The mitral insufficiency in connective tissue dysplasia. What should a doctor know today? A review

Cover Page

Cite item

Full Text

Abstract

The problem of the mitral valve (MV) dysfunction, both in isolation and combination forms with other anomalies and lesions of connective tissue structures, is very current. Mitral insufficiency (MI) in case of the connective tissue dysplasia is the most common valvular pathology, accompanied by different degrees of severity. Nowadays the progradient development of functional and clinical symptoms of this disease has been studied, the questions of epidemiology, diagnosis and prognosis have been clarified and reconsidered. There are important issues of correct diagnosis, time and method of treatment, the decision of usage a valve-repair or valve prosthesis in the certain clinical situation. All these practical algorithm steps depend on the clinical and surgical experience of the medical institutions which work with this pathology. The purpose of this article is to expand the knowledge of practical doctors in the issue of MI in case of connective tissue dysplasia, including the questions from the model of the MV structure to modern methods of treatment which are described in the contemporary literature.

About the authors

Yulia B. Martyanova

Federal Center for Cardiovascular Surgery

Author for correspondence.
Email: martyanova911@mail.ru
ORCID iD: 0000-0002-4344-2672

cardiologist

Russian Federation, Astrakhan

Elena N. Chernysheva

Astrakhan State Medical University

Email: martyanova911@mail.ru
ORCID iD: 0000-0001-8884-1178

D. Sci. (Med.), Assoc. Prof.

Russian Federation, Astrakhan

Dmitry A. Kondratiev

Federal Center for Cardiovascular Surgery

Email: martyanova911@mail.ru
ORCID iD: 0000-0002-9158-8799

Cand. Sci. (Med.)

Russian Federation, Astrakhan

Sergei L. Dzemeshkevich

Petrovsky National Research Center of Surgery

Email: martyanova911@mail.ru
ORCID iD: 0000-0003-0939-1063

D. Sci. (Med.), Prof.

Russian Federation, Moscow

Dmitry G. Tarasov

Federal Center for Cardiovascular Surgery

Email: martyanova911@mail.ru
ORCID iD: 0000-0002-6065-2487

Cand. Sci. (Med.)

