Differences in Quality of Life in Children Across the Spectrum of Congenital Heart Disease.

OBJECTIVE: To create complexity groups based upon a patient's cardiac medical history and to test for group differences in health-related quality of life (HRQOL). METHODS: Patients 8-18 years with congenital heart disease (CHD) and parent-proxies from the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study were included. Outcome variables included PCQLI Total, Disease Impact, and Psychosocial Impact scores. Using a patient's medical history (cardiac, neurologic, psychological, and cognitive diagnosis), latent class analysis (LCA) was used to create CHD complexity groups. Covariates included demographics and burden of illness (number of: school weeks missed, physician visits in the past year, and daily medications). Generalized estimation equations tested for differences in burden of illness and patient and parent-proxy PCQLI scores. RESULTS: Using 1482 CHD patients (60% male; 84% white; age 12.3 ± 3.0 years), latent class analysis (LCA) estimates showed 4 distinct CHD complexity groups (Mild, Moderate 1, Moderate 2, and Severe). Increasing CHD complexity was associated with increased risk of learning disorders, seizures, mental health problems, and history of stroke. Greater CHD complexity was associated with greater burden of illness (P < .01) and lower patient- and parent-reported PCQLI scores (P < .001). CONCLUSIONS: LCA identified 4 congenital heart disease (CHD) complexity groupings. Increasing CHD complexity was associated with higher burden of illness and worse patient- and parent-reported HRQOL.

Behavioral and Emotional Outcomes in Children with Congenital Heart Disease: Effects of Disease Severity, Family Life Stress, Disease-Related Chronic Stress, and Psychosocial Adaptation.

OBJECTIVE: To evaluate whether effects of congenital heart disease (CHD) severity and family life stress on behavioral and emotional functioning are mediated by disease-related chronic stress and psychosocial adaptation. STUDY DESIGN: A cross-sectional analysis of the Pediatric Cardiac Quality of Life Inventory Testing Study was performed. Relationships between CHD severity (comprising 3 groups: mild heart disease, moderate biventricular disease, and single ventricle) and family life stress, on patient- and parent disease-related chronic stress, psychosocial adaptation, and behavioral-emotional outcomes were assessed using structural equation modeling. Patient and parent models were reported separately. RESULTS: There were 981 patient-parent dyads: 22% had mild heart disease, 63% biventricular, and 15% single ventricle; 19% of families reported moderate to major family life stress. Path models revealed that CHD severity and family life stress were mediated by disease-related chronic stress and psychosocial adaptation factors (R2 = 0.18-0.24 for patient outcomes and R2 = 0.33-0.34 for parent outcomes, P < .001, respectively). CONCLUSIONS: The effects of greater CHD severity and family life stress on behavioral-emotional outcomes were mediated by worse disease-related chronic stress and psychosocial adaptation factors. Both disease-related chronic stress and psychosocial adaptation factors may be targets for interventions to improve behavioral and emotional outcomes.

Hearing Loss after Cardiac Surgery in Infancy: An Unintended Consequence of Life-Saving Care.

OBJECTIVES: To investigate the prevalence of hearing loss after cardiac surgery in infancy, patient and operative factors associated with hearing loss, and the relationship of hearing loss to neurodevelopmental outcomes. STUDY DESIGN: Audiologic and neurodevelopmental evaluations were conducted on 348 children who underwent repair of congenital heart disease at the Children's Hospital of Philadelphia as part of a prospective study evaluating neurodevelopmental outcomes at 4 years of age. A prevalence estimate was calculated based on presence and type of hearing loss. Potential risk factors and the impact of hearing loss on neurodevelopmental outcomes were evaluated. RESULTS: The prevalence of hearing loss was 21.6% (95% CI, 17.2-25.9). The prevalence of conductive hearing loss, sensorineural hearing loss, and indeterminate hearing loss were 12.4% (95% CI, 8.8-16.0), 6.9% (95% CI, 4.1-9.7), and 2.3% (95% CI, 0.6-4.0), respectively. Only 18 of 348 subjects (5.2%) had screened positive for hearing loss before this study and 10 used a hearing aid. After adjusting for patient and operative covariates, younger gestational age, longer postoperative duration of stay, and a confirmed genetic anomaly were associated with hearing loss (all P < .01). The presence of hearing loss was associated with worse language, cognition and attention (P <.01). CONCLUSIONS: These findings suggest that the prevalence of hearing loss in preschool children after heart surgery in infancy may be 20-fold higher than in the 1% prevalence seen in the general population. Younger gestational age, presence of a genetic anomaly, and longer postoperative duration of stay were associated with hearing loss. Hearing loss was associated with worse neurodevelopmental outcomes.

Neurodevelopmental Outcomes in Children With Congenital Heart Disease-What Can We Impact?

