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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

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
Circulation 2022 Dec 13;146(24):e334-e482. Epub 2022 Nov 2 doi: 10.1161/CIR.0000000000001106. PMID: 36322642Free PMC Article
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):e72-e227. Epub 2020 Dec 17 doi: 10.1161/CIR.0000000000000923. PMID: 33332150
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

Recent clinical studies

Etiology

Verma R, Cohen G, Colbert J, Fedak PWM
Curr Opin Cardiol 2023 Mar 1;38(2):61-67. Epub 2022 Dec 29 doi: 10.1097/HCO.0000000000001020. PMID: 36718616
Eleid MF, Nkomo VT, Pislaru SV, Gersh BJ
Annu Rev Med 2023 Jan 27;74:155-170. Epub 2022 Nov 18 doi: 10.1146/annurev-med-042921-122533. PMID: 36400067
Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
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
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

Diagnosis

Verma R, Cohen G, Colbert J, Fedak PWM
Curr Opin Cardiol 2023 Mar 1;38(2):61-67. Epub 2022 Dec 29 doi: 10.1097/HCO.0000000000001020. PMID: 36718616
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
Circulation 2022 Dec 13;146(24):e334-e482. Epub 2022 Nov 2 doi: 10.1161/CIR.0000000000001106. PMID: 36322642Free PMC Article
Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
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
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

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
McKellar SH
J Thorac Cardiovasc Surg 2022 Jan;163(1):61-63. Epub 2020 Jul 16 doi: 10.1016/j.jtcvs.2020.02.148. PMID: 32684392
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
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
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

Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
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
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
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
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

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
Eur Heart J 2023 Nov 14;44(43):4549-4562. doi: 10.1093/eurheartj/ehad477. PMID: 37611071
Verma R, Cohen G, Colbert J, Fedak PWM
Curr Opin Cardiol 2023 Mar 1;38(2):61-67. Epub 2022 Dec 29 doi: 10.1097/HCO.0000000000001020. PMID: 36718616
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
Senser EM, Misra S, Henkin S
Cardiol Clin 2021 Nov;39(4):505-515. doi: 10.1016/j.ccl.2021.06.003. PMID: 34686263
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

Saeed Al-Asad K, Martinez Salazar A, Radwan Y, Wang E, Salam MF, Sabanci R, Saeed M, Halboni A, Al-Abcha A, Abela G
Am J Cardiol 2023 Sep 15;203:105-112. Epub 2023 Jul 22 doi: 10.1016/j.amjcard.2023.06.120. PMID: 37487404
Bray JJH, Freer R, Pitcher A, Kharbanda R
Eur Heart J 2023 Sep 1;44(33):3152-3164. doi: 10.1093/eurheartj/ehad320. PMID: 37288540Free PMC Article
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
Xie X, Shi X, Xun X, Rao L
Ann Thorac Cardiovasc Surg 2016 Aug 23;22(4):203-15. Epub 2016 Apr 20 doi: 10.5761/atcs.ra.16-00032. PMID: 27098769Free PMC Article
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

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