Russian Federation, Astrakhan

Elena A. Ljaljukova

Omsk State Medical University

Email: martyanova911@mail.ru
ORCID iD: 0000-0003-4878-0838

D. Sci. (Med.), Prof.

Russian Federation, Omsk

References

  1. Дземешкевич С.Л., Стивенсон Л.У. Болезни митрального клапана. Функция, диагностика, лечение. 2-е изд., доп. М.: ГЭОТАР-Медиа, 2015 [Dzemeshkevich SL, Stivenson LU. Bolezni mitral'nogo klapana. Funktsiia, diagnostika, lechenie. 2-e izd., dop. Moscow: GEOTAR-Media, 2015 (in Russian)].
  2. Freed LA, Levy D, Levine RA, et al. Prevalence and outcome of mitral valve prolapse. N Engl J Med. 1999;341(1):1-7. doi: 10.1056/NEJM199907013410101
  3. Митрофанова Л.Б. Клапанные пороки сердца. Новый взгляд на этиологию, патогенез и морфологию. СПб: Медицинское издательство, 2007 [Mitrofanova LB. Klapannye poroki serdtsa. Novyi vzgliad na etiologiiu, patogenez i morfologiiu. Saint Petersburg: Meditsinskoe izdatel'stvo, 2007 (in Russian)].
  4. Земцовский Э.В., Малев Э.Г., Березовская Г.А., и др. Наследственные нарушения соединительной ткани в кардиологии. Диагностика и лечение. Российские рекомендации (I пересмотр). Российский кардиологический журнал. 2013;1(99, прил.1):1-32. Режим доступа: https://www.dzhmao.ru/spez/RKO/recom2012_soedTkani.pdf. Ссылка активна на 21.06.2022 [Zemtsovskii EV, Malev EG, Berezovskaia GA, et al. Nasledstvennye narusheniia soedinitel'noi tkani v kardiologii. Diagnostika i lechenie. Rossiiskie rekomendatsii (I peresmotr). Russian Journal of Cardiology. 2013;1(99, Suppl. 1):1-32. Available at: https://www.dzhmao.ru/spez/RKO/recom2012_soedTkani.pdf. Accessed: 21.06.2022 (in Russian)].
  5. Клеменов А.В. Пролапс митрального клапана: современные представления и нерешенные вопросы (обзор). Современные технологии в медицине. 2017;9(3):126-37 [Кlemenov AV. Mitral valve prolapse: current views and challenges (review). Sovremennye tehnologii v medicine. 2017;9(3):126-37 (in Russian)]. doi: 10.17691/stm2017.9.3.17
  6. Rabkin E, Aikawa M, Stone JR, et al. Activated interstitial myofibroblasts express catabolic enzymes and mediate matrix remodeling in myxomatous heart valves. Circulation 2001;104(21):2525-32. doi: 10.1161/hc4601.099489
  7. Guy TS, Hill AC. Mitral valve prolapse. Annu Rev Med. 2012;63(1):277-92. doi: 10.1146/annurev-med-022811-091602
  8. Cole VG, Chan D, Hickey A, Wilcken D.E. Collagen composition of normal and myxomatous human mitral heart valves. Biochem J. 1984;219(2):451. doi: 10.1042/bj2190451
  9. Barber JE, Kasper FK, Ratliff NB, et al. Mechanical properties of myxomatous mitral valves. J Thorac Cardiovasc Surg. 2001;122(5):995. doi: 10.1067/mtc.2001.117621
  10. Adams DH, Carabello BA, Castillo J. Mitral valve regurgitation. In: Fuster V, Walsh RA, Harrington RA, eds. Hurst’s the Heart. 13th ed. New York: McGraw-Hill, 2010, p. 1721-37.
  11. Hayek E, Gring CN, Griffin BP. Mitral valve prolapse. Lancet. 2005;365(9458):507-18. doi: 10.1016/S0140-6736(05)17869-6
  12. Avierinos JF, Gersh BJ, Melton LJ, et al. Natural history of asymptomatic mitral valve prolapse in the community. Circulation. 2002;106(11):1355-61. doi: 10.1161/01.cir.0000028933.34260.09
  13. Silbiger JJ. Anatomy, mechanics, and pathophysiology of the mitral annulus. Am Heart J. 2012;164(2):163-76. doi: 10.1016/j.ahj.2012.05.014
  14. Niu Z, Chan V, Mesana T, Ruel M. The evolution of mitral valve prolapse: insights, from the Framingham Heart Study. J Thorac Dis. 2016;8(8):E827-8. doi: 10.21037/jtd.2016.07.58
  15. Delling FN, Rong J, Larson MG, et al. Evolution of mitral valve prolapse: insights from the Framingham Heart Study. Circulation. 2016;133(17):1688-95. doi: 10.1161/CIRCULATIONAHA.115.020621
