BICUSPID AORTIC VALVE (A DEVELOPMENT OF INSIGHT INTO VALVULOPATHIES)
https://doi.org/10.15829/1560-4071-2014-5-49-54
Abstract
Aim. To study different ways of development and combinational abilities of pathological changes common for bicuspid aortic valvulopathy. Material and methods. Totally 207 patients included, who had during previous 5 years been operated for progression of inherited aortic valve defect. The age varied from 17 to 75 years (median 49,6±0,9 y.), men to women — 3:1. Comorbidities included rheumatic fever in 33 (15,9%) patients and infectious endocarditis in the past in 45 (21,7%). In 111 (53,6%) patients aortic stenosis dominated and distension of ascending aorta — in 82 (39,6%) patients.
Results. For most of patients mechanical prostheses were used and only for 12 — biological; 13 patients underwent valvulopastic or no any operation. One third of patients (31,9%) required a prosthesis of ascending aorta. Combinations of additional procedures (as aortic valve plastic or prosthesis, tricuspid plastic, left atrium plastic, septal defects sewing, coronary bypass grafting) were not unusual — in 57. In-hospital mortality reached 2,9%.
Conclusion. Bicuspid aortic valve is a specific kind of valvulopathy, commonly combined with aortopathy. The development of this pathology is probably inherited. Clinical picture of the defect develops during time and its exacerbation might be accelerated by rheumathic fever and infectious endocarditis. Surgical treatment is effective.
About the Authors
S. L. DzemeshkevitchRussian Federation
V. A. Ivanov
Russian Federation
E. R. Charchian
Russian Federation
E. P. Evseev
Russian Federation
Yu. V. Frolova
Russian Federation
A. N. Lugovoy
Russian Federation
S. V. Fedulova
Russian Federation
V. V. Khovrin
Russian Federation
A. A. Bukaeva
Russian Federation
E. V. Zaklyazminskaya
Russian Federation
References
1. Dzemeshkevich S. L., Stevenson SL, Alexi-Meskhishvili VV. Disease aortic valve (function, diagnostics, treatment). M., GEOTAR-MED, 2004, 325 C. Russian (Дземешкевич С. Л., Стивенсон Л. У., Алекси-Месхишвили В. В. Болезни аортального клапана (функция, диагностика, лечение). М., ГЭОТАР-МЕД, 2004, 325 с).
2. Siu S, Silversides C. Bicuspid Aortic Valve Disease. J Am Coll Cardiol 2010; 55: 2789-800.
3. Mordi J, Tzemos N. Bicuspid Aorte Valve Disease: a comprehensive review. Cardiology Research and Practice. Hindawi Publishing Corporation, volume 2012, p. 2-5.
4. Padang R, Bagnall R, Semsarian Ch. Genetic Basis of Familial Valvular Heart disease. Circ Cardiovasc Genet, 2012, 5: 569-80.
5. Padang R, Bannon P, Jeremy R, et al. The genetic and molecular basis of bicuspid aortic valve associated thoracic aortopathy: a link to phenotype heterogenecity. Ann Cardiothorac Surg, 2013; 2 (1): 83-91.
6. Lokshin PS. Artificial circulation. In the book: National guide "Anesthesiology", Ed. But, GEOTAR, M,2011, s.626-48. Russian (Локшин Л. С. Искусственное кровообращение. В кн.: Национальное руководство "Анестезиология", ред. А.А. Бунятян, ГЭОТАР, М.,2011, с.626-48).
7. Trekova NA. Anesthetic management operations on the heart and the aorta. Anesthesiology and reanimatology) 2013, 2: 6-10. Russian (Трекова Н. А. Анестезиологическое обеспечение операций на сердце и аорте. Анестезиология и реаниматология 2013, 2: 6-10).
8. Dzemeshkevich SL, Dementieva II, Zhidkov IL, et al. Long-term conservation of the heart in the solution of the Console and Custodial (experimental study). Transplantul. and arts. bodies, 1996; 3-4: 67-74. Russian (Дземешкевич С. Л., Дементьева И. И., Жидков И. Л. и др. Длительная консервация сердца в растворе Консол и Кустодиол (экспериментальное исследование). Трансплантол. и искусств. органы, 1996; 3-4: 67-74).
9. Warnes CA, Williams RC, Bashore TM, et al. ACC/AHA 2008 Guidelines for the management of adult with congenital heart disease. J Am Coll Cardiol 2008; 52: 1-121.
10. Vahanian A, Alfieri O, Andreotti T, et al. Guidelines on the management of valvular heart disease (version 2012) of the ESC and EACTS. European Heart Journal (2012); 33: 2451-96.
11. Krylova NS, Poteshkina N. G. Gipertrophy myopathy in the elderly. Heart failure, 2011; 12, 5: 309-11. Russian (Крылова Н. С., Потешкина Н. Г. Гипетрофическая кардиомиопатия у лиц пожилого возраста. Сердечная недостаточность, 2011; 12, 5: 309-11).
12. Maron B, Casey S, Hauser R, et al. Clinical course of hypertrophic cardiomyopathy with survival to advanced age. J Am Coll Cardiol, 2003; 42 (5): 882-8.
13. Erscan T., Ekisi F., Atalay S. et al. The prevalence of bicuspid aortic valve in newborns by echocardiography screening. American Heart Journal, 2005; 150, 3: 513-5.
14. Stewart S., Ahmed R., Travill C. et al. Coarctation of the aorta life and health 20-44 years after surgery repair. British Heart Journal, 1993; 69, 1: 65-70.
15. Fedak P, Verma S, David T, et al. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation, 2002; 106, 8: 900-4.
16. Calloway T, Martin J, Zhang X, et al. Risk factors for aortic valve disease in bicuspid aortic valve: a family-based study. Am J Med Genet A, 2011; 155, 5: 1015-20.
17. Dzemeshkevich A. S., Ruskin CENTURIES, Malikova MS et al. Dysplasia mitral valve prolapse in adults: the choice of surgical technique. Surgery, 2013; 2: 40-5. Russian (Дземешкевич А. С., Раскин В. В., Маликова М. С. и др. Дисплазии митрального клапана у взрослых: выбор хирургической методики. Хирургия, 2013; 2: 40-5).
18. Hahn R, Roman M, Mogtader A, et al. Association of aortic dilatation with regurgitant, stenotic and functionally normal bicuspid aortic valves. J Am Coll Cardiol 1992; 19: 283-8.
19. Bonderman D, Charchbaghi-Schnell E, Maurer G, et al. Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation 1999; 99: 2138-43.
20. Fedak P, de Sa M, Verma S, et al. Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. J Thorac Catdiovasc Surg 2003; 126: 797-806.
21. Boyum J, Fellinger E, Schmoker J, et al. Matrix metalloproteinase activity in thoracic aortic aneurisms associated with bicuspid and tricuspid aortic valves. J Thorac Cardiovasc.Surg, 2004; 127: 686-91.
22. Loscalzo M, Goh D, Loeys B, et al. Familial thoracic aortic dilatation and bicomissural aortic valve: a prospective analysis of natural history and inheritance. Am J Med Genet A, 2007; 143A: 1960-7.
23. OMIM (On-line Mendelian Inheritance in Man) www.ncbi.nlm.nih.gov/omim
24. Pepe G, Nistri S, Giusti B, et al. Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome. BMC Medical Genetics 2014, 15: 23.
25. Groenink M, den Hartog AW, Franken R, et al. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial. Eur Heart J. 2013 Dec; 34 (45): 3491-500.
26. Mullen M, Flather M, Jin X, et al. A prospective, randomized, placebo-controlled, double- blind, multicenter study of the effects of irbesartan on aortic dilatation in Marfan syndrome (AIMS trial): study protocol. Mullen et al. Trials 2013, 14: 408.
27. Garg V, Muth AN, Ransom JF, et al. Mutations in NOTCH1 cause aortic valve disease. Nature 2005; 437: 270-4.
28. Tan HL, Glen E, Topf A, et al. Nonsynonymous variants in the SMAD6 gene predispose to congenital cardiovascular malformation. Hum. Mutat. 2012; 33: 720-7.
Review
For citations:
Dzemeshkevitch S.L., Ivanov V.A., Charchian E.R., Evseev E.P., Frolova Yu.V., Lugovoy A.N., Fedulova S.V., Khovrin V.V., Bukaeva A.A., Zaklyazminskaya E.V. BICUSPID AORTIC VALVE (A DEVELOPMENT OF INSIGHT INTO VALVULOPATHIES). Russian Journal of Cardiology. 2014;(5):49-54. (In Russ.) https://doi.org/10.15829/1560-4071-2014-5-49-54