QOL from the beginning
As survival in congenital heart disease (CHD) continues to improve, the importance of functioning beyond periods of critical illness becomes increasingly important. Health-related quality of life (HRQOL) describes multiple facets of daily functioning and perception of an individual's physical or mental health.¹ It is accepted that children with CHD are at increased risk for developmental disorders, learning disabilities, and/or developmental delays.² However, little is known about their neurodevelopmental trajectory and how this relates to quality of life (QOL) throughout their lifespan.

In adolescents and young adults with moderate to severe CHD, illness perception may be more strongly linked to patient functioning than disease severity itself.^3,4 Understanding the reciprocal relationship between behavior and perception is critical. Determining "how" to change maladaptive perceptions and behaviors can be complicated and requires a multidisciplinary approach.

Perception is everything
CHD management begins with a patient, a family, and a medical team. None of these stakeholders operate independently, but function and respond to feedback from the healthcare system. Parents who report low QOL tend to agree with their child's own perception more than those with higher levels of perceived QOL.⁵ Parent-child convergence on perception of child QOL is limited.⁵ Intervention must thus focus on the family system.

Current QOL data
There is a higher prevalence of reported anger, anxiety, distress, depression, hopelessness, and somatic symptoms in parents of children with CHD.^6-9 Parents of children with more severe heart disease, insufficient knowledge regarding their child's diagnosis, and limited family support experienced greater psychological distress.¹⁰ While severity of disease did not correlate with reported HRQOL for children, parent-reported HRQOL scores on physical and psychosocial domains were significantly lower in children with more severe disease.⁴ In families with a mother and father dyad, mothers were at greater risk for high levels of anxiety, somatization, and feelings of hopelessness compared to fathers.^7,9,11

Children with CHD report lower ratings of physical and psychosocial functioning compared to healthy children.⁴ Over 20% of children aged ≥8 years reported significantly impaired psychosocial QOL scores.⁴ Of children with less severe disease, nearly one in five reported significantly impaired psychosocial QOL.⁴ In a sample of CHD patients, compared to parent-proxy ratings of QOL, children's self-rated QOL correlated moderately on psychological and physical functioning.⁵

Can QOL be modified?
Determinants of QOL for children with CHD include: parental support, physical limitations, ability to cope with daily stressors, and anxiety/depression levels.¹² These underscore the importance of psychological and physical factors for well-being.

Psychological adjustment
Externalizing (e.g. behavioral problems) and internalizing (e.g., depression, anxiety) disorders range in prevalence from 15% to 25% in CHD.^13-17 Younger children exhibit less internalizing and externalizing behavioral problems compared to peers, while older children and adolescents exhibit more internalizing behaviors. Externalizing problems occur, though to a lesser degree than controls.¹⁸

Parental modeling of adjustment and perception is critical for children. Medical severity accounted for approximately 3% of variability in child adjustment to disease, but nearly one-third of the variability was accounted for by maternal perception of illness.¹⁹ This suggests that the quality of the parent-child relationship is more pertinent to adjustment than CHD severity.¹⁹

Given its impact on the outcomes of chronic medical conditions, current guidelines recommend monitoring behavior and psychological adjustment throughout the lifespan^2,20 Identified or suspected impairment of psychological adjustment warrants a referral to a mental health specialist.

Physical activity
Avoidance of physical activity, despite medical clearance, may indicate poor adjustment. Medical guidance regarding appropriate physical activity is important, not only for safety, but due to its impact on actual or perceived limitations and HRQOL. Activity restrictions are not static; restrictions may change throughout the disease continuum. Parental perception of tolerance to physical activity may impact QOL. Parental over-protection and disease severity are associated with anxiety in adults with CHD.²¹

In the absence of clear contraindications, it is recommended children with CHD adhere to general physical activity guidelines, including 60 minutes of moderate to vigorous physical activity with less than two hours of sedentary activity per day.^22,23 Physical activity guidelines should be discussed with both caregivers and children/adolescents with CHD, as they may differ in their perception of physical functioning. This in turn impacts social and academic activities.²⁴

Aerobic training in children with CHD influences reported HRQOL.²⁵ While children aged 10-15 years demonstrated improvements in self-reported cognitive functioning and parent-reported social functioning, those aged 16-25 did not.²⁵ Though more research is needed to understand the relationship between exercise and QOL of children with CHD, exercise training appears to improve psychological adjustment in adults. Exercise based cardiac rehabilitation improves HRQOL, decreases cardiovascular mortality, and reduces hospital admissions.²⁶ The 2018 AHA/ACC guideline for the management of the adult with CHD emphasizes the need to address mental health.

Less talk, more action
A focus on family and patient functioning is key in determining the appropriate intervention for a specific family at a specific point in time. Evaluation of perceived QOL, psychological adjustment, and physical activity engagement may help connect patients to clinical resources that may ultimately improve health outcomes. With guidance from the medical team and mental health professionals, small changes in behaviors and family functioning can improve perception of health and mental health itself.

