Hostname: page-component-cd9895bd7-jkksz Total loading time: 0 Render date: 2024-12-27T23:00:52.891Z Has data issue: false hasContentIssue false
  • English
  • Français

Cardiomyopathy in young adults with classic mitral valve prolapse

Published online by Cambridge University Press:  23 July 2013

Eduard Malev ,
Svetlana Reeva ,
Lyubov Vasina ,
Eugeny Timofeev ,
Asiyet Pshepiy ,
Alexandra Korshunova ,
Maria Prokudina  and
Eduard Zemtsovsky
Eduard Malev*
Affiliation:
Department of Connective Tissue Disorders, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia
Svetlana Reeva
Affiliation:
Department of Connective Tissue Disorders, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia Department of Propaedeutics of Internal Diseases, State Pediatric Medical University, Saint-Petersburg, Russia
Lyubov Vasina
Affiliation:
Department of Microcirculation, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia
Eugeny Timofeev
Affiliation:
Department of Connective Tissue Disorders, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia Department of Propaedeutics of Internal Diseases, State Pediatric Medical University, Saint-Petersburg, Russia
Asiyet Pshepiy
Affiliation:
Department of Connective Tissue Disorders, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia
Alexandra Korshunova
Affiliation:
Department of Connective Tissue Disorders, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia Department of Propaedeutics of Internal Diseases, State Pediatric Medical University, Saint-Petersburg, Russia
Maria Prokudina
Affiliation:
Department of Ultrasound, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia
Eduard Zemtsovsky
Affiliation:
Department of Connective Tissue Disorders, Almazov Federal Heart, Blood and Endocrinology Centre, Saint-Petersburg, Russia Department of Propaedeutics of Internal Diseases, State Pediatric Medical University, Saint-Petersburg, Russia
*
Correspondence to: E. Malev, MD, PhD, Almazov Federal Heart, Blood and Endocrinology Centre, 2 Akkuratova Street, Saint-Petersburg 197341, Russia. Tel: 7-921-910-1394; Fax: 7-812-702-3744; E-mail: edwardmalev@hotmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Background: In some inherited connective tissue diseases with involvement of the cardiovascular system, for example, Marfan syndrome, early impairment of left ventricular function, which have been described as Marfan-related cardiomyopathy has been reported. Our aim was to evaluate the left ventricular function in young adults with mitral valve prolapse without significant mitral regurgitation using two-dimensional strain imaging and to determine the possible role of the transforming growth factor-β pathway in its deterioration. Methods: We studied 78 young adults with mitral valve prolapse without mitral regurgitation in comparison with 80 sex-matched and age-matched healthy individuals. Longitudinal strain and strain rates were defined using spackle tracking. Concentrations of transforming growth factor-β1 and β2 in serum were determined by enzyme-linked immunosorbent assays. Results: In 29 patients, classic relapse was identified with a leaflet thickness of ≥ 5 mm; 49 patients had a non-classic mitral valve prolapse. Despite the similar global systolic function, a significant reduction in global strain was found in the classic group (−15.5 ± 2.9%) compared with the non-classic group (−18.7 ± 3.8; p = 0.0002) and the control group (−19.6 ± 3.4%; p < 0.0001). In young adults with non-classic prolapse, a reduction in longitudinal deformation was detected only in septal segments. Transforming growth factor-β1 and β2 serum levels were elevated in patients with classic prolapse as compared with the control group and the non-classic mitral valve prolapse group. Conclusions: These changes in the deformations may be the first signs of deterioration of the left ventricular function and the existence of primary cardiomyopathy in young adults with mitral valve prolapse, which may be caused by increased transforming growth factor-β signalling.

