SVRII Family Factors Study

Survival for one of the most complex forms of congenital heart disease (CHD), hypoplastic
left heart syndrome (HLHS), has improved dramatically; however, survivors are known to be at
risk for both physical and psychosocial morbidity. In congenital heart disease, this
psychosocial morbidity has been manifested as delays in cognitive and social development,
behavior problems, reduced quality of life, as well as parenting stress, anxiety, and
negative impact on the family. We have a relatively limited understanding of the causes of
psychosocial morbidity and what factors may impact risk or protection for the child and
family.

The environment in which a child spends the majority of their time exerts a significant
impact on development. For infants and young children this "environment" is most commonly the
immediate family. Interactions between illness characteristics and individual family member
perceptions and expectations are an important determinant of the family's response to the
presence of heart disease in a child. There is a critical gap in our understanding of how
family-related factors influence psychosocial outcomes in the CHD population and if there are
opportunities to intervene in order to improve outcomes for both children and families.

The primary aim of this study is to investigate the relationship between family factors
including, parental mental health, parental health-related quality of life (HRQOL), family
resources, and family function, and developmental and psychosocial outcomes in children with
HLHS at 6 years of age. Secondary aims are to explore for differences in outcomes between
different family types and to establish the feasibility of longitudinal investigation of the
trajectory of child and family psychosocial function over time in this well-defined cohort of
children and parents.

Longitudinal assessment of these family factors, in conjunction with neurodevelopmental and
psychosocial evaluation of affected children, will provide improved understanding of how and
when these factors influence child development. This will help to:

- Identify children/families at risk for problems,

- Elucidate the etiology and progression of psychosocial problems in children with
congenital heart disease,

- Describe the relationships between child and family outcomes over time, and guide the
development of family-focused interventions with a goal of preventing problems.

This study will be conducted as an ancillary study to the Single Ventricle Reconstruction
Extension Study (SVR II) conducted by the Pediatric Heart Network (PHN). All PHN study sites
will be eligible to participate in this study. Participation will be determined by the PHN
principal investigator at each site. Parents of children enrolled in SVR II will be eligible
to participate in this ancillary study.

A multivariable, descriptive design using survey techniques will be utilized. Mothers and
fathers of children with HLHS will complete study measures at or near the SVR II 6 year
visit. Correlation, regression, and structural equation modeling will be used to test study
hypotheses.

Results of this study have the potential to better define the family implications of this
disease, the risk factors for poor outcomes and ultimately, to guide development of
interventions to improve child and family psychosocial outcomes.

Inclusion Criteria:

- Speak English or Spanish

- Enrolled in the SVR or SVRII studies

Exclusion Criteria:

- Have not completed the 6 year visit and questionnaires