  16. Salustri A, Almaghrabi A. Mitral valve disease correlation between the most important echocardiographic parameters and haemodynamics. e-Journal Cardiol Pract. 2018;16(24). Available at: https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-16/Mitral-Valve-Disease-correlation-between-the-most-important-echocardiographic-parameters-and-haemodynamics. Accessed: 21.06.2022.
  17. Bayer-Topilsky T, Suri RM, Topilsky Y, et al. Mitral valve prolapse, psychoemotional status, and quality of life: prospective investigation in the current era. Am J Med. 2016;129(10):1100-9. doi: 10.1016/j.amjmed.2016.05.004
  18. Dawber TR, Meadors GF, Moore FE Jr. Epidemiological approaches to heart disease: the Framingham Study. Am J Public Health Nations Health. 1951;41(3):279-86. doi: 10.2105/ajph.41.3.279
  19. Земцовский Э.В., Малев Э.Г. Пролапс митрального клапана: современный взгляд на проблему. Бюллетень Федерального центра сердца, крови и эндокринологии им. В.А. Алмазова. 2011;3:25-30 [Zemtsovsky EV, Malev EG. Mitral valve prolapse: modern approach to the problem. Bulletin of Almazov Federal Heart, Blood and Endocrinology Centre. 2011;3:25-30 (in Russian)].
  20. Enriquez-Sarano M, Basmadjian AJ, Rossy A, et al. Progression of mitral regurgitation: a prospective Doppler echocardiographic study. J Am Coll Cardiol. 1999;34(4):1137-44. doi: 10.1016/s0735-1097(99)00313-7
  21. Samad Z, Kaul P, Shaw LK, et al. Impact of early surgery on survival of patients with severe mitral regurgitation. Heart. 2011;97(3):221. doi: 10.1136/hrt.2010.202432
  22. Avierinos JF, Inamo J, Grigioni F, et al. Sex differences in morphology and outcomes of mitral valve prolapse. Ann Intern Med. 2008;149(11):787-95. doi: 10.7326/0003-4819-149-11-200812020-00003
  23. Малев Э.Г., Пшепий А.Р., Васина Л.В., и др. Ремоделирование миокарда и диастолическая дисфункция левого желудочка при пролапсе митрального клапана. Российский кардиологический журнал. 2013;100(2):12-7 [Malev EG, Pshepiy AR, Vasina LV, et al. Left ventricular remodelling and diastolic dysfunction in mitral valve prolapse. Russian Journal of Cardiology. 2013;100(2):12-7 (in Russian)]. doi: 10.15829/1560-4071-2013-2-12-17
  24. Freed LA, Benjamin EJ, Levy D, et al. Mitral valve prolapse in the general population: the benign nature of echocardiographic features in the Framingham Heart Study. J Am Coll Cardiol. 2002;40(7):1298-304. doi: 10.1016/s0735-1097(02)02161-7
  25. Levine RA, Stathogiannis E, Newell JB, et al. Reconsideration of echocardiographic standards for mitral valve prolapse: lack of association between leaflet displacement isolated to the apical four chamber view and independent echocardiographic evidence of abnormality. J Am Coll Cardiol. 1988;11(5):1010-9. doi: 10.1016/s0735-1097(98)90059-6
  26. Uretsky S, Gillam L, Lang R, et al. Discordance between echocardiography and MRI in the assessment of mitral regurgitation severity: a prospective multicenter trial. J Am Coll Cardiol. 2015;65(11):1078-88. doi: 10.1016/j.jacc.2014.12.047
  27. Земцовский Э.В., Малев Э.Г., Реева С.В. Пролапс митрального клапана: «много шума из ничего» или реально нерешенная проблема. Кардио- васкулярная терапия и профилактика. 2010;9(8):69-74 [Zemtsovsky EV, Malev EG, Reeva SV. Mitral valve prolapse: «Much Ado About Nothing», or a real unsolved problem? Cardiovascular Therapy and Prevention. 2010;9(8):69-74 (in Russian)].
  28. Anyanwu AC, Adams DH. Etiologic classification of degenerative mitral valve disease: Barlows disease and fibroelastic deficiency. Semin Thorac Cardiovasc Surg. 2007;19(2):90-6. doi: 10.1053/j.semtcvs.2007.04.002
