Less Talk, More Action: Determinants of Quality of Life in Congenital Heart Disease

Quick Takes

  • Illness perception, not necessarily severity, is linked to patient functioning in adolescents/young adults with moderate to severe CHD.
  • Up to 25% of CHD patients experience anxiety, depression, or behavioral challenges.
  • Aerobic training is linked to improved psychosocial functioning in children and adolescents with CHD.
  • Modifiable factors that may ease parental psychological distress include education regarding their child's diagnosis and family support. Mothers are more vulnerable to psycho-emotional challenges.

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 lifespan2,20 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.


  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.
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Clinical Topics: 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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