title: Cardiomyopathy in young adults with classic mitral valve prolapse.
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.
authors.affiliation: 1Department of Connective Tissue Disorders,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Eduard
authors.initials: E
authors.lastname: Malev
authors.affiliation: 1Department of Connective Tissue Disorders,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Svetlana
authors.initials: S
authors.lastname: Reeva
authors.affiliation: 3Department of Microcirculation,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Lyubov
authors.initials: L
authors.lastname: Vasina
authors.affiliation: 1Department of Connective Tissue Disorders,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Eugeny
authors.initials: E
authors.lastname: Timofeev
authors.affiliation: 1Department of Connective Tissue Disorders,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Asiyet
authors.initials: A
authors.lastname: Pshepiy
authors.affiliation: 1Department of Connective Tissue Disorders,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Korshunova
authors.affiliation: 4Department of Ultrasound,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Maria
authors.initials: M
authors.lastname: Prokudina
authors.affiliation: 1Department of Connective Tissue Disorders,Almazov Federal Heart,Blood and Endocrinology Centre,Saint-Petersburg,Russia.
authors.forename: Eduard
authors.initials: E
authors.lastname: Zemtsovsky
mesh_terms: D000293:Adolescent; D009202:Cardiomyopathies / Q000097:blood / Q000150:complications / Q000000981:diagnostic imaging*; D016022:Case-Control Studies; D004452:Echocardiography; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008297:Male; D008945:Mitral Valve Prolapse / Q000097:blood / Q000150:complications / Q000000981:diagnostic imaging*; D053773:Transforming Growth Factor beta1 / Q000097:blood*; D053781:Transforming Growth Factor beta2 / Q000097:blood*; D055815:Young Adult
chemical_list: C508960:TGFB1 protein, human; C509041:TGFB2 protein, human; D053773:Transforming Growth Factor beta1; D053781:Transforming Growth Factor beta2