Survival in hypoplastic left heart syndrome (HLHS) has greatly improved since the 1980s, with ∼90% of infant survivors now living to 18 years.1  As survival rates increase, optimization of quality of life (QoL) and functional abilities becomes the true measure of success. In this issue of Pediatrics, Goldberg et al2  present a multicenter evaluation of functional ability, behavior, and QoL in children with HLHS as an arm of the Pediatric Heart Network Single Ventricle Reconstruction Trial. Although it is accepted that children with heart disease have behavioral and neurodevelopmental sequelae, the severity of these associations and their impact on daily functioning is unknown.3 

QoL is multifaceted, with at least 3 domains (physical, psychological, and social functioning) affected by disease and treatment. Current outcomes no longer focus on survival but have shifted to an evaluation of the whole person, family and/or caregiver dynamics, and functional trajectory. In adolescents and young adults with moderate to severe congenital heart disease, patient illness perception more strongly correlates with functional or psychological impairment than actual disease severity.4  Assessment tools for these constructs may provide observable, trackable, and modifiable targets for intervention.

Somatization and physical functioning were reported to be worse in patients with HLHS when compared with the normative population.2  In addition to disease severity, could these also have a strong association with subjective perception in children with HLHS? Could caregivers play a role in enhancing somatization and physical dysfunction? Could disruption of typical childhood development have a confounding impact on functioning in addition to the effects of the disease itself?

Parental reporting, and not professional objective assessment, was used as a proxy of child functioning.2  Deviations from typical functioning in this population were identified at 14 months and 3 years and persist at year 6.2,5  Is there perhaps too much reliance on parent-report data to understand child psychological and developmental functioning? There may be noncongruence between caregiver and patient perceptions in children with heart disease.6  Parental stress was also found to be negatively associated with perceptions of their children’s QoL in certain populations.7 

This study indeed identifies behavioral associations in children with HLHS but cannot determine causality. Controllable risk factors, such as the choice of Norwood shunt type, have not been found to alter these associations.

It appears that many children with HLHS do have functional impairment. More research is necessary to determine if other interventions, such as therapies specifically tailored to a child’s particular developmental stage and increased family intervention (eg, education, support groups, and coping skills) for infants with HLHS, regardless of identified early functional impairment, may improve the trajectory of functioning and QoL of these children and their families.8  Other interventions such as physical exercise, early medical recognition of complications, and optimized nutrition may also be beneficial. Would parental interventions be more effective in specific childhood developmental stages? If so, we need to determine the best way to incorporate developmental targets into treatment. Although adverse sociodemographic conditions negatively impact behavior and QoL, we need to identify the most effective way to tailor and disseminate interventions. Perhaps financial and resource support programs should be a priority in any attempt at QoL improvement.

Opinions expressed in these commentaries are those of the authors and not necessarily those of the American Academy of Pediatrics or its Committees.

FUNDING: No external funding.

COMPANION PAPER: A companion to this article can be found online at www.pediatrics.org/cgi/doi/10.1542/peds.2019-1010.

HLHS

hypoplastic left heart syndrome

QoL

quality of life

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Competing Interests

POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.