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Congenital heart diseases are the second leading cause of death in infancy and childhood, as well as the only cause of heart disease in the pediatric population in the developing countries. Aim : The aim of the present study was to review the literature about factors that affect the quality of life in children with congenital heart disease. The method οf this study included bibliography research from both the review and the research literature which referred to the factors that affect the quality of life in children with congenital heart disease. Results: Although assessment of the children’s quality of life began recently and more specifically during the 1980’s, nowadays it is widely used in the daily clinical practice as a means for comparison of treatment options and evaluation of their effectiveness. In regard to factors affecting the quality of life in children with congenital heart disease, research studies have showed that the change in body image including the delay in physical growth exerts a negative influence on their quality of life. Furthermore, other factors that affect negatively their quality of life are the lack of social acceptance, the limitations imposed by the physical impairment, as well as the anxiety and depression that children usually experience. Other important factors that play a significant role not only on their quality of life but also on the diagnosis and the outcome of the disease are the socio-economic and educational status of the parents. In the literature is cited that quality of life in children with congenital heart diseases should be assessed according to age, growth level, severity and acceptability of the disease, as well as personality features. Conclusions : Assessment of the quality of life by health professionals should be an integral part of the patients’ treatment since it allows the identification and evaluation of personal considerations, priorities, or problems depending on the physical growth stage in regard to the severity of the disease.