Objective: to investigate the factors that affect the quality of life of children with congenital heart defects. Respondents and methods: The study involved 98 respondents of both sexes. The age range is from 8 to 18 years of age. A questionnaire on sociodemographic data was used, which consisted of questions about age, gender, place of residence, the type of congenital heart defect diagnosed, the forms of treatment to date, and the type of therapy. In addition to the questionnaire on sociodemographic data, a validated questionnaire Pediatric Quality of Life Inventory™ (PedsQL™) Cardiac Module for children from 4 to 18 years of age was used. Results: The overall quality of life is reduced and for men it is 81.6, and for women 80.6 out of a maximum of 100 points.There are no significant differences in the assessment of overall quality of life in relation to the place of residence, gender of respondents, type of congenital defect, and whether the defect is one of the characteristics of a syndrome. The only difference is that children who have been prescribed therapy have a lower rated domain of heart health problems compared to those who do not have been prescribed therapy. Older respondents have more problems with perception of physical appearance, cognitive functions and communication, while younger respondents have more problems with anxiety. A larger number of surgeries so far has been associated with a poorer assessment of the domain of heart health problems. Conclusion: Congenital heart defects impair life quality, especially in children with more severe forms of the disease who, after multiple surgeries, are left with consequences that negatively affect their psychological, emotional and social aspects of life.