Contemporary clinical perspectives, multi-modality imaging, and management strategies for bicuspid aortic valves

Abstract

Bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, characterized by the presence of two functional aortic valve leaflets instead of the usual three. It affects approximately 1 - 2% of the population and is often associated with other cardiovascular complications, including aortic stenosis, aortic regurgitation, and aortic root dilation. BAV can present with a wide range of clinical manifestations, from asymptomatic individuals to those with severe aortic valve dysfunction or aortic dissection. The diagnosis of BAV is commonly established through non-invasive imaging techniques such as transthoracic and transesophageal echocardiography, with advanced modalities, such as computed tomography and magnetic resonance imaging providing superior delineation of valve morphology, aortic root measurements, and associated pathology. Management of BAV is multifaceted and depends on the severity of valvular and aortic involvement. Lifelong surveillance is often necessary, with serial imaging to monitor disease progression. Surgical intervention is indicated for patients with severe valve dysfunction, symptomatic disease, or aortic aneurysm exceeding established threshold diameters. Common procedures include aortic valve replacement (AVR) and composite graft repair, such as the Bentall or valve-sparing David procedure. Transcatheter AVR has also emerged as a less invasive alternative to surgical valve replacement within this patient population, with its indications expanding significantly in recent years due to growing interest and advancements in the field. Despite substantial progress in diagnostic and therapeutic strategies, BAV poses clinical challenges due to its heterogeneous presentation, genetic underpinnings, and the requirement for tailored management approaches.

Keywords

Bicuspid aortic valve / Aortic valve / Aortic stenosis / Aortic regurgitation / Aortic aneurysm

Acknowledgments

None.

Funding

None.

Conflict of interest

The authors declare they have no competing interests.

Author contributions

Conceptualization: Faysal Massad, Tom Kai Ming Wang

Visualization: Faysal Massad, Simrat Kuaur, Tom Kai Ming Wang

Writing - original draft: Faysal Massad, Ahmad Safdar, Elio Haroun, Aro Daniela Arockiam, Mahmoud Alshneikat, Simrat Kaur, Tom Kai Ming Wang

Writing - review & editing: Faysal Massad, Elio Haroun, Akiva Rosenzveig, Mahmoud Alshneikat, Tom Kai Ming Wang

Ethics approval and consent to participate

Not applicable.

Consent for publication

Not applicable.

Availability of data

Not applicable.

References

[1] Kong WKF, Bax JJ, Michelena HI, Delgado V. Sex differences in bicuspid aortic valve disease. Prog Cardiovasc Dis. 2020; 63(4):452-456. doi: 10.1016/j.pcad.2020.06.004

[2] Schreurs BA, Hopman MTE, Bakker CM, et al. Associations of lifelong exercise characteristics with valvular function and aortic diameters in patients with a bicuspid aortic valve. J Am Heart Assoc. 2024; 13(3):e031850. doi: 10.1161/JAHA.123.031850

[3] Otto CM, Nishimura RA, Bonow RO, et al. 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. Circulation. 2021; 143(5):e72-e227. doi: 10.1161/CIR.0000000000000923

[4] Grewal N, Gittenberger-de Groot AC, Lindeman JH, et al. Normal and abnormal development of the aortic valve and ascending aortic wall: A comprehensive overview of the embryology and pathology of the bicuspid aortic valve. Ann Cardiothorac Surg. 2022; 11(4):380-388. doi: 10.21037/acs-2021-bav-14

[5] Martin PS, Kloesel B, Norris RA, Lindsay M, Milan D, Body SC. Embryonic development of the bicuspid aortic valve. J Cardiovasc Dev Dis. 2015; 2(4):248-272. doi: 10.3390/jcdd2040248

[6] Fedak PWM, Verma S, David TE, Leask RL, Weisel RD, Butany J. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation. 2002; 106(8):900-904. doi: 10.1161/01.CIR.0000027905.26586.E8

[7] Falcone MW, Roberts WC, Morrow AG, Perloff JK. Congenital aortic stenosis resulting from a unicommissural valve. Clinical and anatomic features in twenty-one adult patients. Circulation. 1971; 44(2):272-280. doi: 10.1161/01.CIR.44.2.272

[8] Kalra A, Das R, Alkhalil M, et al. Bicuspid aortic valve disease: Classifications, treatments, and emerging transcatheter paradigms. Struct Heart. 2024; 8(1):100227. doi: 10.1016/j.shj.2023.100227

[9] Sievers HH, Schmidtke C. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg. 2007; 133(5):1226-1233. doi: 10.1016/j.jtcvs.2007.01.039

