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Two-raphe bicuspid aortic valve

MedGen UID:
1376892
•Concept ID:
C4476982
•
Congenital Abnormality
Synonyms: 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
 
HPO: HP:0031122

Definition

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 HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Two-raphe bicuspid aortic valve

Professional guidelines

PubMed

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.
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
Circulation 2022 Dec 13;146(24):e334-e482. Epub 2022 Nov 2 doi: 10.1161/CIR.0000000000001106. PMID: 36322642Free PMC Article
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.
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
Circulation 2021 Feb 2;143(5):e35-e71. Epub 2020 Dec 17 doi: 10.1161/CIR.0000000000000932. PMID: 33332149
Management of Patients With Aortic Valve Stenosis.
Kanwar A, Thaden JJ, Nkomo VT
Mayo Clin Proc 2018 Apr;93(4):488-508. doi: 10.1016/j.mayocp.2018.01.020. PMID: 29622096

Recent clinical studies

Etiology

Thoracic Aortic Aneurysm: A Clinical Review.
Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Stenosis.
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
Circulation 2021 Mar 9;143(10):1043-1061. Epub 2021 Mar 8 doi: 10.1161/CIRCULATIONAHA.120.048048. PMID: 33683945
Bicuspid Aortic Valve Morphology and Outcomes After Transcatheter Aortic Valve Replacement.
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
J Am Coll Cardiol 2020 Sep 1;76(9):1018-1030. doi: 10.1016/j.jacc.2020.07.005. PMID: 32854836
Genetics in bicuspid aortic valve disease: Where are we?
Bravo-Jaimes K, Prakash SK
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):398-406. Epub 2020 Jun 27 doi: 10.1016/j.pcad.2020.06.005. PMID: 32599026Free PMC Article
Bicuspid aortic valve: Basics and beyond.
Shah SY, Higgins A, Desai MY
Cleve Clin J Med 2018 Oct;85(10):779-784. doi: 10.3949/ccjm.85a.17069. PMID: 30289756

Diagnosis

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.
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
Circulation 2022 Dec 13;146(24):e334-e482. Epub 2022 Nov 2 doi: 10.1161/CIR.0000000000001106. PMID: 36322642Free PMC Article
Thoracic Aortic Aneurysm: A Clinical Review.
Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Stenosis.
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
Circulation 2021 Mar 9;143(10):1043-1061. Epub 2021 Mar 8 doi: 10.1161/CIRCULATIONAHA.120.048048. PMID: 33683945
State-of-the art bicuspid aortic valve repair in 2020.
Ehrlich T, de Kerchove L, Vojacek J, Boodhwani M, El-Hamamsy I, De Paulis R, Lansac E, Bavaria JE, El Khoury G, Schäfers HJ
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):457-464. Epub 2020 May 5 doi: 10.1016/j.pcad.2020.04.010. PMID: 32380025
Acute aortic dissection: pathogenesis, risk factors and diagnosis.
Gawinecka J, Schönrath F, von Eckardstein A
Swiss Med Wkly 2017;147:w14489. Epub 2017 Aug 25 doi: 10.4414/smw.2017.14489. PMID: 28871571

Therapy

Transcatheter aortic valve implantation in patients with bicuspid valve morphology: a roadmap towards standardization.
Xiong TY, Ali WB, Feng Y, Hayashida K, Jilaihawi H, Latib A, Lee MK, Leon MB, Makkar RR, Modine T, Naber C, Peng Y, Piazza N, Reardon MJ, Redwood S, Seth A, Sondergaard L, Tay E, Tchetche D, Yin WH, Chen M, Prendergast B, Mylotte D
Nat Rev Cardiol 2023 Jan;20(1):52-67. Epub 2022 Jun 20 doi: 10.1038/s41569-022-00734-5. PMID: 35726019
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.
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
J Thorac Cardiovasc Surg 2021 Aug;162(2):e183-e353. Epub 2021 May 8 doi: 10.1016/j.jtcvs.2021.04.002. PMID: 33972115
Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Stenosis.
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
Circulation 2021 Mar 9;143(10):1043-1061. Epub 2021 Mar 8 doi: 10.1161/CIRCULATIONAHA.120.048048. PMID: 33683945
Management of Patients With Aortic Valve Stenosis.
Kanwar A, Thaden JJ, Nkomo VT
Mayo Clin Proc 2018 Apr;93(4):488-508. doi: 10.1016/j.mayocp.2018.01.020. PMID: 29622096
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).
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
Eur Heart J 2014 Nov 1;35(41):2873-926. Epub 2014 Aug 29 doi: 10.1093/eurheartj/ehu281. PMID: 25173340

