As survival of children with CHD needing surgery has improved significantly, the need for follow-up in terms of health-related quality of life has become increasingly important. In this study, we sought to describe health-related quality of life in children with CHD in relation to cardiac surgery. Methods: A retrospective Swedish National Registry for Congenital Heart Disease survey measured using DISABKIDS chronic generic measure-short version included 337 children (age 9-17 years; 39% girls). The majority (n= 319, 95%) of children had a biventricular heart, whereas the remaining had a univentricular heart. Cardiac surgery was performed in 197 (58%) children. Health-related quality of life was expressed as total score (100 highest) and given as medians and 10-90th percentiles. Results: The overall total score was 95 (88-100). Children with a biventricular heart who had undergone three or more surgeries (n= 31; 9%) had the lowest total score of 81 (61-97; p< 0.001). Children with two or more surgeries and those with univentricular heart were classified in NYHA II more frequently than children with one or no cardiac surgery (p= 0.005 and < 0.001, respectively). Children with three or more surgeries and those with univentricular heart needed more help at school (p< 0.001). Compared with children with other chronic diseases, children with CHD had a high total score except for children with three or more surgeries who had comparable total scores with children with other chronic diseases. Conclusion: Children with three or more cardiac surgeries and those with a univentricular heart appear to have lower health-related quality of life, cognitive ability, and NYHA classification.