[10] Sillesen AS, Vøgg O, Pihl C, et al. Prevalence of bicuspid aortic valve and associated aortopathy in newborns in Copenhagen, Denmark. JAMA. 2021; 325(6):561-567. doi: 10.1001/jama.2020.27205

[11] Michelena HI. Speaking a common language: The international consensus on bicuspid aortic valve nomenclature and classification. Ann Cardiothorac Surg. 2022; 11(4):40217-40417. doi: 10.21037/acs-2022-bav-24

[12] Evangelista A, Gallego P, Calvo-Iglesias F, et al. Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease. Heart. 2018; 104(7):566-573. doi: 10.1136/heartjnl-2017-311560

[13] Kusner JJ, Brown JY, Gleason TG, Edelman ER. The natural history of bicuspid aortic valve disease. Struct Heart. 2022; 7(2):100119. doi: 10.1016/j.shj.2022.100119

[14] Massad F, Bavishi SN, Scott CG, et al. Temporal trends in prevalence of bicuspid aortic valves in patients undergoing surgical aortic valve replacement. JACC Cardiovasc Interv. 2024; 17(17):2088-2089. doi: 10.1016/j.jcin.2024.07.024

[15] Siu SC, Silversides CK.Bicuspid aortic valve disease. J Am Coll Cardiol. 2010; 55(25):2789-2800. doi: 10.1016/j.jacc.2009.12.068

[16] Lewin MB, Otto CM. The bicuspid aortic valve: Adverse outcomes from infancy to old age. Circulation. 2005; 111(7):832-834. doi: 10.1161/01.CIR.0000157137.59691.0B

[17] Yang LT, Ye Z, Wajih Ullah M, et al. Bicuspid aortic valve: Long-term morbidity and mortality. Eur Heart J. 2023; 44(43):4549-4562. doi: 10.1093/eurheartj/ehad477

[18] Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Circulation. 2005; 111(7):920-925. doi: 10.1161/01.CIR.0000155623.48408.C5

[19] Benini A, Benfari G, Pilati M, Luciani GB, Ribichini FL, Prioli MA. Bicuspid aortic valve disease from infancy to older age: A 25-year experience from an Italian referral center. J Cardiovasc Echogr. 2021; 31(1):29-34. doi: 10.4103/jcecho.jcecho_115_20

[20] Masri A, Svensson LG, Griffin BP, Desai MY. Contemporary natural history of bicuspid aortic valve disease: A systematic review. Heart. 2017; 103(17):1323-1330. doi: 10.1136/heartjnl-2016-309916

[21] Masri A, Kalahasti V, Alkharabsheh S, et al. Characteristics and long-term outcomes of contemporary patients with bicuspid aortic valves. J Thorac Cardiovasc Surg. 2016; 151(6):1650-1659.e1. doi: 10.1016/j.jtcvs.2015.12.019

[22] Zegri-Reiriz I, De Alarcón A, Muñoz P, et al. Infective endocarditis in patients with bicuspid aortic valve or mitral valve prolapse. J Am Coll Cardiol. 2018; 71(24):2731-2740. doi: 10.1016/j.jacc.2018.03.534

[23] Fleisher LA, Beckman JA, Brown KA, et al.ACC/AHA 2007 Guidelines on perioperative cardiovascular evaluation and care for noncardiac surgery: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 2002 guidelines on perioperative cardiovascular evaluation for noncardiac surgery): Developed in collaboration with the American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society for Vascular Medicine and Biology, and Society for Vascular Surgery. Circulation. 2007; 116(17):e418-e499. doi: 10.1161/CIRCULATIONAHA.107.185699

[24] Habib G, Hoen B, Tornos P, et al. Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): The task force on the prevention, diagnosis, and treatment of infective endocarditis of the European Society of Cardiology (ESC). Endorsed by the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) and the International Society of Chemotherapy (ISC) for Infection and Cancer. Eur Heart J. 2009; 30(19):2369-2413. doi: 10.1093/eurheartj/ehp285

[25] 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: 10.1016/j.mayocp.2015.10.011

[26] Michelena HI, Khanna AD, Mahoney D, et al. Incidence of aortic complications in patients with bicuspid aortic valves. JAMA. 2011; 306(10):1104-1112. doi: 10.1001/jama.2011.1286

[27] Michelena HI, Prakash SK, Della Corte A, et al. Bicuspid aortic valve: Identifying knowledge gaps and rising to the challenge from the international bicuspid aortic valve consortium (BAVCon). Circulation. 2014; 129(25):2691-2704. doi: 10.1161/CIRCULATIONAHA.113.007851

[28] Ahmed HS, Jayaram PR, Gupta D. Clinical presentation and surgical outcomes in patients with Shone’s complex: A systematic review. Gen Thorac Cardiovasc Surg. 2024; 72(10):621-640. doi: 10.1007/s11748-024-02067-1

[29] Braverman AC, Cheng A. The bicuspid aortic valve and associated aortic disease. In: Valvular Heart Disease: A Companion to Braunwald’s Heart Disease. Amsterdam, Netherlands: Elsevier Health Sciences; 2020. Last accessed on 2024 Dec 31].

