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Abnormal aortic valve cusp morphology

MedGen UID:
1623463
•Concept ID:
C4531034
•
Anatomical Abnormality
HPO: HP:0031567

Definition

Any structural anomaly of the aortic valve leaflets. [from HPO]

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
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
Investigation of raphe function in the bicuspid aortic valve and its influence on clinical criteria-A patient-specific finite element study.
Dallard J, Labrosse MR, Sohmer B, Beller CJ, Boodhwani M
Int J Numer Method Biomed Eng 2018 Oct;34(10):e3117. Epub 2018 Jul 23 doi: 10.1002/cnm.3117. PMID: 29905015

Recent clinical studies

Etiology

Predictors of Aortic Valve Repair Failure.
Yanagawa B, Mazine A, El-Hamamsy I
Innovations (Phila) 2019 Jun;14(3):199-208. Epub 2019 May 14 doi: 10.1177/1556984519845905. PMID: 31084444
Aortic valve repair for aortic regurgitation and preoperative echocardiographic assessment.
Kunihara T
J Med Ultrason (2001) 2019 Jan;46(1):51-62. Epub 2018 Sep 19 doi: 10.1007/s10396-018-0903-0. PMID: 30232651
Bicuspid aortic valve-associated aortopathy: update on biomarkers.
Maredia AK, Greenway SC, Verma S, Fedak PWM
Curr Opin Cardiol 2018 Mar;33(2):134-139. doi: 10.1097/HCO.0000000000000481. PMID: 29095713
Reexamining remodeling.
Schäfers HJ, Raddatz A, Schmied W, Takahashi H, Miura Y, Kunihara T, Aicher D
J Thorac Cardiovasc Surg 2015 Feb;149(2 Suppl):S30-6. Epub 2014 Sep 18 doi: 10.1016/j.jtcvs.2014.09.048. PMID: 25439784
Cusp repair during aortic valve-sparing operation: technical aspects and impact on results.
Settepani F, Cappai A, Raffa GM, Basciu A, Barbone A, Berwick D, Citterio E, Ornaghi D, Tarelli G, Malvindi PG
J Cardiovasc Med (Hagerstown) 2015 Apr;16(4):310-7. doi: 10.2459/JCM.0000000000000031. PMID: 24978873

Diagnosis

Aortic cusp abnormalities in patients with trileaflet aortic valve and root aneurysm.
Ehrlich T, Hagendorff A, Abeln K, Froede L, Giebels C, Schäfers HJ
Heart 2022 Dec 13;109(1):55-62. doi: 10.1136/heartjnl-2022-320905. PMID: 35803710
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
Aortic valve repair for aortic regurgitation and preoperative echocardiographic assessment.
Kunihara T
J Med Ultrason (2001) 2019 Jan;46(1):51-62. Epub 2018 Sep 19 doi: 10.1007/s10396-018-0903-0. PMID: 30232651
Bicuspid aortic valve-associated aortopathy: update on biomarkers.
Maredia AK, Greenway SC, Verma S, Fedak PWM
Curr Opin Cardiol 2018 Mar;33(2):134-139. doi: 10.1097/HCO.0000000000000481. PMID: 29095713
Reexamining remodeling.
Schäfers HJ, Raddatz A, Schmied W, Takahashi H, Miura Y, Kunihara T, Aicher D
J Thorac Cardiovasc Surg 2015 Feb;149(2 Suppl):S30-6. Epub 2014 Sep 18 doi: 10.1016/j.jtcvs.2014.09.048. PMID: 25439784