  29. Enriquez-Sarano M, Tajik AJ, Schaff HV, et al. Echocardiographic prediction of left ventricular function after correction of mitral regurgitation: results and clinical implication. J Am Coll Cardiol. 1994;24(6):1536-43. doi: 10.1016/0735-1097(94)90151-1
  30. Tribouilloy C, Grigioni F, Avierinos JF, et al. Survival implication of left ventricular end-systolic diameter in mitral regurgitation due to flail leaflets a long-term follow-up multicenter study. J Am Coll Cardiol. 2009;54(21):1961-8. doi: 10.1016/j.jacc.2009.06.047
  31. Bolling SF, Li S, O'Brien SM, et al. Predictors of mitral valve repair: clinical and surgeon factors. Ann Thorac Surg. 2010;90(6):1904-11;discussion 1912. doi: 10.1016/j.athoracsur.2010.07.062
  32. Braunberger E, Deloche A, Berrebi A, et al. Very long-term results (more than 20 years) of valve repair with Carpentiers techniques innonrheumatic mitral valve insufficiency. Circulation. 2001;104(12 Suppl. 1):I8-11.
  33. Mohty D, Orszulak TA, Schaff HV, et al. Very long-term survival and durability of mitral valve repair for mitral valve prolapse. Circulation. 2001;104(12 Suppl. 1):I1-7. doi: 10.1161/hc37t1.094903
  34. Sayeed R. Mitral valve disease: when should we call in the cardiac surgeon. e-Journal Cardiol Pract. 2018;16(28). Available at: https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-16/Mitral-valve-disease-when-should-we-call-in-the-cardiac-surgeon. Accessed: 21.06.2022.
  35. Ling LH, Enriquez-Sarano M, Sevard JB, et al. Early surgery in patients with mitral regurgitation due to flail leaflets: a long-term outcome study. Circulation. 1997;96(6):1819-25. doi: 10.1161/01.cir.96.6.1819
  36. Castillo JG, Anyanwu AC, Fuster V, Adams DH. A near 100% repair rate for mitral valve prolapse is achievable in a reference center: implications for future guidelines. J Thorac Cardiovasc Surg. 2012;144(2):308-12. doi: 10.1016/j.jtcvs.2011.12.054
  37. Enriquez-Sarano M. Timing of mitral valve surgery. Heart. 2002;87(1):79-85. doi: 10.1136/heart.87.1.79
  38. Ross J Jr. Adaptations of the left ventricle to chronic volume overload. Circ Res. 1974;35(2; Suppl. II):64-70. doi: 10.1016/0002-9149(75)90774-2
  39. Dziadzko V, Clavel MA, Dziadzko M, et al. Outcome and undertreatment of mitral regurgitation: a community cohort study. Lancet. 2018;391(10124):960-9. doi: 10.1016/S0140-6736(18)30473-2
  40. Quintana E, Suri RM, Thalji NM, et al. Left ventricular dysfunction after mitral valve repair – the fallacy of «normal» preoperative myocardial function. J Thorac Cardiovasc Surg. 2014;148(6):2752-60. doi: 10.1016/j.jtcvs.2014.07.029
  41. Клинические рекомендации. Пролапс митрального клапана. М.: МЗ РФ, 2016. Режим доступа: https://racvs.ru/clinic/files/2016/Mitral-valve-prolapse.pdf. Ссылка активна на 21.06.2022 [Klinicheskie rekomendatsii. Prolaps mitral'nogo klapana. Moscow: MZ RF, 2016. Available at: https://racvs.ru/clinic/files/2016/Mitral-valve-prolapse.pdf. Accessed: 21.06.2022 (in Russian)].
  42. Земцовский Э.В. Пролапс митрального клапана: монография. СПб: Общество «Знание», 2010 [Zemtsovsky EV. Prolaps mitral'nogo klapana: monografiia. Saint Petersburg: Obshchestvo Znanie, 2010 (in Russian)].
  43. Назаров В.М., Афанасьев А.В., Демин И.И. Коррекция митральной недостаточности при болезни Барлоу. Патология кровообращения и кардиохирургия. 2014;18(1):70-3 [Nazarov VM, Afanasev AV, Demin II. Mitral valve repair in Barlow disease. Patologiya krovoobrashcheniya i kardiokhirurgiya = Circulation Pathology and Cardiac Surgery. 2014;18(1):70-3 (in Russian)]. doi: 10.21688/1681-3472-2014-1-70-73
