denev S, Bohbot Y, Hubert S, Renard S, Camoin L, Casalta AC, Casalta JP, Gouriet F, Riberi A, Lepidi H, Collart F, Raoult D, Drancourt M, Galasso G, DeSimone DC, Citro R, Habib G
J Am Soc Echocardiogr 2023 Jul;36(7):760-768. Epub 2023 Jan 20 doi: 10.1016/j.echo.2023.01.010. PMID: 36682434
Machine-learning phenotypic classification of bicuspid aortopathy.
Wojnarski CM, Roselli EE, Idrees JJ, Zhu Y, Carnes TA, Lowry AM, Collier PH, Griffin B, Ehrlinger J, Blackstone EH, Svensson LG, Lytle BW
J Thorac Cardiovasc Surg 2018 Feb;155(2):461-469.e4. Epub 2017 Sep 14 doi: 10.1016/j.jtcvs.2017.08.123. PMID: 29042101
A Regulated Trial of Bicuspid Aortic Valve Repair Supported by Geometric Ring Annuloplasty.
Mazzitelli D, Pfeiffer S, Rankin JS, Fischlein T, Choi YH, Wahlers T, Nöbauer C, Schreiber C, Lange R
Ann Thorac Surg 2015 Jun;99(6):2010-6. Epub 2015 Apr 10 doi: 10.1016/j.athoracsur.2015.01.050. PMID: 25865762
Patterns of aortic dilatation in bicuspid aortic valve-associated aortopathy.
Khoo C, Cheung C, Jue J
J Am Soc Echocardiogr 2013 Jun;26(6):600-5. Epub 2013 Apr 3 doi: 10.1016/j.echo.2013.02.017. PMID: 23562085

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
Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology.
Benvenga RM, Tribouilloy C, Michelena HI, Silverio A, Arregle F, Martel H, Denev S, Bohbot Y, Hubert S, Renard S, Camoin L, Casalta AC, Casalta JP, Gouriet F, Riberi A, Lepidi H, Collart F, Raoult D, Drancourt M, Galasso G, DeSimone DC, Citro R, Habib G
J Am Soc Echocardiogr 2023 Jul;36(7):760-768. Epub 2023 Jan 20 doi: 10.1016/j.echo.2023.01.010. PMID: 36682434
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
Machine-learning phenotypic classification of bicuspid aortopathy.
Wojnarski CM, Roselli EE, Idrees JJ, Zhu Y, Carnes TA, Lowry AM, Collier PH, Griffin B, Ehrlinger J, Blackstone EH, Svensson LG, Lytle BW
J Thorac Cardiovasc Surg 2018 Feb;155(2):461-469.e4. Epub 2017 Sep 14 doi: 10.1016/j.jtcvs.2017.08.123. PMID: 29042101
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

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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