Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement

ABSTRACT

Among the most significant factors affecting quality of life in individuals with critical congenital heart disease (CCHD) are neurodevelopmental challenges, including deficits in visuospatial processing and academic achievement. Few studies have compared outcomes across CCHD subgroups, despite their significant differences in anatomy/physiology and medical/surgical courses. This study compared visuospatial processing abilities using the Developmental Scoring System for the Rey–Osterrieth Complex Figure (DSS-ROCF) across groups of adolescents with CCHD (d-transposition of the great arteries [TGA, n = 139], Tetralogy of Fallot [TOF, n = 68], single-ventricle cardiac anatomy requiring the Fontan operation [SVF, n = 145]) and a group of healthy controls (CTR, n = 111), and examined the validity of visuospatial processing in predicting concurrent academic outcomes. The CCHD subgroups were found to differ in Organization, ps < .001, Structural Accuracy, ps < .001, and Incidental Elements Accuracy scores, ps ≤ .008; the post hoc analyses show that the SVF group tended to underperform compared to the other CCHD groups. With respect to academic skills, all CCHD groups scored lower than the CTR group, ps ≤ .007; however, the CCHD groups were not different from each other, ps > .23. The regression results showed that the DSS-ROCF Style rating (reflecting integration) accounted for a small yet statistically significant portion of unique variance in “assembled” academic outcomes, over and above the variance already accounted for by DSS-ROCF Organization, p < .01. These findings support the need for comprehensive neuropsychological assessment and monitoring of children and adolescents with CCHD, as well as targeted intervention for organization and integration deficits that may increase their risk for academic underachievement.

KEYWORDS:

• Rey–Osterrieth Complex Figure
• Visuospatial
• Cardiac
• