BICUSPID AORTIC VALVE: WHEN AN ANATOMICAL VARIANT BECOMES A SURGICAL PROBLEM

  2. 2026
  3. BICUSPID AORTIC VALVE: WHEN AN ANATOMICAL VARIANT BECOMES A SURGICAL PROBLEM

Kuzevanova M. V., Koniushevska A. A., Kozarenko T. M., Storozhchuk Y. O., Smyrnova G. S.

BICUSPID AORTIC VALVE: WHEN AN ANATOMICAL VARIANT BECOMES A SURGICAL PROBLEM

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About the author:

Kuzevanova M. V., Koniushevska A. A., Kozarenko T. M., Storozhchuk Y. O., Smyrnova G. S.

Heading:

LITERATURE REVIEWS

Type of article:

Scientific article

Annotation:

According to current evidence, most individuals with BAV develop valvular and/or aortic complications, which occur in approximately 50% of cases. Although an association between the natural history and phenotype of BAV has been recognized, our understanding of the development of the anomaly itself and the mechanisms underlying its complications remains incomplete. Why one BAV becomes stenotic, another becomes regurgitant, another is associated with aortic dilatation, while yet another remains functionally unaffected throughout life remains fundamentally unknown and unpredictable. The search for approaches to early diagnosis and subsequent lifelong surveillance continues to make BAV a clinical challenge for physicians. The systematic integration of the extensive available evidence, together with a potential shift in the clinical perspective on patients with features of connective tissue dysplasia as a possible step toward early diagnostic screening for BAV, requires further investigation. Premature valve degeneration associated with early calcification and the search for optimal approaches to cardiac surgical correction, particularly in the context of the potential future development of aortopathy and its associated increased risk of postoperative complications, also warrant further study. A patient with BAV requires multidisciplinary management, with consistent application of valvular, aortic, and genetic classification systems in both clinical practice and research. Such an approach may contribute to a better understanding of disease patterns that have both prognostic and therapeutic significance.

Tags:

aortopathy, associated defects, bicuspid aortic valve, echocardiographic screening, surgical treatment

Bibliography:

  1. Kusner JJ, Brown JY, Gleason TG, Edelman ER. The Natural History of Bicuspid Aortic Valve Disease. Struct Heart. 2022;7(2):100119. DOI: https://doi.org/10.1016/j.shj.2022.100119
  2. Pepe G, Nistri S, Giusti B, Sticchi E, Attanasio M, Porciani C, et al. Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome. BMC Med Genet. 2014;15:23. DOI: https://doi.org/10.1186/1471-2350-15-23
  3. Rippe M, De Backer J, Kutsche K, Mosquera LM, Schüler H, Rybczynski M, et al. Mitral valve prolapse syndrome and MASS phenotype: Stability of aortic dilatation but progression of mitral valve prolapse. Int J Cardiol Heart Vasc. 2016;10:39-46. DOI: https://doi.org/10.1016/j.ijcha.2016.01.002
  4. Yang LT, Ye Z, Ullah MW, Maleszewski JJ, Scott CG, Padang R, et al. Bicuspid aortic valve: long-term morbidity and mortality. Eur Heart J. 2023;44(43):4549-4562. DOI: https://doi.org/10.1093/eurheartj/ehad477
  5. Katsaros O, Ktenopoulos N, Korovesis T, Benetos G, Apostolos A, Koliastasis L, et al. Bicuspid aortic valve disease: from pathophysiology to treatment. J Clin Med. 2024;13(17):4970. DOI: https://doi.org/10.3390/jcm13174970
  6. Brückner A, Brandtner A, Rieck S, Billig H, Masson W, Weber A, et al. Bicuspid aortic valve stenosis is characterized by increased angiogenesis, inflammation, and a higher valvular-to-systemic calcification ratio than tricuspid aortic valve stenosis. Basic Res Cardiol. 2026;121:801-816. DOI: https://doi.org/10.1007/s00395-026-01191-8
  7. Hamatani Y, Ishibashi-Ueda H, Nagai T, Sugano Y, Kanzaki H, Yasuda S, et al. Pathological investigation of congenital bicuspid aortic valve stenosis, compared with atherosclerotic tricuspid aortic valve stenosis and congenital bicuspid aortic valve regurgitation. PLoS One. 2016;11(8):e0160208. DOI: https://doi.org/10.1371/journal.pone.0160208
  8. Yener N, Oktar GL, Erer D, Yardimci MM, Yener A. Bicuspid aortic valve. Ann Thorac Cardiovasc Surg. 2002;8(5):264-7.
  9. Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Enriquez-Sarano M, Bax JJ, et al. Speaking a common language: introduction to a standard terminology for the bicuspid aortic valve and its aortopathy. Prog Cardiovasc Dis. 2020;63(4):419-424. DOI: https://doi.org/10.1016/j.pcad.2020.06.006
  10. Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, et al. International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes. Eur J Cardiothorac Surg. 2021;60(3):448-476. DOI: https://doi.org/10.1093/ejcts/ezab038
  11. Kalra A, Das R, Alkhalil M, Dykun I, Candreva A, Jarral O, et al. Bicuspid aortic valve disease: classifications, treatments, and emerging transcatheter paradigms. Struct Heart. 2024;8(1):100227. DOI: https://doi.org/10.1016/j.shj.2023.100227
  12. Paglianiti DA, Aurigemma C, Busco M, Cappannoli L, Bianchini F, Romagnoli E, et al. TAVI for bicuspid aortic valve: addressing technical challenges and optimizing outcomes. J Clin Med. 2025;14(21):7860. DOI: https://doi.org/10.3390/jcm14217860
  13. Zanda G, Bellini B, Romano V, Iannopollo G, Vella C, Russo F, et al. Transcatheter aortic valve replacement in forme fruste bicuspid aortic valve with the dedicated LIRA sizing method. Struct Heart. 2026;10(5):100818. DOI: https://doi.org/10.1016/j.shj.2026.100818
  14. Borger MA, Fedak PWM, Stephens EH, Gleason TG, Girdauskas E, Ikonomidis JS, et al. The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: full online-only version. J Thorac Cardiovasc Surg. 2018;156(2):e41-e74. DOI: https://doi.org/10.1016/j.jtcvs.2018.02.115
  15. Guala A, Rodriguez-Palomares J, Galian-Gay L, Teixido-Tura G, Johnson KM, Wieben O, et al. Partial aortic valve leaflet fusion is related to deleterious alteration of proximal aorta hemodynamics: a 4-dimensional flow magnetic resonance imaging study. Circulation. 2019;139(23):2707-2709. DOI: https://doi.org/10.1161/circulationaha.119.039693
  16. Jahanyar J, de Kerchove L, El Khoury G. Forme fruste unicuspid aortic valves. Eur Heart J. 2024;45(38):4139. DOI: https://doi.org/10.1093/eurheartj/ehae432