Keywords

Mitral valve prolapsecardiomyopathyleft ventricular systolic functionmyocardial deformationtransforming growth factor-β
Type
Original Articles
Information
Cardiology in the Young , Volume 24 , Issue 4 , August 2014 , pp. 694 - 701
Copyright
Copyright © Cambridge University Press 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Kiotsekoglou, A, Saha, S, Moggridge, JC, et al. Impaired biventricular deformation in Marfan syndrome: a strain and strain rate study in adult unoperated patients. Echocardiography 2011; 28: 416–430.Google Scholar
2. Mcdonnell, NB, Gorman, BL, Mandel, KW, et al. Echocardiographic findings in classical and hypermobile Ehlers–Danlos syndromes. Am J Med Genet A 2006; 140: 129–136.Google Scholar
3. Migliaccio, S, Barbaro, G, Fornari, R, et al. Impairment of diastolic function in adult patients affected by osteogenesis imperfecta clinically asymptomatic for cardiac disease: casuality or causality? Int J Cardiol 2009; 131: 200–203.Google Scholar
4. Nguyen, LD, Terbah, M, Daudon, P, Martin, L. Left ventricular systolic and diastolic function by echocardiogram in pseudoxanthoma elasticum. Am J Cardiol 2006; 97: 1535–1537.Google Scholar
5. Alpendurada, F, Wong, J, Kiotsekoglou, A, et al. Evidence for Marfan cardiomyopathy. Eur J Heart Fail 2010; 12: 1085–1091.Google Scholar
6. Pope, AJ, Sands, GB, Smaill, BH, LeGrice, IJ. Three-dimensional transmural organization of perimysial collagen in the heart. Am J Physiol Heart Circ Physiol 2008; 295: H1243–H1252.Google Scholar
7. Murphy-Ryan, M, Psychogios, A, Lindor, NM. Hereditary disorders of connective tissue: a guide to the emerging differential diagnosis. Genet Med 2010; 12: 344–354.Google Scholar
8. Grau, JB, Pirelli, L, Yu, PJ, et al. The genetics of mitral valve prolapse. Clin Genet 2007; 72: 288–295.Google Scholar
9. Kyndt, F, Gueffet, JP, Probst, V, et al. Mutations in the gene encoding filamin A as a cause for familial cardiac valvular dystrophy. Circulation 2007; 115: 40–49.Google Scholar
10. Bonow, RO, Carabello, BA, Chatterjee, K, et al. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice. J Am Coll Cardiol 2008; 52: e1–e142.Google Scholar
11. 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: 1298–1304.Google Scholar
12. Anyanwu, AC, Adams, DH. Etiologic classification of degenerative mitral valve disease: Barlow's disease and fibroelastic deficiency. Semin Thorac Cardiovasc Surg 2007; 19: 90–96.Google Scholar
13. Sasaki, A, Masuda, Y, Ohta, Y, et al. Filamin associates with Smads and regulates transforming growth factor-β signaling. J Biol. Chem 2001; 276: 17871–17877.Google Scholar
14. Ng, CM, Cheng, A, Myers, L, et al. TGF-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome. J Clin Invest 2004; 114: 1586–1592.Google Scholar
15. Khan, R, Sheppard, R. Fibrosis in heart disease: understanding the role of transforming growth factor-beta in cardiomyopathy, valvular disease and arrhythmia. Immunology 2006; 118: 10–24.Google Scholar
16. Delhomme, C, Casset-Senon, D, Babuty, D, et al. A study of 36 cases of mitral valve prolapse by isotopic ventricular tomography. Arch Mal Coeur Vaiss 1996; 89: 1127–1135.Google Scholar
17. Lumia, FJ, LaManna, MM, Atfeh, M, Maranhao, V. Exercise first-pass radionuclide assessment of left and right ventricular function and valvular regurgitation in symptomatic mitral valve prolapse. Angiology 1989; 40: 443–449.Google Scholar