Therapy

Late results of aortic valve repair for isolated severe aortic regurgitation.
Tamer S, Mastrobuoni S, Vancraeynest D, Lemaire G, Navarra E, Khoury GE, de Kerchove L
J Thorac Cardiovasc Surg 2023 Mar;165(3):995-1006.e3. Epub 2021 Apr 16 doi: 10.1016/j.jtcvs.2021.04.011. PMID: 34049711
Almost All Incompetent BAV Should Be Repaired.
David TE
Semin Thorac Cardiovasc Surg 2019 Winter;31(4):661-663. Epub 2019 Apr 10 doi: 10.1053/j.semtcvs.2019.03.009. PMID: 30980931
Reexamining remodeling.
Schäfers HJ, Raddatz A, Schmied W, Takahashi H, Miura Y, Kunihara T, Aicher D
J Thorac Cardiovasc Surg 2015 Feb;149(2 Suppl):S30-6. Epub 2014 Sep 18 doi: 10.1016/j.jtcvs.2014.09.048. PMID: 25439784
Cusp repair during aortic valve-sparing operation: technical aspects and impact on results.
Settepani F, Cappai A, Raffa GM, Basciu A, Barbone A, Berwick D, Citterio E, Ornaghi D, Tarelli G, Malvindi PG
J Cardiovasc Med (Hagerstown) 2015 Apr;16(4):310-7. doi: 10.2459/JCM.0000000000000031. PMID: 24978873
Management of bicuspid aortic valve with or without involvement of ascending aorta and aortic root.
Neragi-Miandoab S
J Cardiovasc Surg (Torino) 2014 Jun;55(3):435-44. Epub 2012 Nov 8 PMID: 23138607

Prognosis

Is Bicuspid Aortic Valve Morphology Genetically Determined? A Family-Based Study.
Tessler I, Goudot G, Albuisson J, Reshef N, Zwas DR, Carmi S, Shpitzen S, Levin G, Kelman G, Cheng C, Mazzella JM, Levin Y, Messas E, Gilon D, Durst R
Am J Cardiol 2022 Jan 15;163:85-90. Epub 2021 Nov 17 doi: 10.1016/j.amjcard.2021.09.051. PMID: 34799086
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
Predictors of Aortic Valve Repair Failure.
Yanagawa B, Mazine A, El-Hamamsy I
Innovations (Phila) 2019 Jun;14(3):199-208. Epub 2019 May 14 doi: 10.1177/1556984519845905. PMID: 31084444
Bicuspid aortic valve-associated aortopathy: update on biomarkers.
Maredia AK, Greenway SC, Verma S, Fedak PWM
Curr Opin Cardiol 2018 Mar;33(2):134-139. doi: 10.1097/HCO.0000000000000481. PMID: 29095713
Reexamining remodeling.
Schäfers HJ, Raddatz A, Schmied W, Takahashi H, Miura Y, Kunihara T, Aicher D
J Thorac Cardiovasc Surg 2015 Feb;149(2 Suppl):S30-6. Epub 2014 Sep 18 doi: 10.1016/j.jtcvs.2014.09.048. PMID: 25439784

Clinical prediction guides

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
Sex differences in bicuspid aortic valve disease.
Kong WKF, Bax JJ, Michelena HI, Delgado V
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):452-456. Epub 2020 Jun 27 doi: 10.1016/j.pcad.2020.06.004. PMID: 32599025
Predictors of Aortic Valve Repair Failure.
Yanagawa B, Mazine A, El-Hamamsy I
Innovations (Phila) 2019 Jun;14(3):199-208. Epub 2019 May 14 doi: 10.1177/1556984519845905. PMID: 31084444
Bicuspid aortic valve-associated aortopathy: update on biomarkers.
Maredia AK, Greenway SC, Verma S, Fedak PWM
Curr Opin Cardiol 2018 Mar;33(2):134-139. doi: 10.1097/HCO.0000000000000481. PMID: 29095713
Reexamining remodeling.
Schäfers HJ, Raddatz A, Schmied W, Takahashi H, Miura Y, Kunihara T, Aicher D
J Thorac Cardiovasc Surg 2015 Feb;149(2 Suppl):S30-6. Epub 2014 Sep 18 doi: 10.1016/j.jtcvs.2014.09.048. PMID: 25439784

Recent systematic reviews

Haemodynamic assessment of bicuspid aortic valve aortopathy: a systematic review of the current literature.
Edlin J, Youssefi P, Bilkhu R, Figueroa CA, Morgan R, Nowell J, Jahangiri M
Eur J Cardiothorac Surg 2019 Apr 1;55(4):610-617. doi: 10.1093/ejcts/ezy312. PMID: 30239633
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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