[30] Michelena HI, Della Corte A, Evangelista A, 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. J Thorac Cardiovasc Surg. 2021; 162(3):e383-e414. doi: 10.1016/j.jtcvs.2021.06.019

[31] Sperling JS, Lubat E. Forme fruste or ‘incomplete’ bicuspid aortic valves with very small raphes: The prevalence of bicuspid valve and its significance may be underestimated. Int J Cardiol. 2015; 184:1-5. doi: 10.1016/j.ijcard.2015.02.013

[32] Guala A, Rodriguez-Palomares J, Galian-Gay L, 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: 10.1161/CIRCULATIONAHA.119.039693

[33] Bicuspid Aortic Valve Workup: Laboratory Studies, Imaging Studies, Other Tests. Last accessed on 2024 Dec 31].

[34] Spindola-Franco H, Fish B, Dachman A, Grose R, Attai L. Recognition of bicuspid aortic valve by plain film calcification. AJR Am J Roentgenol. 1982; 139(5):867-872. doi: 10.2214/ajr.139.5.867

[35] Ayad RF, Grayburn PA, Ko JM, Filardo G, Roberts WC. Accuracy of two-dimensional echocardiography in determining aortic valve structure in patients >50 years of age having aortic valve replacement for aortic stenosis. Am J Cardiol. 2011; 108(11):1589-1599. doi: 10.1016/j.amjcard.2011.09.006

[36] Hillebrand M, Koschyk D, Ter Hark P, 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: 10.21037/cdt.2017.05.03

[37] Chu JW, Picard MH, Agnihotri AK, Fitzsimons MG. Diagnosis of congenital unicuspid aortic valve in adult population: The value and limitation of transesophageal echocardiography. Echocardiography. 2010; 27(9):1107-1112. doi: 10.1111/j.1540-8175.2010.01209.x

[38] Yousry M, Rickenlund A, Petrini J, et al. Aortic valve type and calcification as assessed by transthoracic and transoesophageal echocardiography. Clin Physiol Funct Imaging. 2015; 35(4):306-313. doi: 10.1111/cpf.12166

[39] Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: A report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019; 73(12):e81-e192. doi: 10.1016/j.jacc.2018.08.1029

[40] Biaggi P, Matthews F, Braun J, Rousson V, Kaufmann PA, Jenni R. Gender, age, and body surface area are the major determinants of ascending aorta dimensions in subjects with apparently normal echocardiograms. J Am Soc Echocardiogr. 2009; 22(6):720-725. doi: 10.1016/j.echo.2009.03.012

[41] Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: An update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015; 28(1):1-39.e14. doi: 10.1016/j.echo.2014.10.003

[42] Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2017; 135(25):e1159-e1195. doi: 10.1161/CIR.0000000000000503

[43] Pellikka PA, Sarano ME, Nishimura RA, et al. Outcome of 622 adults with asymptomatic, hemodynamically significant aortic stenosis during prolonged follow-up. Circulation. 2005; 111(24):3290-3295. doi: 10.1161/CIRCULATIONAHA.104.495903

[44] Zoghbi WA, Adams D, Bonow RO, et al. Recommendations for noninvasive evaluation of native valvular regurgitation: A report from the American Society of echocardiography developed in collaboration with the society for cardiovascular magnetic resonance. J Am Soc Echocardiogr. 2017; 30(4):303-371. doi: 10.1016/j.echo.2017.01.007

[45] Tsampasian V, Victor K, Bhattacharyya S, Oxborough D, Ring L. Echocardiographic assessment of aortic regurgitation: A narrative review. Echo Res Pract. 2024; 11(1):1. doi: 10.1186/s44156-023-00036-7

[46] Hagendorff A, Evangelista A, Fehske W, Schäfers HJ. Improvement in the assessment of aortic valve and aortic aneurysm repair by 3-dimensional echocardiography. JACC Cardiovasc Imaging. 2019; 12(11):2225-2244. doi: 10.1016/j.jcmg.2018.06.032

[47] Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/ American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014; 63(22):e57-185. doi: 10.1016/j.jacc.2014.02.536

[48] Warnes CA, Williams RG, Bashore TM, et al.ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008; 118(23):e714-833. doi: 10.1161/CIRCULATIONAHA.108.190690

