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Background: As survival rates for infants born with severe forms of cardiac defects (congenital heart defect [CHD]) improve, attention is directed to evaluating factors that affect the child's short- and long-term outcomes including parental quality of life (QOL)., Purpose: The purpose of this review was to identify how parental QOL is affected when having a child with a CHD. Factors that influence parental QOL when having a child with a CHD will also be described., Methods: A systematic search of CINAHL, EMBASE, PsycINFO, and PubMed databases was performed. Thirty-three quantitative cross-sectional or cohort studies were selected for inclusion and analyzed for quality reporting using Strengthening the Reporting of Observational Studies in Epidemiology guidelines., Results: Heart defect severity, age of child, perceived support, and availability of economic resources were identified as factors affecting parental QOL. Parent gender was related to QOL and family functioning factors. Paternal outcomes were reported in 23 of the 33 studies (70%), with an average father participation rate of 40%., Conclusions: Having a child with CHD negatively affects parental QOL. Future research should include targeting fathers to improve understanding of their unique perceptions and needs. Longitudinal studies should also describe correlations of parental QOL with their child's developmental outcomes. Efficacy studies testing supportive interventions on outcomes such as improved adjustment and QOL are needed.