Abstract

Bicuspid aortic valve is one of the most common congenital heart diseases with low manifestation in childhood and severe consequences in adults that determines the importance in early diagnostics of myocardial changes in this anomaly. According to the literature the polymorphisms in the genes of NFATC family could result both in impaired embriogenetic valves formation and development of postnatal myocardial hypertrophy. The aim of the study was to detect the early changes of intracardial hemodynamics at aortic valve in children with bicuspid aortic valve (BAV) and establish their interrelations to the signs of myocardial hypertrophy in these children. Materials and methods: Dopplerograhphic study of basic intracardiac hemodynamics parameters in 38 children with BAV and in 28 children of control group was conducted. The results were processed statistically by Student’s t-test, correlation analysis and multiple regression. Results: In the result of study the moderate concentric left ventricle myocardial hypertrophy development was detected in 62 % of children with BAV which is accompanying to significant increasing of blood flow velocity and pressure gradient at aortic valve. There were not established significant correlations between the parameters of hemodynamics at valve and left ventricle’s posterior wall depth and septum depth whereas the highest inputs of these values were obtained in the left ventricle systolic dimension and volume and less in the hypertrophic signs. Conclusions: In children with BAV the moderate concentric myocardial hypertrophy with significant changes of intracardial hemodynamics at aortic valve takes place with the highest inputs in left ventricle volumetric values The obtained data serves as a substantiation for the treatment and prevention of it further development.

Highlights

  • Congenital heart defects (CHD) in children has recently attracted the attention of the medical community

  • It is must be emphasized that one of the bicuspid aortic valve (BAV) manifestations is the myocardial hypertrophy with slow progression caused either by morphologic and functional features of this CHD or by independent co-morbid condition related to polymorphisms of NFATC genes that, by turn, leads to formation of valve anomalies within the embryogenesis and postnatal stress-induced myocardial hypertrophy

  • In 24 from 38 patients with BAV the left ventricle posterior wall depth (LVPWd) and ventricu­ lar septum depth was more than 8 mm and their rLVPWd was 0.49 mm ± 0.04 mm compaired to those with LVPWd

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Summary

Introduction

Congenital heart defects (CHD) in children has recently attracted the attention of the medical community. It is must be emphasized that one of the BAV manifestations is the myocardial hypertrophy with slow progression caused either by morphologic and functional features of this CHD or by independent co-morbid condition related to polymorphisms of NFATC genes that, by turn, leads to formation of valve anomalies within the embryogenesis and postnatal stress-induced myocardial hypertrophy. These genes are represented by 5 modifications with an expression in different tissues and participate in hypertrophic response of bones and heart muscles. In some studies the role of NFATC proteins in development of cardiac dysfunction and myocardial hypertrophy was proven [6,7]

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