[49] Tanaka R, Yoshioka K, Niinuma H, Ohsawa S, Okabayashi H, Ehara S. Diagnostic value of cardiac CT in the evaluation of bicuspid aortic stenosis: Comparison With echocardiography and operative findings. Am J Roentgenol. 2010; 195(4):895-899. doi: 10.2214/AJR.09.3164

[50] Gleeson TG, Mwangi I, Horgan SJ, Cradock A, Fitzpatrick P, Murray JG. Steady‐state free‐precession (SSFP) cine MRI in distinguishing normal and bicuspid aortic valves. J Magn Reson Imaging. 2008; 28(4):873-878. doi: 10.1002/jmri.21547

[51] Evangelista Masip A, Galian-Gay L, Guala A, et al. Unraveling bicuspid aortic valve enigmas by multimodality imaging: Clinical implications. J Clin Med. 2022; 11(2):456. doi: 10.3390/jcm11020456

[52] Naito S, Petersen J, Reichenspurner H, Girdauskas E. The impact of coronary anomalies on the outcome in aortic valve surgery: Comparison of bicuspid aortic valve versus tricuspid aortic valve morphotype. Interact Cardiovasc Thorac Surg. 2018; 26(4):617-622. doi: 10.1093/icvts/ivx396

[53] Machowiec P, Przybylski P, Czekajska-Chehab E, Drop A. Patients with a Bicuspid Aortic Valve (BAV) diagnosed with ECG-gated cardiac multislice computed tomography-analysis of the reasons for referral, classification of morphological phenotypes, co-occurring cardiovascular abnormalities, and coronary artery stenosis. J Clin Med. 2024; 13(13):3790. doi: 10.3390/jcm13133790

[54] DiLorenzo MP, Grosse-Wortmann L. Myocardial fibrosis in congenital heart disease and the role of MRI. Radiol Cardiothorac Imaging. 2023; 5(3):e220255. doi: 10.1148/ryct.220255

[55] Rodríguez-Palomares JF, Dux-Santoy L, Guala A, et al. Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease. J Cardiovasc Magn Reson. 2018; 20(1):28. doi: 10.1186/s12968-018-0451-1

[56] Elbaz MSM, Scott MB, Barker AJ, et al. Four-dimensional virtual catheter: Noninvasive assessment of intra-aortic hemodynamics in bicuspid aortic valve disease. Radiology. 2019; 293(3):541-550. doi: 10.1148/radiol.2019190411

[57] Bissell MM, Loudon M, Hess AT, et al. Differential flow improvements after valve replacements in bicuspid aortic valve disease: A cardiovascular magnetic resonance assessment. J Cardiovasc Magn Reson. 2018; 20(1):10. doi: 10.1186/s12968-018-0431-5

[58] Bohbot Y, Renard C, Manrique A, et al. Usefulness of cardiac magnetic resonance imaging in aortic stenosis. Circ Cardiovasc Imaging. 2020; 13(5):e010356. doi: 10.1161/CIRCIMAGING.119.010356

[59] Nayak KS, Nielsen JF, Bernstein MA, et al. Cardiovascular magnetic resonance phase contrast imaging. J Cardiovasc Magn Reson. 2015; 17(1):71. doi: 10.1186/s12968-015-0172-7

[60] Dyverfeldt P, Bissell M, Barker AJ, et al. 4D flow cardiovascular magnetic resonance consensus statement. J Cardiovasc Magn Reson. 2015; 17(1):72. doi: 10.1186/s12968-015-0174-5

[61] Ribeiro HB, Orwat S, Hayek SS, et al. Cardiovascular magnetic resonance to evaluate aortic regurgitation after transcatheter aortic valve replacement. J Am Coll Cardiol. 2016; 68(6):577-585. doi: 10.1016/j.jacc.2016.05.059

[62] Pape LA, Awais M, Woznicki EM, et al. Presentation, diagnosis, and outcomes of acute aortic dissection: 17-year trends from the international registry of acute aortic dissection. J Am Coll Cardiol. 2015; 66(4):350-358. doi: 10.1016/j.jacc.2015.05.029

[63] Dinsmore RE, Liberthson RR, Wismer GL, et al. Magnetic resonance imaging of thoracic aortic aneurysms: Comparison with other imaging methods. AJR Am J Roentgenol. 1986; 146(2):309-314. doi: 10.2214/ajr.146.2.309

[64] Geiger J, Rahsepar AA, Suwa K, et al. 4D flow MRI, cardiac function, and T₁-mapping: Association of valve-mediated changes in aortic hemodynamics with left ventricular remodeling. J Magn Reson Imaging. 2018; 48(1):121-131. doi: 10.1002/jmri.25916

