Health-Related Quality of Life Experienced by Children With Chromosomal Abnormalities and Congenital Heart Defects

Pediatric Cardiology (Impact Factor: 1.31). 03/2013; 35(3). DOI: 10.1007/s00246-013-0820-3


Long-term outcomes are fundamental in advising parents about the potential future of their children with congenital heart disease (CHD). No published reports have described the health-related quality of life (HRQL) experienced by children with chromosomal abnormalities who had surgery in early infancy for CHD. A study was undertaken to assess HRQL among children with chromosomal abnormalities and CHD. The authors hypothesized that these children have a worse HRQL than healthy children or a cohort of children matched for CHD diagnosis. Infants with chromosomal abnormalities undergoing cardiac surgery for CHD at 6 weeks of age or younger at the Stollery Children’s Hospital between July 2000 and June 2005 were included in the study. The HRQL of these infants was assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales completed by their parents at a 4-year follow-up evaluation. The study compared the scores for 16 children with normative data. The children with chromosomal abnormalities and CHD had significantly lower mean total PedsQL (71.3 vs. 87.3; p < 0.0001), Psychosocial Summary (70.3 vs. 86.1; p < 0.0001), and Physical Summary (74.3 vs. 89.2; p = 0.0006) scores. Compared with the matched children, those with chromosomal abnormalities had a significantly lower median total PedsQL (75.0 vs. 84.6; p = 0.03), Physical Summary (79.5 vs. 96.9; p = 0.007), and School Functioning (68.5 vs. 83.0; p = 0.03) scores. A better understanding of the mechanisms and determinants of HRQL in these children has the potential to yield important implications for clinical practice including clarity for treatment decision making as well as determination of targeted supports and services to meet the needs of these children and their families differentially.

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    ABSTRACT: Currently, in the United States, there are approximately 1 in 150 adults living with congenital heart disease (CHD) (Go et al., 2014). Infant and childhood mortality related to CHD decreased by 31% between 1987 and 2005 (Khairy et al., 2010). This survival trend is predicted to increase each year due to advancements in treatment and management of CHD. This significant shift in the epidemiology of CHD requires nurses to take action in preparing children with CHD and their families for their future. The school-age child is the ideal age for nurses to begin teaching the child about their unique needs as well as how to care for themselves in preparation for the future. The school-age child with CHD has specific physical, intellectual, emotional, and developmental needs that must be considered and managed using a multidisciplinary approach. Pediatric nurses must be aware of these needs as they are in a unique position to help the child and their family seamlessly and successfully transition into young adulthood as a happy and healthy CHD survivor.
    MCN The American Journal of Maternal/Child Nursing 10/2014; 40(1). DOI:10.1097/NMC.0000000000000092 · 0.90 Impact Factor