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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: [email protected]
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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 

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