[65] Grajewski KG, Stojanovska J, Ibrahim ESH, Sayyouh M, Attili A. Left ventricular hypertrophy: Evaluation with cardiac MRI. Curr Probl Diagn Radiol. 2020; 49(6):460-475. doi: 10.1067/j.cpradiol.2019.09.005

[66] Gallo G, Volpe M, Rubattu S. Angiotensin receptor blockers in the management of hypertension: A real-world perspective and current recommendations. Vasc Health Risk Manag. 2022; 18:507-515. doi: 10.2147/VHRM.S337640

[67] Verma S, Siu SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med. 2014; 370(20):1920-1929. doi: 10.1056/NEJMra1207059

[68] Borger MA, Fedak PWM, Stephens EH, et al. The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: Executive summary. J Thorac Cardiovasc Surg. 2018; 156(2):473-480. doi: 10.1016/j.jtcvs.2017.10.161

[69] Boonyasirinant T, Rajiah P, Flamm SD. Abnormal aortic stiffness in patients with bicuspid aortic valve: Phenotypic variation determined by magnetic resonance imaging. Int J Cardiovasc Imaging. 2019; 35(1):133-141. doi: 10.1007/s10554-018-1433-y

[70] Wedin JO, Vedin O, Rodin S, et al. Patients with bicuspid aortic stenosis demonstrate adverse left ventricular remodeling and impaired cardiac function before surgery with increased risk of postoperative heart failure. Circulation. 2022; 146(17):1310-1322. doi: 10.1161/CIRCULATIONAHA.122.060125

[71] Yang LT, Michelena HI, Maleszewski JJ, Schaff HV, Pellikka PA. Contemporary etiologies, mechanisms, and surgical approaches in pure native aortic regurgitation. Mayo Clin Proc. 2019; 94(7):1158-1170. doi: 10.1016/j.mayocp.2018.11.034

[72] Girdauskas E, Disha K, Secknus M, Borger M, Kuntze T. Increased risk of late aortic events after isolated aortic valve replacement in patients with bicuspid aortic valve insufficiency versus stenosis. J Cardiovasc Surg (Torino). 2013; 54(5):653-659.

[73] David TE, Feindel CM. An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg. 1992; 103(4):617-621; discussion 622.

[74] Isselbacher EM, Preventza O, Hamilton Black J 3rd, 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: 10.1161/CIR.0000000000001106

[75] Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease: Developed by the Task Force for the management of valvular heart disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Rev Esp Cardiol (Engl Ed). 2022; 75(6):524. doi: 10.1016/j.rec.2022.05.006

[76] Boucek RJ, Gunja-Smith Z, Noble NL, Simpson CF. Modulation by propranolol of the lysyl cross-links in aortic elastin and collagen of the aneurysm-prone turkey. Biochem Pharmacol. 1983; 32(2):275-280. doi: 10.1016/0006-2952(83)90555-5

[77] Brooke BS, Habashi JP, Judge DP, Patel N, Loeys B, Dietz HC 3rd. Angiotensin II blockade and aortic-root dilation in Marfan’s syndrome. N Engl J Med. 2008; 358(26):2787-2795. doi: 10.1056/NEJMoa0706585

[78] Rao V, Van Arsdell GS, David TE, Azakie A, Williams WG. Aortic valve repair for adult congenital heart disease: A 22-year experience. Circulation. 2000; 102(19 Suppl 3):III40-III43. doi: 10.1161/01.cir.102.suppl_3.iii-40

[79] Tzemos N, Therrien J, Yip J, et al. Outcomes in adults with bicuspid aortic valves. JAMA. 2008; 300(11):1317-1325. doi: 10.1001/jama.300.11.1317

[80] Hutchins GM, Nazarian IH, Bulkley BH. Association of left dominant coronary arterial system with congenital bicuspid aortic valve. Am J Cardiol. 1978; 42(1):57-59. doi: 10.1016/0002-9149(78)90985-2

[81] Beaver T, Bavaria JE, Griffith B, et al. Seven-year outcomes following aortic valve replacement with a novel tissue bioprosthesis. J Thorac Cardiovasc Surg. 2024; 168(3):781-791. doi: 10.1016/j.jtcvs.2023.09.047

[82] Malaisrie SC, Barnhart GR, Farivar RS, et al. Current era minimally invasive aortic valve replacement: Techniques and practice. J Thorac Cardiovasc Surg. 2014; 147(1):6-14. doi: 10.1016/j.jtcvs.2013.08.086