OBJECTIVES: The objectives of this review are to discuss the scope of neurologic injuries in newborns with congenital heart disease, the mechanisms of injury, including prenatal, pre-, intra-, and postoperative factors, neurodevelopmental outcomes, and therapeutic strategies for the timely intervention and prevention of neurologic injury. DATA SOURCE: MEDLINE and PubMed. CONCLUSION: At the current time, important research is underway to 1) better understand the developing brain in the fetus with complex congenital heart disease, 2) to identify modifiable risk factors in the operating room and ICU to maximize long-term neurodevelopmental outcomes, and 3) develop strategies to improve family psychosocial health, childhood development, and health-related quality of life following hospital discharge. Crucial in this effort is the identification of an early postoperative surrogate variable with good predictive validity for long-term outcomes. If an appropriate surrogate variable for long-term outcomes can be identified, and measured relatively early after surgical intervention for complex congenital heart disease, reliable clinical trials can be undertaken to improve upon current outcomes.

Prediction by clinicians of quality of life for children and adolescents with cardiac disease.

OBJECTIVE: To determine whether clinicians could reliably predict health-related quality of life (HRQOL) for children with cardiac disease, the level of agreement in predicted HRQOL scores between clinician sub-types, and agreement between clinician-predicted HRQOL scores and patient and parent-proxy reported HRQOL scores. STUDY DESIGN: In this multicenter, cross-sectional study, a random sample of clinical summaries of children with cardiac disease and related patient and parent-proxy reported HRQOL scores were extracted from the Pediatric Cardiac Quality of Life Inventory data registry. We asked clinicians to review each clinical summary and predict HRQOL. RESULTS: Experienced pediatric cardiac clinicians (n = 140), including intensive care physicians, outpatient cardiologists, and intensive care, outpatient, and advanced practice nurses, each predicted HRQOL for the same 21 pediatric cardiac patients. Reliability within clinician subspecialty groups for predicting HRQOL was poor (intraclass correlation coefficients, 0.34-0.38). Agreement between clinician groups was low (Pearson correlation coefficients, 0.10-0.29). When comparing the average clinician predicted HRQOL scores to those reported by patients and parent-proxies by Bland Altman plots, little systematic bias was present, but substantial variability existed. Proportional bias was found, in that clinicians tended to overestimate HRQOL for those patients and parent-proxies who reported lower HRQOL, and underestimate HRQOL for those reporting higher HRQOL. CONCLUSIONS: Clinicians perform poorly when asked to predict HRQOL for children with cardiac disease. Clinicians should be cognizant of these data when providing counseling. Incorporating reported HRQOL into clinical assessment may help guide individualized treatment decision-making.

Health-related quality of life outcomes in children and adolescents with congenital heart disease.

OBJECTIVES: To compare health-related quality of life (HRQOL) in a group of pediatric patients with congenital heart disease (CHD) and healthy controls and patients with other chronic diseases, and to compare HRQOL among patients with CHD of various severity categories with one another, with controls, and with patients with other chronic diseases. STUDY DESIGN: In this cross-sectional survey, t tests were used to compare patient and proxy-reported Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL) scores (including total, physical health, and psychosocial health summary scores) in children (aged 8-12 years) and adolescents (aged 13-18 years) between controls and (1) a composite CHD population; and (2) patients in each of 3 CHD severity categories: mild (no intervention), biventricle (BV; postintervention), and single ventricle (SV; postpalliation). PedsQL scores among CHD severity categories were compared by ANOVA. PedsQL scores were also compared in the CHD population and children with other chronic diseases without age stratification using t tests. RESULTS: There were 1138 (children, n = 625; adolescents, n = 513) and 771 (children, n = 528; adolescents, n = 243) patient and/or proxy reporters in the CHD and healthy control groups, respectively. Total, physical health, and psychosocial health summary scores were lower in the composite CHD, BV, and SV groups compared with controls (P < .0001). There were significant differences among disease severity categories for all scores (P < .01). The composite CHD, BV, and SV groups had similar PedsQL scores as end-stage renal disease, asthma, and obesity populations. CONCLUSION: Children and adolescents with BV and SV CHD have significantly lower HRQOL than healthy controls and similar HRQOL as patients with other chronic pediatric diseases. Interventions targeting both physical and psychosocial domains are needed to improve HRQOL in this high-risk population.

Length of stay after infant heart surgery is related to cognitive outcome at age 8 years.

OBJECTIVE: To test the hypothesis that postoperative length of stay (LOS) after infant heart surgery might be an easily measured surrogate marker for various events that culminate in later adverse cognitive outcome. METHODS: Among 160 eligible patients with D-transposition of the great arteries undergoing reparative surgery in infancy, 155 (97%) were reevaluated at age 8 years with IQ and achievement testing. We explored whether LOS quartiles were associated with these outcomes when adjusting for perioperative and sociodemographic variables. RESULTS: Longer cardiac intensive care unit (CICU) LOS quartiles were associated at age 8 years with lower full-scale IQ (P=.02), lower verbal IQ (P=.02), and with tendencies toward lower performance IQ (P=.08) and math achievement (P=.08) in adjusted models. Compared with patients in the first quartile of CICU LOS, those in the fourth quartile had mean scores for full-scale IQ that were lower by 7.2 points (P=.01); verbal IQ, 7.3 points (P=.02); performance IQ, 5.8 points (P=.05); and math achievement, 6.0 points (P=.07). Analyses on hospital LOS quartile were similar. CONCLUSIONS: Longer postoperative LOS is associated with worse later cognitive function, even when adjusted for perioperative events, perfusion times, and sociodemographic variables. Further research is necessary to determine the mechanisms underlying this relation.