References

1. Measuring healthy days: population assessment of health-related quality of life (CDC website). 2000. Available at: https://www.cdc.gov/hrqol/pdfs/mhd.pdf. Accessed 09/20/2020.
2. Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 2012;126:1143-72.
3. O'Donovan CE, Painter L, Lowe B, Robinson H, Broadbent E. The impact of illness perceptions and disease severity on quality of life in congenital heart disease. Cardiol Young 2016;26:100-9.
4. Uzark K, Jones K, Slusher J, Limbers CA, Burwinkle TM, Varni JW. Quality of life in children with heart disease as perceived by children and parents. Pediatrics 2008;121:e1060-7.
5. Goldbeck L, Melches J. Quality of life in families of children with congenital heart disease. Qual Life Res 2005;14:1915-24.
6. Dale MTG, Solberg Ø, Holmstrøm H, Landolt MA, Eskedal LT, Vollrath ME. Mothers of infants with congenital heart defects: well-being from pregnancy through the child's first six months. Qual Life Res 2012;21:115-2.
7. Lawoko S, Soares JJF. Distress and hopelessness among parents of children with congenital heart disease, parents of children with other diseases, and parents of healthy children. J Psychosom Res 2002;52:193-208.
8. Lawoko S, Soares JJF. Psychosocial morbidity among parents of children with congenital heart disease: a prospective longitudinal study. Heart Lung 2006;35:301-4.
9. Jackson AC, Frydenberg E, Liang RP-T, Higgins RO, Murphy BM. Familial impact and coping with child heart disease: a systematic review. Pediatr Cardiol 2015;36:695-712.
10. Brosig CL, Whitstone BN, Frommelt MA, Frisbee SJ, Leuthner SR. Psychological distress in parents of children with severe congenital heart disease: the impact of prenatal versus postnatal diagnosis. J Perinatol 2007;27:687-92.
11. Doherty N, McCusker CG, Molloy B, et al. Predictors of psychological functioning in mothers and fathers of infants born with severe congenital heart disease. J Reprod Infant Psychol 2009;27:390-400.
12. Apers S, Luyckx K, Moons P. Quality of life in adult congenital heart disease: what do we already know and what do we still need to know? Curr Cardiol Rep 2013;15:407.
13. Bellinger DC, Wypij D, Rivkin MJ, et al. Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure: neuropsychological assessment and structural brain imaging. Circulation 2011;124:1361-9.
14. Hövels-Gürich HH, Seghaye M-C, Schnitker R, et al. Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation. J Thorac Cardiovasc Surg 2002;124:448-58.
15. Hövels-Gürich HH, Konrad K, Skorzenski D, Herpertz-Dahlmann B, Messmer BJ, Seghaye MC. Attentional dysfunction in children after corrective cardiac surgery in infancy. Ann Thorac Surg 2007;83:1425-30.
16. Bellinger DC, Newburger JW, Wypij D, Kuban KCK, duPlesssis AJ, Rappaport LA. Behaviour at eight years in children with surgically corrected transposition: the Boston Circulatory Arrest Trial. Cardiol Young 2009;19:86-97.
17. Ellerbeck KA, Smith ML, Holden EW, et al. Neurodevelopmental outcomes in children surviving d-transposition of the great arteries. J Dev Behav Pediatr 1998;19:335-41.
18. Karsdorp PA, Everaerd W, Kindt M, Mulder BJ. Psychological and cognitive functioning in children and adolescents with congenital heart disease: a meta-analysis. J Pediatr Psychol 2007;32:527-41.
19. DeMaso DR, Campis LK, Wypij D, Bertram S, Lipshitz M, Freed M. The impact of maternal perceptions and medical severity on the adjustment of children with congenital heart disease. J Pediatr Psychol 1991;16:137-49.
20. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019;73:1494-1563.
21. Ong L, Nolan RP, Irvine J, Kovacs AH. Parental overprotection and heart-focused anxiety in adults with congenital heart disease. Int J Behav Med 2011;18:260-7.
22. Strong WB, Malina RM, Blimkie CJR, et al. Evidence based physical activity for school-age youth. J Pediatr 2005;146:732-7.
23. Takken T, Giardini A, Reybrouck T, et al. Recommendations for physical activity, recreation sport, and exercise training in paediatric patients with congenital heart disease: a report from the Exercise, Basic & Translational Research Section of the European Association of Cardiovascular Prevention and Rehabilitation, the European Congenital Heart and Lung Exercise Group, and the Association for European Paediatric Cardiology. Eur J Prev Cardiol 2012;19:1034-65.
24. Lambert LM, Minich LL, Newburger JW, et al. Parent-versus child-reported functional health status after the Fontan procedure. Pediatrics 2009;124:e942-9.
25. Dulfer K, Duppen N, Kuipers IM, et al. Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: a randomized controlled trial. J Adolesc Health 2014;55:65-72.
26. Anderson L, Oldridge N, Thompson DR, et al. Exercise-based cardiac rehabilitation for coronary heart disease: Cochrane systematic review and meta-analysis. J Am Coll Cardiol 2016;67:1-12.

Clinical Topics: Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Congenital Heart Disease, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement

Keywords: Heart Defects, Congenital, Pediatrics, Quality of Life, Self Report, Caregivers, Mental Health, Prevalence, Longevity, Critical Illness, Cardiac Rehabilitation

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