[83] Merk DR, Lehmann S, Holzhey DM, et al. Minimal invasive aortic valve replacement surgery is associated with improved survival: A propensity-matched comparison. Eur J Cardiothorac Surg. 2015; 47(1):11-17. doi: 10.1093/ejcts/ezu068

[84] Byrne JG, Aranki SF, Couper GS, Adams DH, Allred EN, Cohn LH. Reoperative aortic valve replacement: Partial upper hemisternotomy versus conventional full sternotomy. J Thorac Cardiovasc Surg. 1999; 118(6):991-997. doi: 10.1016/S0022-5223(99)70092-9

[85] Mazine A, David TE, Stoklosa K, Chung J, Lafreniere- Roula M, Ouzounian M. Improved outcomes following the ross procedure compared with bioprosthetic aortic valve replacement. J Am Coll Cardiol. 2022; 79(10):993-1005. doi: 10.1016/j.jacc.2021.12.026

[86] Hanke T, Charitos EI, Stierle U, et al. The ross operation - a feasible and safe option in the setting of a bicuspid aortic valve? Eur J Cardio-Thorac Surg. 2010; 38(3):333-339. doi: 10.1016/j.ejcts.2010.01.064

[87] Poh CL, Buratto E, Larobina M, et al. The ross procedure in adults presenting with bicuspid aortic valve and pure aortic regurgitation: 85% freedom from reoperation at 20 years. Eur J Cardiothorac Surg. 2018; 54(3):420-426. doi: 10.1093/ejcts/ezy073

[88] Yokoyama Y, Kuno T, Toyoda N, et al. Ross procedure versus mechanical versus bioprosthetic aortic valve replacement: A network meta-analysis. J Am Heart Assoc. 2023; 12(1):e8066. doi: 10.1161/JAHA.122.027715

[89] Lansakara M, Unai S, Ozaki S. Ozaki procedure-re-construction of aortic valve leaflets using autologous pericardial tissue: A review. Indian J Thorac Cardiovasc Surg. 2023; 39(Suppl 2):260-269. doi: 10.1007/s12055-023-01635-z

[90] Mentias A, Feng K, Alashi A, et al. Long-term outcomes in patients with aortic regurgitation and preserved left ventricular ejection fraction. J Am Coll Cardiol. 2016; 68(20):2144-2153. doi: 10.1016/j.jacc.2016.08.045

[91] Wong CHM, Chan JSK, Sanli D, Rahimli R, Harky A. Aortic valve repair or replacement in patients with aortic regurgitation: A systematic review and meta-analysis. J Card Surg. 2019; 34(6):377-384. doi: 10.1111/jocs.14032

[92] Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax. 1968; 23(4):338-339. doi: 10.1136/thx.23.4.338

[93] Schäfers H, Fries R, Langer F, Nikoloudakis N, Graeter T, Grundmann U. Valve-preserving replacement of the ascending aorta: Remodeling versus reimplantation. J Thorac Cardiovasc Surg. 1998; 116(6):990-996. doi: 10.1016/s0022-5223(98)70051-0

[94] Borger MA, Fedak PWM, Stephens EH, 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: 10.1016/j.jtcvs.2018.02.115

[95] Di Eusanio M, Trimarchi S, Patel HJ, et al. Clinical presentation, management, and short-term outcome of patients with type A acute dissection complicated by mesenteric malperfusion: Observations from the International Registry of Acute Aortic Dissection. J Thorac Cardiovasc Surg. 2013; 145(2):385-390.e1. doi: 10.1016/j.jtcvs.2012.01.042

[96] Pan E, Kytö V, Savunen T, Gunn J. Early and late outcomes after open ascending aortic surgery: 47-year experience in asingle centre. Heart Vessels. 2018; 33(4):427-433. doi: 10.1007/s00380-017-1075-3

[97] Schneider U, Feldner SK, Hofmann C, et al. Two decades of experience with root remodeling and valve repair for bicuspid aortic valves. J Thorac Cardiovasc Surg. 2017; 153(4):S65-S71. doi: 10.1016/j.jtcvs.2016.12.030

[98] Lansac E, Di Centa I, Sleilaty G, et al. Remodeling root repair with an external aortic ring annuloplasty. J Thorac Cardiovasc Surg. 2017; 153(5):1033-1042. doi: 10.1016/j.jtcvs.2016.12.031

[99] Miyahara S, Schneider U, Morgenthaler L, Schäfers HJ. (Almost) all nonstenotic bicuspid aortic valves should be preserved or repaired. Semin Thorac Cardiovasc Surg. 2019; 31(4):656-660. doi: 10.1053/j.semtcvs.2019.03.008

