Cardiomyopathy in young adults with classic mitral valve prolapse.

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-ß 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-ß1 and ß2 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-ß1 and ß2 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-ß signalling.

Preoperative left ventricular function in degenerative mitral valve disease.

AIM: The aim of the study is to determine the impact of the underlying etiology (Barlow's disease or fibroelastic deficiency) on left ventricular function in patients with degenerative mitral valve disease and severe mitral regurgitation. METHODS: We studied 233 patients (mean age: 53.8 ±â€Š12.9) undergoing surgery for severe mitral regurgitation due to degenerative mitral valve disease at Almazov Federal Heart Centre between 2009 and 2011. Pathologic diagnoses for valvular tissue specimens were provided by an experienced pathologist. Preoperative strain and strain rate were determined using speckle tracking (Vivid 7 Dimension, EchoPAC'08). RESULTS: Barlow's disease was identified by the pathologist in 60 patients (25.8%), and fibroelastic deficiency in 173 patients (74.2%). There were no significant differences between groups in preoperative mitral regurgitation volume (70.5 ±â€Š9.6 vs. 71.6 ±â€Š8.5 ml, P = 0.40), and in global systolic (ejection fraction: 52.7 ±â€Š6.6 vs. 52.0 ±â€Š7.4%, P = 0.53) and diastolic (E/e': 12.2 ±â€Š3.9 vs. 12.8 ±â€Š4.2, P = 0.35) left ventricular function. Despite the lack of difference in ejection fraction and diastolic tissue Doppler parameters, in patients with Barlow's disease in comparison with fibroelastic deficiency a significant decrease of the left ventricular longitudinal systolic strain (-13.5 ± 2.2 vs. -15.6 ± 2.3%, P = 0.00001) and early diastolic strain rate (1.04 ± 0.20 vs. 1.14 ± 0.18 s, P = 0.0004) were detected. CONCLUSION: Patients with severe mitral regurgitation due to Barlow's disease have a lower preoperative left ventricular systolic function than those with fibroelastic deficiency, which may affect their postoperative prognosis.

Evaluation of left ventricular systolic function in young adults with mitral valve prolapse.

OBJECTIVE: To evaluate left ventricular function in young adults with mitral valve prolapse (MVP) without significant mitral regurgitation using two-dimensional strain imaging. METHODS AND RESULTS: A total of 58 asymptomatic young subjects (mean [± SD] age 19.7±1.6 years; 72% male) with MVP were compared with 60 sex- and age-matched healthy subjects. MVP was diagnosed by billowing one or both mitral leaflets >2 mm above the mitral annulus in the long-axis parasternal view. Longitudinal, radial and circumferential strain and strain rate were determined using speckle tracking with a grey-scale frame rate of 50 fps to 85 fps. There were no significant differences in the global systolic left ventricular function of the subjects with MVP compared with the control group. In the MVP group, most of the global myocardial systolic deformation indexes were not reduced. Only the global circumferential strain showed a decrease in the prolapse subjects. Regional, longitudinal, circumferential and radial strain and strain rate were decreased only in septal segments. A decrease in the rotation of the same septal segments at the basal level was also observed. CONCLUSION: Regional septal myocardial deformation indexes decrease in subjects with MVP. These changes may be the first sign indicating the deterioration of left ventricular systolic function as well as the existence of primary cardiomyopathy in asymptomatic young subjects with MVP.