Role of dysplastic features in evaluation of qt interval disorder in patients with small heart anomalies

Abstract

S mallheart anomalies are a heterogeneous group of cardiovascular system development disorder in the form of anatomical abnormalities of heart structure and great vessels which occurred in embryoor ontogenesis [8,10]. Small heart anomalies are usually being interpreted as pathology only in cases of overt forms (arrhythmias, clinically significant valvular regurgitation, etc) and rarely being considered from these positions in relation to young people -even on a stage of occupational guidance or military medical examination. Meanwhile, small heart anomalies are one of the pathologies which caused high-frequency of rhythm disorder (from 50 % up to 90 %) including young age and development of sudden arrhythmic death [11]. This is precisely why timely detection is important in population of small heart anomalies of patients with high risk of cardiovascular complications, first of all, arrhythmia.One of the risk factor of dangerous arrhythmia and sudden cardiac death is long/short QT interval [5,12]. In case of hereditary QT prolongation one can observe specific gene mutations, disorder of genetic control of ion channel proteins with electrophysiological consequences. Data comparison of genetic research with associated phenotypic manifestations of different molecular genetic variations of long and short QT syndrome made it possible to refine their diagnostics on the stage proceeding to genotyping. In recent years there is an active search for new mutations takes place and diagnostics of so called «silent» pathology forms which are being manifested under certain conditions is still actual [9]. In addition to this, there is lack of information in literature about frequency and clinical significance of changes

Cardiac ventricular false tendons: A meta‐analysis

“…Miao et al (1987) performed an echocardiographic study revealing FTs in 30 of 251 children aged 2–16 years. Yagoda et al (2014) found 94 patients with echocardiographic ventricular false tendons of 128 patients studied for small heart anomalies.…”

Guidelines of the Russian scientific medical society of internal medicine on the diagnosis, treatment and rehabilitation of patients with the connective tissue dysplasia (first edition)

“…Патологический QT ассоциирован с марфаноподобным фенотипом, доброкачественной гипермобильностью суставов и с миксоматозной дегенерацией ПМК. В этой группе лиц следует исключать приём препаратов, удлиняю-щих QT-интервал, или контролировать приём, так как даже однократное их применение в случаях исходно нормального QT увеличивало его продолжительность, причём почти в половине случаев -на «критическую» величину, превышающую 60 мс [204,205,206].…”

Mitral Valve Prolapse in Pregnancy: Modern Concept

“…With the exclusion of MVP syndrome, prolapse of the valves without their thickening and significant mitral regurgitation can be regarded as one of the variants of a small heart anomaly, the number of which, as is well known, closely correlates with the number of external signs of dysembryogenesis detected [24].…”