  17. Ackah RL, Yasuhara J, Garg V. Genetics of aortic valve disease. Curr Opin Cardiol. 2023;38(3):169-178. DOI: https://doi.org/10.1097/hco.0000000000001028
  18. Bravo-Jaimes K, Prakash SK. Genetics in bicuspid aortic valve disease: where are we? Prog Cardiovasc Dis. 2020;63(4):398-406. DOI: https://doi.org/10.1016/j.pcad.2020.06.005
  19. Junco-Vicente A, del Río-García Á, Martín M, Rodríguez I. Update in biomolecular and genetic bases of bicuspid aortopathy. Int J Mol Sci. 2021;22(11):5694. DOI: https://doi.org/10.3390/ijms22115694
  20. Tessler I, Goudot G, Albuisson J, Reshef N, Zwas DR, Carmi S, et al. Is bicuspid aortic valve morphology genetically determined? A familybased study. Am J Cardiol. 2022;163:85-90. DOI: https://doi.org/10.1016/j.amjcard.2021.09.051
  21. Tessler I, Albuisson J, Goudot G, Carmi S, Shpitzen S, Messas E, et al. Bicuspid aortic valve: genetic and clinical insights. Aorta (Stamford). 2021;9(4):139-146. DOI: https://doi.org/10.1055/s-0041-1730294
  22. Sticchi E, De Cario R, Magi A, Giglio S, Provenzano A, Nistri S, et al. Bicuspid aortic valve: role of multiple gene variants in influencing the clinical phenotype. Biomed Res Int. 2018;2018:8386123. DOI: https://doi.org/10.1155/2018/8386123
  23. Sillesen A, Vogg O, Pihl C, Axelsson Raja A, Sundberg K, Vedel C, et al. Prevalence of bicuspid aortic valve and associated aortopathy in newborns in Copenhagen, Denmark. JAMA. 2021;325(6):561-567. DOI: https://doi.org/10.1001/jama.2020.27205
  24. Rizza V, Ancona F, Ingallina G, Stella S, Margonato D, Tavernese A, et al. Prevalence, clinical characterization, management and evolution of bicuspid aortic valve classified according to the 2021 International Consensus Statement in a tertiary care hospital. Eur J Cardiothorac Surg. 2025;67(4):ezaf109. DOI: https://doi.org/10.1093/ejcts/ezaf109
  25. Yang LT, Tribouilloy C, Masri A, Bax JJ, Delgado V, Girdauskas E, et al. Clinical presentation and outcomes of adults with bicuspid aortic valves: 2020 update. Prog Cardiovasc Dis. 2020;63:434-441. DOI: https://doi.org/10.1016/j.pcad.2020.05.010
  26. Song S, Seo J, Cho I, Hong GR, Ha JW, Shim CY. Progression and outcomes of non-dysfunctional bicuspid aortic valve: longitudinal data from a large Korean bicuspid aortic valve registry. Front Cardiovasc Med. 2021;7:603323. DOI: https://doi.org/10.3389/fcvm.2020.603323
  27. Kong WKF, Regeer MV, Ng ACT, McCormack L, Poh KK, Yeo TC, et al. Valve disease and aortopathy associations of bicuspid aortic valve phenotypes differ between men and women. Open Heart. 2021;8(2):e001857. DOI: https://doi.org/10.1136/openhrt-2021-001857
  28. Choi BH, Ko SM, Shin JK, Chee HK, Kim JS. The association between morphological and functional characteristics of the bicuspid aortic valve and bicuspid aortopathy. Korean J Radiol. 2021;22(6):890-900. DOI: https://doi.org/10.3348/kjr.2020.0538
  29. Szöcs K, Toprak B, Schön G, Rybczynski M, Brinken T, Mahlmann A, et al. Concomitant cardiovascular malformations in isolated bicuspid aortic valve disease: a retrospective cross-sectional study and meta-analysis. Cardiovasc Diagn Ther. 2022;12(4):400-414. DOI: https://doi.org/10.21037/cdt-22-112
  30. Shen M, Tastet L, Capoulade R, Arsenault M, Bédard É, Clavel MA, Pibarot P. Effect of bicuspid aortic valve phenotype on progression of aortic stenosis. Eur Heart J Cardiovasc Imaging. 2020;21(7):727-734. DOI: https://doi.org/10.1093/ehjci/jeaa068
  31. Yoon SH, Kim WK, Dhoble A, Pio SM, Babaliaros V, Jilaihawi H, et al. Bicuspid aortic valve morphology and outcomes after transcatheter aortic valve replacement. J Am Coll Cardiol. 2020;76(9):1018-1030. DOI: https://doi.org/10.1016/j.jacc.2020.07.005
  32. Pereira SC, Abrantes AL, António PS, Morais P, Sousa C, David C et al. Infective endocarditis risk in patients with bicuspid aortic valve: systematic review and meta-analysis. Int J Cardiol Heart Vasc. 2023;47:101249. DOI: https://doi.org/10.1016/j.ijcha.2023.101249