[100] Ehrlich T, de Kerchove L, Vojacek J, et al. State-of-the art bicuspid aortic valve repair in 2020. Prog Cardiovasc Dis. 2020; 63(4):457-464. doi: 10.1016/j.pcad.2020.04.010

[101] Waksman R, Medranda GA. TAVR for low-risk bicuspid aortic stenosis: When in doubt, randomize. JACC Cardiovasc Interv. 2022; 15(5):533-535. doi: 10.1016/j.jcin.2022.01.291

[102] Yoon SH, Bleiziffer S, De Backer O, et al. Outcomes in transcatheter aortic valve replacement for bicuspid versus tricuspid aortic valve stenosis. J Am Coll Cardiol. 2017; 69(21):2579-2589. doi: 10.1016/j.jacc.2017.03.017

[103] Tang GHL, Amat-Santos IJ, De Backer O, et al. Rationale, definitions, techniques, and outcomes of commissural alignment in TAVR: From the ALIGN-TAVR consortium. JACC Cardiovasc Interv. 2022; 15(15):1497-1518. doi: 10.1016/j.jcin.2022.06.001

[104] Merdler I, Case B, Bhogal S, et al. Early experience with the evolut FX self-expanding valve vs. evolut PRO+ for patients with aortic stenosis undergoing TAVR. Cardiovasc Revasc Med. 2023; 56:1-6. doi: 10.1016/j.carrev.2023.06.003

[105] Bulut HI, Arjomandi Rad A, Syrengela AA, et al. A comprehensive review of management strategies for Bicuspid Aortic Valve (BAV): Exploring epidemiology, aetiology, aortopathy, and interventions in light of recent guidelines. J Cardiovasc Dev Dis. 2023; 10(9):398. doi: 10.3390/jcdd10090398

[106] Nerla R, Castriota F, Micari A, de Campos Martins E, Cremonesi A. Repositionable and retrievable Lotus valve system for the treatment of bicuspid aortic stenosis: Is it time to treat all valves? Expert Rev Med Devices. 2017; 14(7):557-563. doi: 10.1080/17434440.2017.1344095

[107] Halim SA, Edwards FH, Dai D, et al. Outcomes of transcatheter aortic valve replacement in patients with bicuspid aortic valve disease: A report from the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry. Circulation. 2020; 141(13):1071-1079. doi: 10.1161/CIRCULATIONAHA.119.040333

[108] Lelasi A, Moscarella E, Mangieri A, et al. Procedural and clinical outcomes of type 0 versus type 1 bicuspid aortic valve stenosis undergoing trans-catheter valve replacement with new generation devices: Insight from the BEAT international collaborative registry. Int J Cardiol. 2021; 325:109-114. doi: 10.1016/j.ijcard.2020.10.050

[109] Moscarella E, Mangieri A, Giannini F, et al. Annular size and interaction with trans-catheter aortic valves for treatment of severe bicuspid aortic valve stenosis: Insights from the BEAT registry. Int J Cardiol. 2022; 349:31-38. doi: 10.1016/j.ijcard.2021.11.055

[110] Chen CHJ, Jiang H, Martin O, Wilson-Smith AR. Procedural and clinical outcomes of transcatheter aortic valve replacement in bicuspid aortic valve patients: A systematic review and meta-analysis. Ann Cardiothorac Surg. 2022; 11(4):351-362. doi: 10.21037/acs-2022-bav-22

[111] Montalto C, Sticchi A, Crimi G, et al. Outcomes after transcatheter aortic valve replacement in bicuspid versus tricuspid anatomy: A systematic review and meta-analysis. JACC Cardiovasc Interv. 2021; 14(19):2144-2155. doi: 10.1016/j.jcin.2021.07.052

[112] Nistri S, Basso C, Marzari C, Mormino P, Thiene G. Frequency of bicuspid aortic valve in young male conscripts by echocardiogram. Am J Cardiol. 2005; 96(5):718-721. doi: 10.1016/j.amjcard.2005.04.051

[113] Basso C, Boschello M, Perrone C, et al. An echocardiographic survey of primary school children for bicuspid aortic valve. Am J Cardiol. 2004; 93(5):661-663. doi: 10.1016/j.amjcard.2003.11.031

[114] Stefani L, Galanti G, Toncelli L, et al. Bicuspid aortic valve in competitive athletes. Br J Sports Med. 2008; 42(1):31-35; discussion 35. doi: 10.1136/bjsm.2006.033530

[115] Sanghavi M, Rutherford JD. Cardiovascular physiology of pregnancy. Circulation. 2014; 130(12):1003-1008. doi: 10.1161/CIRCULATIONAHA.114.009029

