Characteristics of bicuspid aortic valve aortopathy and association with valvular phenotype and dysfunction_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

SUN Jing ¹ , SUN Cheng ¹ , HOU Zhihui ² , QI Hongxia ³ , QIAN Xiangyang ¹ ,  ZHENG Zhe ¹

1. Department of Cardiovascular Surgery, National Center for Cardiovascular Diseases and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, P. R. China;
2. Department of Radiology, National Center for Cardiovascular Diseases and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, P. R. China;
3. Department of Ultrasound, National Center for Cardiovascular Diseases and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, P. R. China;

Corresponding author：

ZHENG Zhe, Email: zhengzhe@fuwai.com

Keywords：

Bicuspid aortic valve; aortic dilation; aneurysm; aortic stenosis; aortic insufficiency

DOI：

10.7507/1007-4848.202101004

Video：

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Objective To summarize the characteristics of bicuspid aortic valve (BAV) aortopathy and analyze the association between aortopathy and BAV phenotype and patterns of valvular dysfunction.Methods Clinical data of 191 patients who underwent the first aortic valve replacement in Fuwai Hospital from June 2017 to March 2018 were retrospectively analyzed, including 143 males and 48 females with an average age of 53.91±12.52 years. All patients underwent multidetector computed tomography (MDCT) and echocardiography before the operation, excluding patients with aortic coarctation. The BAV aortopathy phenotype was classified during operation. The characteristics of BAV aortopathy were analyzed by cluster and artificial analysis. BAV anatomic phenotype was divided into two types according to the direction of valve opening: BAV-AP and BAV-LR.Results Four distinct BAV aortopathy phenotypes were identified: a common type (n=70, 36.6%), with no dilation or mild dilation of aorta; a root type (n=24, 12.6%), with predominant dilatation of aortic sinus; an ascending aorta type (n=72, 37.7%), with predominant dilatation of ascending aorta; an arch type (n=25, 13.1%), with predominant dilatation of aortic arch dilatation. The root type was mainly in young patients, while the arch type was mainly in elderly patients (P<0.05). BAV-AP and aortic insufficiency were most prevalent in root type, while BAV-LR and aortic stenosis were most prevalent in arch type (P<0.05). There were 111 (58.1%) patients undergoing aortic surgery, and the coincidence rate of BAV aortopathy phenotype and aortic surgery was 80.6%.Conclusion According to the location of aortic dilation, BAV aortopathy can be divided into four types. There is an association between BAV aortopathy and valvular phenotype and dysfunction.

Citation： SUN Jing, SUN Cheng, HOU Zhihui, QI Hongxia, QIAN Xiangyang, ZHENG Zhe. Characteristics of bicuspid aortic valve aortopathy and association with valvular phenotype and dysfunction. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2022, 29(6): 729-735. doi: 10.7507/1007-4848.202101004 Copy

1.	Verma S, Siu SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med, 2014, 370(20): 1920-1929.
2.	Gillis E, Kumar AA, Luyckx I, et al. Candidate gene resequencing in a large bicuspid aortic valve-associated thoracic aortic aneurysm cohort: SMAD6 as an important contributor. Front Physiol, 2017, 8: 400.
3.	Mahadevia R, Barker AJ, Schnell S, et al. Bicuspid aortic cusp fusion morphology alters aortic three-dimensional outflow patterns, wall shear stress, and expression of aortopathy. Circulation, 2014, 129(6): 673-682.
4.	Della Corte A, Bancone C, Quarto C, et al. Predictors of ascending aortic dilatation with bicuspid aortic valve: A wide spectrum of disease expression. Eur J Cardiothorac Surg, 2007, 31(3): 397-404.
5.	Schaefer BM, Lewin MB, Stout KK, et al. The bicuspid aortic valve: An integrated phenotypic classification of leaflet morphology and aortic root shape. Heart, 2008, 94(12): 1634-1638.
6.	Fazel SS, Mallidi HR, Lee RS, et al. The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch. J Thorac Cardiovasc Surg, 2008, 135(4): 901-907.
7.	Kang JW, Song HG, Yang DH, et al. Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: Comprehensive evaluation using MDCT and echocardiography. JACC Cardiovasc Imaging, 2013, 6(2): 150-161.
8.	Wojnarski CM, Roselli EE, Idrees JJ, et al. Machine-learning phenotypic classification of bicuspid aortopathy. J Thorac Cardiovasc Surg, 2018, 155(2): 461-469.
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.
10.	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.
11.	Baumgartner H, Hung J, Bermejo J, 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.
12.	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.
13.	孙境, 郑哲. 主动脉瓣二瓣化畸形合并主动脉病变的研究进展. 中国循环杂志, 2020, 35(6): 615-618.
14.	Hardikar AA, Marwick TH. The natural history of guidelines: The case of aortopathy related to bicuspid aortic valves. Int J Cardiol, 2015, 199: 150-153.
15.	Nissen AP, Truong VTT, Alhafez BA, et al. Surgical repair of bicuspid aortopathy at small diameters: Clinical and institutional factors. J Thorac Cardiovasc Surg, 2020, 159(6): 2216-2226.
16.	Kari FA, Fazel SS, Mitchell RS, et al. Bicuspid aortic valve configuration and aortopathy pattern might represent different pathophysiologic substrates. J Thorac Cardiovasc Surg, 2012, 144(2): 516-517.
17.	Sievers HH, Stierle U, Hachmann RM, et al. New insights in the association between bicuspid aortic valve phenotype, aortic configuration and valve haemodynamics. Eur J Cardiothorac Surg, 2016, 49(2): 439-446.
18.	Sun C, Si K, Zheng Y, et al. Phenotypes of aortic valve disease according to detailed anatomical classification of patients who underwent aortic valve replacement surgery. Cardiovasc Pathol, 2019, 41: 1-7.

1. Verma S, Siu SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med, 2014, 370(20): 1920-1929.
2. Gillis E, Kumar AA, Luyckx I, et al. Candidate gene resequencing in a large bicuspid aortic valve-associated thoracic aortic aneurysm cohort: SMAD6 as an important contributor. Front Physiol, 2017, 8: 400.
3. Mahadevia R, Barker AJ, Schnell S, et al. Bicuspid aortic cusp fusion morphology alters aortic three-dimensional outflow patterns, wall shear stress, and expression of aortopathy. Circulation, 2014, 129(6): 673-682.
4. Della Corte A, Bancone C, Quarto C, et al. Predictors of ascending aortic dilatation with bicuspid aortic valve: A wide spectrum of disease expression. Eur J Cardiothorac Surg, 2007, 31(3): 397-404.
5. Schaefer BM, Lewin MB, Stout KK, et al. The bicuspid aortic valve: An integrated phenotypic classification of leaflet morphology and aortic root shape. Heart, 2008, 94(12): 1634-1638.
6. Fazel SS, Mallidi HR, Lee RS, et al. The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch. J Thorac Cardiovasc Surg, 2008, 135(4): 901-907.
7. Kang JW, Song HG, Yang DH, et al. Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: Comprehensive evaluation using MDCT and echocardiography. JACC Cardiovasc Imaging, 2013, 6(2): 150-161.
8. Wojnarski CM, Roselli EE, Idrees JJ, et al. Machine-learning phenotypic classification of bicuspid aortopathy. J Thorac Cardiovasc Surg, 2018, 155(2): 461-469.
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.
10. 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.
11. Baumgartner H, Hung J, Bermejo J, 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.
12. 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.
13. 孙境, 郑哲. 主动脉瓣二瓣化畸形合并主动脉病变的研究进展. 中国循环杂志, 2020, 35(6): 615-618.
14. Hardikar AA, Marwick TH. The natural history of guidelines: The case of aortopathy related to bicuspid aortic valves. Int J Cardiol, 2015, 199: 150-153.
15. Nissen AP, Truong VTT, Alhafez BA, et al. Surgical repair of bicuspid aortopathy at small diameters: Clinical and institutional factors. J Thorac Cardiovasc Surg, 2020, 159(6): 2216-2226.
16. Kari FA, Fazel SS, Mitchell RS, et al. Bicuspid aortic valve configuration and aortopathy pattern might represent different pathophysiologic substrates. J Thorac Cardiovasc Surg, 2012, 144(2): 516-517.
17. Sievers HH, Stierle U, Hachmann RM, et al. New insights in the association between bicuspid aortic valve phenotype, aortic configuration and valve haemodynamics. Eur J Cardiothorac Surg, 2016, 49(2): 439-446.
18. Sun C, Si K, Zheng Y, et al. Phenotypes of aortic valve disease according to detailed anatomical classification of patients who underwent aortic valve replacement surgery. Cardiovasc Pathol, 2019, 41: 1-7.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Characteristics of bicuspid aortic valve aortopathy and association with valvular phenotype and dysfunction

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