  33. Giusti B, Sticchi E, De Cario R, Magi A, Nistri S, Pepe G. Genetic bases of bicuspid aortic valve: the contribution of traditional and highthroughput sequencing approaches on research and diagnosis. Front Physiol. 2017;8:612. DOI: https://doi.org/10.3389/fphys.2017.00612
  34. Pepe G, Giusti B, Colonna S, Fugazzaro MP, Sticchi E, De Cario R, et al. When should a rare inherited connective tissue disorder be suspected in bicuspid aortic valve by primary-care internists and cardiologists? Proposal of a score. Intern Emerg Med. 2021;16(3):609- 615. DOI: https://doi.org/10.1007/s11739-020-02458-1
  35. Michelena HI, Chandrasekaran K, Topilsky Y, Messika-Zeitoun D, Della Corte A, Evangelista A, et al. The bicuspid aortic valve condition: the critical role of echocardiography and the case for a standard nomenclature consensus. Prog Cardiovasc Dis. 2018;61(5-6):404-415. DOI: https://doi.org/10.1016/j.pcad.2018.11.003
  36. Hillebrand M, Koschyk D, ter Hark P, Schüler H, Rybczynski M, Berger J, et al. Diagnostic accuracy study of routine echocardiography for bicuspid aortic valve: a retrospective study and meta-analysis. Cardiovasc Diagn Ther. 2017;7(4):367-379. DOI: https://doi.org/10.21037/cdt.2017.05.03
  37. Kim J, Kim SM, Ahn J, Kim J, Choe YH. Diagnostic performance of cardiac CT and transthoracic echocardiography for detection of surgically confirmed bicuspid aortic valve: effect of calcium extent and valve subtypes. J Korean Soc Radiol. 2023;84(6):1324-1336. DOI: https://doi.org/10.3348/jksr.2022.0170
  38. Noly PE, Basmadjian L, Bouhout I, Viet Le VH, Poirier N, El-Hamamsy I. New insights into unicuspid aortic valve disease in adults: not just a subtype of bicuspid aortic valves. Can J Cardiol. 2016;32(1):110-116. DOI: https://doi.org/10.1016/j.cjca.2015.10.028
  39. Niaz T, Poterucha JT, Johnson JN, Craviari C, Nienaber T, Palfreeman J, et al. Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects. Congenit Heart Dis. 2017;12(3):261-269. DOI: https://doi.org/10.1111/chd.12429
  40. Michelena HI, Katan O, Suri RM, Baddour LM, Enriquez-Sarano M. Incidence of infective endocarditis in patients with bicuspid aortic valves in the community. Mayo Clin Proc. 2016;91(1):122-123. DOI: https://doi.org/10.1016/j.mayocp.2015.10.011
  41. Chong A, Stanton T, Taylor A, Prior D, La Gerche A, Anderson B, et al. 2024 CSANZ Position Statement on indications, assessment and monitoring of structural and valvular heart disease with transthoracic echocardiography in adults. Heart Lung Circ. 2024;33(6):773-827. DOI: https://doi.org/10.1016/j.hlc.2023.11.028
  42. Michelena HI. Speaking a common language: the international consensus on bicuspid aortic valve nomenclature and classification. Ann Cardiothorac Surg. 2022;11(4):402-417. DOI: https://doi.org/10.21037/acs-2022-bav-24
  43. Szyszka A, Płońska-Gościniak E. Transesophageal echocardiography. J Ultrason. 2019;19(76):62-65 DOI: https://doi.org/10.15557/JoU.2019.0009
  44. Evangelista A, Sitges M, Jondeau G, Nijveldt R, Pepi M, Cuellar H, et al. Multimodality imaging in thoracic aortic diseases: a clinical consensus statement from the European Association of Cardiovascular Imaging and the European Society of Cardiology working group on aorta and peripheral vascular diseases. Eur Heart J Cardiovasc Imaging. 2023;24(5):e65-e85. DOI: https://doi.org/10.1093/ehjci/jead024
  45. Saric M, Armour AC, Arnaout MS, Chaudhry FA, Grimm RA, Kronzon I, et al. Guidelines for the use of echocardiography in the evaluation of a cardiac source of embolism. J Am Soc Echocardiogr. 2016;29(1):1-42. DOI: https://doi.org/10.1016/j.echo.2015.09.011
  46. Takagi H, Yoshizawa M, Orii M, Kumagai A, Tashiro A, Chiba T, et al. Additive value of CT to age, aortic diameter, and echocardiography in diagnosis and classification of bicuspid aortic valve in patients with severe aortic stenosis. Radiol ardiothorac Imaging. 2021;3(1):e200423. DOI: https://doi.org/10.1148/ryct.2021200423