[116] Herzig MS, McIlvaine S, Feinberg L, Spiel MH, Carroll BJ. Outcomes of bicuspid aortic valve in pregnancy. Am J Cardiol. 2024; 221:74-76. doi: 10.1016/j.amjcard.2024.04.038

[117] Belkadi S, Milleron O, Eliahou L, et al. Pregnancy in women with bicuspid aortic valve. Eur Heart J. 2021; 42(Supplement_1):ehab724.2894. doi: 10.1093/eurheartj/ehab724.2894

[118] Immer FF, Krähenbühl E, Hagen U, et al. Large area of the false lumen favors secondary dilatation of the aorta after acute type A aortic dissection. Circulation. 2005; 112(9 Suppl):I249-I252. doi: 10.1161/CIRCULATIONAHA.104.525733

[119] Braverman AC, Mittauer E, Harris KM, et al. Clinical features and outcomes of pregnancy-related acute aortic dissection. JAMA Cardiol. 2021; 6(1):58-66. doi: 10.1001/jamacardio.2020.4876

[120] Yamauchi MSW, Puchalski MD, Weng HT, et al. Disease progression and variation in clinical practice for isolated bicuspid aortic valve in children. Congenit Heart Dis. 2018; 13(3):432-439. doi: 10.1111/chd.12591

[121] Blais S, Meloche-Dumas L, Fournier A, Dallaire F, Dahdah N. Long-term risk factors for dilatation of the proximal aorta in a large cohort of children with bicuspid aortic valve. Circ Cardiovasc Imaging. 2020; 13(3):e009675. doi: 10.1161/CIRCIMAGING.119.009675

[122] Spaziani G, Ballo P, Favilli S, et al. Clinical outcome, valve dysfunction, and progressive aortic dilation in a pediatric population with isolated bicuspid aortic valve. Pediatr Cardiol. 2014; 35(5):803-809. doi: 10.1007/s00246-013-0856-4

[123] Mahle WT, Sutherland JL, Frias PA. Outcome of isolated bicuspid aortic valve in childhood. J Pediatr. 2010; 157(3):445-449. doi: 10.1016/j.jpeds.2010.03.004

[124] Baleilevuka-Hart M, Teng BJ, Carson KA, Ravekes WJ, Holmes KW. Sports participation and exercise restriction in children with isolated bicuspid aortic valve. Am J Cardiol. 2020; 125(11):1673-1677. doi: 10.1016/j.amjcard.2020.02.039

[125] Boe BA, Zampi JD, Kennedy KF, et al. Acute Success of balloon aortic valvuloplasty in the current era: A national cardiovascular data registry study. JACC Cardiovasc Interv. 2017; 10(17):1717-1726. doi: 10.1016/j.jcin.2017.08.001

[126] Auld B, Carrigan L, Ward C, Justo R, Alphonso N, Anderson B. Balloon aortic valvuloplasty for congenital aortic stenosis: A 14-year single centre review. Heart Lung Circ. 2019; 28(4):632-636. doi: 10.1016/j.hlc.2018.02.014

[127] d’Udekem Y. Aortic valve repair in pediatrics-time to swing the pendulum back? Ann Cardiothorac Surg. 2019; 8(3):396-398. doi: 10.21037/acs.2019.04.15

[128] Stolte T, Boeddinghaus J, Allegra G, et al. Incidence and outcomes of valve-in-valve transcatheter aortic valve implantation in failed bioprosthetic valves. J Clin Med. 2023; 12(18):5868. doi: 10.3390/jcm12185868

[129] Van Nieuwkerk AC, Santos RB, Fernandez-Nofrerias E, et al. Outcomes in valve-in-valve transcatheter aortic valve implantation. Am J Cardiol. 2022; 172:81-89. doi: 10.1016/j.amjcard.2022.02.028

[130] Kim WK, Liebetrau C, Fischer-Rasokat U, et al. Challenges of recognizing bicuspid aortic valve in elderly patients undergoing TAVR. Int J Cardiovasc Imaging. 2020; 36(2):251-256. doi: 10.1007/s10554-019-01704-8

[131] Stefani L, Galanti G, Innocenti G, Mercuri R, Maffulli N. Exercise training in athletes with bicuspid aortic valve does not result in increased dimensions and impaired performance of the left ventricle. Cardiol Res Pract. 2014; 2014:238694. doi: 10.1155/2014/238694

[132] Pelliccia A, Sharma S, Gati S, et al. 2020 ESC guidelines on sports cardiology and exercise in patients with cardiovascular disease. Eur Heart J. 2021; 42(1):17-96. doi: 10.1093/eurheartj/ehaa605