Prognosis

Thoracic Aortic Aneurysm: A Clinical Review.
Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
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
Clinical Features and Outcomes of Pregnancy-Related Acute Aortic Dissection.
Braverman AC, Mittauer E, Harris KM, Evangelista A, Pyeritz RE, Brinster D, Conklin L, Suzuki T, Fanola C, Ouzounian M, Chen E, Myrmel T, Bekeredjian R, Hutchison S, Coselli J, Gilon D, O'Gara P, Davis M, Isselbacher E, Eagle K
JAMA Cardiol 2021 Jan 1;6(1):58-66. doi: 10.1001/jamacardio.2020.4876. PMID: 33052376Free PMC Article
Bicuspid Aortic Valve Morphology and Outcomes After Transcatheter Aortic Valve Replacement.
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
J Am Coll Cardiol 2020 Sep 1;76(9):1018-1030. doi: 10.1016/j.jacc.2020.07.005. PMID: 32854836
State-of-the art bicuspid aortic valve repair in 2020.
Ehrlich T, de Kerchove L, Vojacek J, Boodhwani M, El-Hamamsy I, De Paulis R, Lansac E, Bavaria JE, El Khoury G, Schäfers HJ
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):457-464. Epub 2020 May 5 doi: 10.1016/j.pcad.2020.04.010. PMID: 32380025

Clinical prediction guides

Transcatheter aortic valve implantation in patients with bicuspid valve morphology: a roadmap towards standardization.
Xiong TY, Ali WB, Feng Y, Hayashida K, Jilaihawi H, Latib A, Lee MK, Leon MB, Makkar RR, Modine T, Naber C, Peng Y, Piazza N, Reardon MJ, Redwood S, Seth A, Sondergaard L, Tay E, Tchetche D, Yin WH, Chen M, Prendergast B, Mylotte D
Nat Rev Cardiol 2023 Jan;20(1):52-67. Epub 2022 Jun 20 doi: 10.1038/s41569-022-00734-5. PMID: 35726019
Thoracic Aortic Aneurysm: A Clinical Review.
Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
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
Bicuspid Aortic Valve Morphology and Outcomes After Transcatheter Aortic Valve Replacement.
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
J Am Coll Cardiol 2020 Sep 1;76(9):1018-1030. doi: 10.1016/j.jacc.2020.07.005. PMID: 32854836
State-of-the art bicuspid aortic valve repair in 2020.
Ehrlich T, de Kerchove L, Vojacek J, Boodhwani M, El-Hamamsy I, De Paulis R, Lansac E, Bavaria JE, El Khoury G, Schäfers HJ
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):457-464. Epub 2020 May 5 doi: 10.1016/j.pcad.2020.04.010. PMID: 32380025

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
Prevalence of Bicuspid Aortic Valve in Chinese Patients with Aortic Valve Disease: A Systematic Review.
Wang X, Wang W
J Heart Valve Dis 2017 May;26(3):274-280. PMID: 29092111
Contemporary natural history of bicuspid aortic valve disease: a systematic review.
Masri A, Svensson LG, Griffin BP, Desai MY
Heart 2017 Sep;103(17):1323-1330. Epub 2017 May 10 doi: 10.1136/heartjnl-2016-309916. PMID: 28490615
Bicuspid aortic valve aortopathy: genetics, pathophysiology and medical therapy.
Abdulkareem N, Smelt J, Jahangiri M
Interact Cardiovasc Thorac Surg 2013 Sep;17(3):554-9. Epub 2013 May 31 doi: 10.1093/icvts/ivt196. PMID: 23728086Free PMC Article
Valve-preserving surgery on the bicuspid aortic valve.
Vohra HA, Whistance RN, De Kerchove L, Punjabi P, El Khoury G
Eur J Cardiothorac Surg 2013 May;43(5):888-98. Epub 2013 Jan 4 doi: 10.1093/ejcts/ezs664. PMID: 23293321

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