[Relationship between heart development and pathogenesis of congenital heart defects in current literature]

Abstract

This work presents current ideas on embryonic heart development and is an attempt to elucidate pathogenesis of congenital heart defects. The heart develops from 2 areas, the so-called "heart fields", which differ in the expression of various genes and in sensitivity to certain substances (for example retinoid acid). Heart fields are the source of cardiomyocytes and endocardial endothelial cells for the developing heart tube. Neural crest cells attaining the heart take part in the formation of tunica media of great vessels and the aorto-pulmonary septum. An interaction between the cells of the secondary heart field and neural crest cells migrating to the heart plays a significant role in heart development. This interaction is accomplished by various mediators released into the cell environment (e.g. activation of Notch, which induces secretion of growth factors such as Fgf8). Disturbances in signaling between these cellular populations lead to malformations of the cardiac outflow tract. The sinus venosus is the source of endothelial cells of the coronary vasculature, whereas the proepicardium provides cells for the epicardium, smooth muscle cells and fibroblasts.