  47. Sylivris A, Liu ZF, Theuerle J, Lim RP. Diagnosis of bicuspid aortic valves: CT vs. TTE. Int J Cardiovasc Imaging. 2025;41(4):659-667. DOI: https://doi.org/10.1007/s10554-024-03290-w
  48. Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, et al. International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes. Eur J Cardiothorac Surg. 2021;11;60(3):448-476. DOI: https://doi.org/10.1093/ejcts/ezab038.
  49. Mani P, Reyaldeen R, Xu B. Multimodality imaging assessment of bicuspid aortic valve disease, thoracic aortic ectasia, and thoracic aortic aneurysmal disease. Cardiovasc Diagn Ther. 2021;11(3):896-910. DOI: https://doi.org/10.21037/cdt-20-279
  50. Praz F, Borger MA, Lanz J, Marin-Cuartas M, Abreu A, Adamo M, et al. 2025 ESC/EACTS Guidelines for the management of valvular heart disease. Eur Heart J. 2025;46(44):4635-4736. DOI: https://doi.org/10.1093/eurheartj/ehaf194
  51. Czerny M, Grabenwöger M, Berger T, Aboyans V, Della Corte A, Chen EP, et al. EACTS/STS Guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ. Eur J Cardiothorac Surg. 2024;65(2):ezad426. DOI: https://doi.org/10.1093/ejcts/ezad426
  52. Kang JJH, Fialka NM, El-Andari R, Watkins A, Hong Y, Mathew A, et al. Surgical vs transcatheter aortic valve replacement in bicuspid aortic valve stenosis: a systematic review and meta-analysis. Trends Cardiovasc Med. 2024;34(5):304-313. DOI: https://doi.org/10.1016/j.tcm.2023.04.004
  53. De Paulis R, Chirichilli I, de Kerchove L, Della Corte A, El Khoury G, Michelena HI, et al. Current status of aortic valve repair surgery. Eur Heart J. 2025;46(15):1394-1411. DOI: https://doi.org/10.1093/eurheartj/ehaf038
  54. Isselbacher EM, Preventza O, Black JH 3rd, Augoustides JG, Beck AW, Bolen MA, et al. 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. Circulation. 2022;146(24):e334-e482. DOI: https://doi.org/10.1161/cir.0000000000001106
  55. Otto CM, Nishimura RA, Bonow RO, Carabello BA, Erwin JP 3rd, Gentile F, et al. 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. J Am Coll Cardiol. 2021;77(4):450-500. DOI: https://doi.org/10.1161/cir.0000000000000932
  56. Della Corte A, Michelena HI, Citarella A, Votta E, Piatti F, Lo Presti F, et al. Risk stratification in bicuspid aortic valve aortopathy: emerging evidence and future perspectives. Curr Probl Cardiol. 2021;46(3):100428. DOI: https://doi.org/10.1016/j.cpcardiol.2019.06.002
  57. Girdauskas E, Geist L, Disha K, Kazakbaev I, Groß T, Schulz S, et al. Genetic abnormalities in bicuspid aortic valve root phenotype: preliminary results. Eur J Cardiothorac Surg. 2017;52(1):156-162. DOI: https://doi.org/10.1093/ejcts/ezx065
  58. Zaki H, Albaroudi B, Shaban E, Alkahlout B, Yigit Y, Elnabawy W, et al. Comparison between transthoracic echocardiography and transoesophageal echocardiography in the diagnosis of acute aortic dissection from an emergency perspective: a systematic review and meta-analysis. Front Cardiovasc Med. 2024;10(10):1283703. DOI: https://doi.org/10.3389/fcvm.2023.1283703
  59. Giauffret F, Hocq F, Lafond S, Autret A, Elias A. European Carotid Surgical Trial-based diameter measurement using B-mode ultrasound imaging to quantify low-grade carotid artery stenosis: The QUAMUS study. JVS-Vasc Insights. 2024;2(Suppl C):100061. DOI: https://doi.org/10.1016/j.jvsvi.2024.00009-6
  60. Baumgartner H, Hung J, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, et al. Recommendations on the echocardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. J Am Soc Echocardiogr. 2017;30(4):372-392. DOI: https://doi.org/10.1016/j.echo.2017.02.009

Publication of the article:

«Bulletin of problems biology and medicine», 2026 Issue 3, 182, 63-75 pages, index UDC 616.12-007.2:616.13-089

DOI:

10.29254/2077-4214-2026-3-182-63-75

2026 Issue 3, 182
aortopathy associated defects bicuspid aortic valve echocardiographic screening surgical treatment
29.09.2026
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