  44. Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014;63(22):2438-88. doi: 10.1016/j.jacc.2014.02.537
  45. Magne J, Lancellotti P, Pierard LA. Exercise pulmonary hypertension in asymptomatic degenerative mitral regurgitation. Circulation 2010;122:33. doi: 10.1161/CIRCULATIONAHA.110.938241
  46. David TE, Ivanov J, Armstrong S, et al. A comparison of outcomes of mitral valve repair for degenerative disease with posterior, anterior, and bileaflet prolapse. J Thorac Cardiovasc Surg. 2005;130(5):1242-9. doi: 10.1016/j.jtcvs.2005.06.046
  47. Enriquez-Sarano M, Avierinos JF, Messika-Zeitoun D, et al. Quantitative determinants of the outcome of asymptomatic mitral regurgitation. N Engl J Med. 2005;352(9):875-83. doi: 10.1056/NEJMoa041451
  48. Grigioni F, Tribouilloy C, Avierinos JF, et al. Outcomes in mitral regurgitation due to flail leaflets a multicenter European study. JACC Cardiovasc Imaging. 2008;1(2):133-41. doi: 10.1016/j.jcmg.2007.12.005
  49. Grigioni F, Enriquez-Sarano M, Ling LH, et al. Sudden death in mitral regurgitation due to flail leaflet. J Am Coll Cardiol. 1999;34(7):2078-85. doi: 10.1016/s0735-1097(99)00474-x
  50. Enriquez-Sarano M, Suri RM, Clavel MA, et al. Is there an outcome penalty linked to guideline-based indications for valvular surgery? Early and long-term analysis of patients with organic mitral regurgitation. J Thorac Cardiovasc Surg. 2015;150(1):50-8. doi: 10.1016/j.jtcvs.2015.04.009
  51. Eguchi K, Ohtaki E, Matsumura T, et al. Pre-operative atrial fibrillation as the key determinant of outcome of mitral valve repair for degenerative mitral regurgitation. Eur Heart J. 2005;26:1866. doi: 10.1093/eurheartj/ehi272
  52. Flameng W, Herijgers P, Bogaerts K. Recurrence of mitral regurgitation after mitral valve repair in degenerative valve disease. Circulation. 2003;107(12):1609-13. doi: 10.1161/01.CIR.0000058703.26715.9D
  53. Tribouilloy CM, Enriquez-Sarano M, Schaff HV, et al. Impact of preoperative symptoms on survival after surgical correction of organic mitral regurgitation: rationale for optimizing surgical indications. Circulation. 1999;99(3):400-5. doi: 10.1161/01.cir.99.3.400
  54. Vassileva CM, Mishkel G, McNeely C, et al. Long-term survival of patients undergoing mitral valve repair and replacement: a longitudinal analysis of Medicare fee-for-service beneficiaries. Circulation. 2013;127(18):1870-6. doi: 10.1161/CIRCULATIONAHA.113.002200
  55. El Sabbagh A, Reddy YNV, Nishimura RA. Mitral Valve Regurgitation in the contemporary Era. JACC Cardiovasc Imaging. 2018;11(4):628-43. doi: 10.1016/j.jcmg.2018.01.009
  56. Кондратьев Д.А., Молочков А.В., Мартьянова Ю.Б., и др. Правосторонняя мини-торакотомия как безопасный и эффективный доступ в хирургии соединительнотканной дисплазии митрального клапана. Клиническая и экспериментальная хирургия. Журнал им. академика Б.В. Петровского. 2020;8(3):40-50 [Kondratyev DA, Molochkov AV, Martyanova YB, et al. Right minithoracotomy as safe and effective approach for surgical treatment of mitral valve dysplasia. Clinical and Experimental Surgery. Petrovsky Journal. 2020;8(3):40-50 (in Russian)]. doi: 10.33029/2308-1198-2020-8-3-40-50

Supplementary files

Supplementary Files
Action
1. JATS XML
Download
2. Fig. 1. The scheme of bileaflet prolapse of MV.

Download (49KB)
Indexing metadata
3. Fig. 2. The left ventricle adaptation in case of chronic MV regurgitation.

Download (78KB)
Indexing metadata
4. Fig. 3. The guideline for the management of patients with primary MV regurgitation (adapted from [56]).

Download (237KB)
Indexing metadata

Copyright (c) 2022 Eco-Vector

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies