Assessing Health-Related Quality of Life in Congenital Heart Disease

Marino BS, Cassedy A, Drotar D, Wray J.
The Impact of Neurodevelopmental and Psychosocial Outcomes on Health-Related Quality of Life in Survivors of Congenital Heart Disease. J Pediatr 2016;174:11-22.e2.

The following are 10 points to remember about the impact of neurodevelopmental and psychosocial outcomes on health-related quality of life (HRQOL) in survivors of congenital heart disease (CHD):

  1. QOL is the ability to function in daily life situations from a physical, psychological, and social perspective, and derive personal satisfaction from doing so.
  2. Although neonatal mortality is <10%, and the approximate 20-year survival is about 80% after therapy for complex CHD, these patients suffer morbidity that decreases their QOL.
  3. Measuring QOL is essential in the evaluation of long-term outcomes and assessing the efficacy of medical therapies and health care policies.
  4. HRQOL assessment is difficult due to the wide spectrum of age, diagnoses, therapies, results, and other factors such as race and socioeconomic status. The ideal strategy is to utilize instruments that are both generic and disease-specific, with patient-self and guardian-proxy reporting, validated to be applicable to a wide age range and easily self-administered. The ideal tool must be “responsive” (i.e., sensitive to change after intervention or over time), diagnostic, evaluative, and prognostic. Several generic and disease-specific questionnaires are currently available.
  5. Lower self- and proxy-reported HRQOL is associated with increased disease severity, greater medical utilization, increased behavioral and academic problems, and lower self-esteem. However, there were unidentified factors that increase or decrease each individual’s QOL over time. Surgical variables such as greater number of surgeries, the utilization of deep hypothermic circulatory arrest, low cardiac output syndrome, need for extracorporeal membrane oxygenator (ECMO) use, and cardiopulmonary resuscitation were associated with impaired psychosocial functioning.
  6. In children with CHD, neurodevelopmental concerns may begin in infancy, but become more magnified over time, resulting in academic difficulties and inattention, deficient perception, communication, gross and fine motor skills, and executive functioning. Many require special education; tutoring; and physical, occupational, and speech therapy.
  7. The American Heart Association issued a scientific statement ( on neurodevelopmental outcomes, risk stratification, and recommendations for children with CHD, endorsed by the American Academy of Pediatrics. This paper outlines which patients are at high risk for neurodevelopmental abnormalities and makes recommendations regarding screening, surveillance, and intervention.
  8. Neurodevelopmental and psychosocial morbidity may limit adulthood education achievement, earning capacity, and insurability.
  9. Identifying risk factors for low QOL may help develop strategies that prevent its development or guide treatment for those found to have it.
  10. Ideally, HRQOL evaluation would be a routine part of clinical care, involving simple data collection, with interpretable scores stored in a database, and findings communicated to care providers, with the goal of improving patient outcomes and ensuring effective resource utilization.

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