Background: Congenital heart defects (CHD) is one of the major congenital irregularities among children, representing as a major worldwide wellbeing issue. Aim:this study aimed toexamine a guiding program for mothers to improve the quality of life of their children with congenital heart disease. Design: A quasi-experimental design was used. Setting: The study was carried out at the Cardiology Department and Outpatient Clinic of Mansoura University Children Hospital (MUCH). Subjects: A convenient sample of 70 children with congenital heart defects and their accompanying mothers at MUCH, their age ranged from 3 to 12 years, from both sex and confirmed the diagnosis as congenital heart disease, either cyanotic or a cyanotic. Tools: Data were collected using, an interview questionnaire to collect socio-demographic data, for both mothers and their children with CHD. Assessment of mothers' knowledge and practice about congenital heart defects, and quality of life scale for studied children (QOL scale).Results: high statistical significant were found between knowledge score and practice post immediate intervention as well as statistically significant differences between immediately post-program knowledge score and immediately post- program QOL score. Conclusion: The study concluded that mothers' knowledge, reported practice, and quality of life of their children with congenital heart disease were improved after the implementation of the guiding program either immediately or 3 months later than before its implementation. Recommendation: Organizing training programs for children with CHD in their homes about their public life such as nutrition, sports, and protection from infection.