discussed above The AHA statement outlines the recommended screening tools to evaluate the behavior and development of the child with CHD.3 In the United States, children with developmental or educational disabilities can qualify for school-based support to support their learning needs The educational plans are updated on an annual basis and ensure that children with identified disabilities can receive special instruction or services to support their learning needs.176 Additional school-based interventions are listed in Box 76.5.177 Box 76.5 School-Based Interventions for Children With Congenital Heart Disease ■ Education of teachers, school psychologists, classroom aids, and administrators about the child's medical condition, the risk for developmental delays, and specific learning needs ■ Routine neuropsychologic or psychoeducational evaluations ■ Providing extended time for homework, exams, and projects ■ Providing “note taker” and/or recorded classes ■ Assistance of a reader or scribe for exams ■ Quiet room with decreased distractions for exams ■ Physical, occupational, and speech therapies ■ Applied behavior analysis (ABA) therapy ■ Keyboarding as an option instead of handwriting Health-Related Quality of Life Health-Related Quality of Life Scores in Pediatric Cardiac and Congenital Heart Disease Survivors (See Also Chapter 82) Most early outcome studies in patients with CHD described mortality and morbidity or health status, including anatomic or hemodynamic outcome, electrophysiologic sequelae, and/or exercise capacity following surgical interventions or included sociocultural parameters such as marital status, number of offspring, employment status, or educational attainment in adults with CHD.178 More recent studies have recognized the multidimensional nature of HRQOL and have included not only physical health status and physical functioning but also psychologic status and social functioning However, there are relatively few studies that have evaluated the patient's self-perceptions of QOL Self-reported QOL related to physical health, psychosocial health, social functioning, and school functioning for children with CHD is reduced compared to healthy children.179–183 Mussatto and colleagues found that the greatest negative impact on QOL was reported in the areas of social and educational functioning, despite the perception that CHD primarily has physical effects.181 In a large, single-center study of QOL in children with heart disease, Uzark and colleagues evaluated both parent-proxy and self-reported perceptions utilizing the PedsQL 4.0 Core scales.182 As perceived by parents, worse physical and psychosocial QOL was related to the severity of heart disease While most children with heart disease reported good overall QOL, 20% of the children with heart disease reported significantly impaired psychosocial QOL, including children with mild or repaired heart disease The largest multicenter study assessing QOL in the United States and United Kingdom utilizing a reliable, valid, and generalizable disease-specific QOL measure including both child and adolescent self-report and parent/guardian proxy-report was performed by Marino et al in the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study.179,184–188 In this study, 2420 patient– parent pairs (4840 total respondents) participated from 10 geographically diverse centers in the United States and the United Kingdom Patients with both CHD (68% of the cohort) and acquired HD (32% of the cohort) were included This study demonstrated that lower patient and parent-reported QOL scores were associated with higher disease severity and greater medical care utilization (number of cardiac surgeries, cardiac-related hospital admissions, and doctor visits in the last 12 months), poorer patient self-perception and competency, and increased behavioral and emotional problems in the pediatric heart disease population.179,187 PCQLI scores (Total, Disease Impact, and Psychosocial Impact) differed significantly among disease severity subgroups (mild CHD, biventricular CHD status postsurgical repair or palliation, and single ventricle CHD status post Fontan completion) across all age categories and respondent types, and are consistent with widespread clinical observations that increased disease severity is associated with a lower QOL Worse PCQLI Total score was significantly correlated with lower Global Self-Worth score (self-perception) on the Self Perception Profile for Children and Adolescents for both age groups A statistically significant positive correlation was noted between the PCQLI Total score and the Achenbach Youth Self-Report and Child Behavior Checklist Total Competency score (competence in physical, social, and school activities) In addition, there was a statistically significant inverse correlation between PCQLI Total score and both the Achenbach Internalizing Problems Summary Scale score (anxiety, depression/withdrawal, and somatic complaints) and DSM-IV Oriented Scale scores (Affective Disorder, Anxiety Disorder, Somatic Disorder, Attention Deficit Hyperactivity Disorder) for all groups.179 Variation in Health-Related Quality of Life Scores in Congenital Heart Disease Surgical Survivors Interestingly, in the PCQLI study there was significant variation noted in the specific diagnosis and procedural groups for acyanotic two-ventricle (e.g., aortic stenosis), cyanotic two-ventricle (e.g., TOF) and the palliated single ventricle Fontan populations.179 While each specific population group segregated into a particular QOL score range (aortic stenosis, 80s; TOF, 70s; and Fontan, 60s) based on the underlying disease severity and the medical, catheter-based, and surgical therapy required, there were complex single ventricle Fontan patients that had QOL scores as high as aortic stenosis patients who had not undergone intervention, and aortic stenosis patients who had undergone intervention who had QOL scores that were worse than the typical Fontan These data suggest that there are resilience and depressant factors that increase or decrease each ...Health-Related Quality of Life Health-Related Quality of Life Scores in Pediatric Cardiac and Congenital Heart Disease Survivors (See Also Chapter 82) Most early outcome studies in patients with CHD described mortality and... measure including both child and adolescent self-report and parent/guardian proxy-report was performed by Marino et al in the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study.179,184–188 In this study, 2420 patient–... visits in the last 12 months), poorer patient self-perception and competency, and increased behavioral and emotional problems in the pediatric heart disease population.179,187 PCQLI scores (Total, Disease Impact, and Psychosocial Impact) differed