18. Casset-Senon, D, Babuty, D, Philippe, L, et al. Fourier phase analysis of SPECT equilibrium radionuclide angiography in symptomatic patients with mitral valve prolapse without significant mitral regurgitation: assessment of biventricular functional abnormalities suggesting a cardiomyopathy. J Nucl Cardiol 2000; 7: 471–477.Google Scholar
19. Marciniak, A, Sutherland, GR, Marciniak, M, et al. Prediction of postoperative left ventricular systolic function in patients with chronic mitral regurgitation undergoing valve surgery – the role of deformation imaging. Eur J Cardiothorac Surg 2011; 40: 1131–1137.Google Scholar
20. Lancellotti, P, Cosyns, B, Zacharakis, D, et al. Importance of left ventricular longitudinal function and functional reserve in patients with degenerative mitral regurgitation: assessment by two-dimensional speckle tracking. J Am Soc Echocardiogr 2008; 21: 1331–1336.Google Scholar
21. Mor-Avi, V, Lang, RM, Badano, LP, et al. Current and evolving echocardiographic techniques for the quantitative evaluation of cardiac mechanics: ASE/EAE consensus statement on methodology and indications endorsed by the Japanese Society of Echocardiography. Eur J Echocardiogr 2011; 12: 167–205.Google Scholar
22. Lancellotti, P, Moura, L, Pierard, LA, et al. European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 2: mitral and tricuspid regurgitation (native valve disease). Eur J Echocardiogr 2010; 11: 307–332.CrossRefGoogle ScholarPubMed
23. Yiginer, O, Keser, N, Ozmen, N, et al. Classic mitral valve prolapse causes enlargement in left ventricle even in the absence of significant mitral regurgitation. Echocardiography 2012; 29: 123–129.Google Scholar
24. Rizzo, S, Carturan, E, Gerosa, G, et al. Non syndromic mitral valve prolapse: evidence of TGF-beta pathway activation in surgically resected leaflets. Circulation 2008; 118: S1072 [Abstract].Google Scholar
25. Hulin, A, Deroanne, CF, Lambert, CA, et al. Metallothionein-dependent up-regulation of TGF-β2 participates in the remodelling of the myxomatous mitral valve. Cardiovasc Res 2012; 93: 480–489.Google Scholar
26. Klein, G, Schaefer, A, Hilfiker-Kleiner, D, et al. Increased collagen deposition and diastolic dysfunction but preserved myocardial hypertrophy after pressure overload in mice lacking PKCepsilon. Circ Res 2005; 96: 748–755.Google Scholar
27. Meyer, A, Wang, W, Qu, J, et al. Platelet TGF-β1 contributions to plasma TGF-β1, cardiac fibrosis, and systolic dysfunction in a mouse model of pressure overload. Blood 2012; 119: 1064–1074.Google Scholar
28. Maron, BJ, Towbin, JA, Thiene, G, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee. Circulation 2006; 113: 1807–1816.CrossRefGoogle ScholarPubMed
29. Bijnens, BH, Cikes, M, Claus, P, Sutherland, GR. Velocity and deformation imaging for the assessment of myocardial dysfunction. Eur J Echocardiogr 2009; 10: 216–226.Google Scholar
30. Matos-Souza, JR, Fernandes-Santos, ME, Hoehne, EL, et al. Isolated mitral valve prolapse is an independent predictor of aortic root size in a general population. Eur J Echocardiogr 2010; 11: 302–305.Google Scholar
31. Matt, P, Schoenhoff, F, Habashi, J, et al. Circulating transforming growth factor-beta in Marfan syndrome. Circulation 2009; 120: 526–532.Google Scholar
32. Jones, JA, Spinale, FG, Ikonomidis, JS. Transforming growth factor-beta signaling in thoracic aortic aneurysm development: a paradox in pathogenesis. J Vasc Res 2009; 46: 119–137.CrossRefGoogle ScholarPubMed
33. Han, Y, Peters, DC, Salton, CJ, et al. Cardiovascular magnetic resonance characterization of mitral valve prolapse. JACC Cardiovasc Imaging 2008; 1: 294–303.Google Scholar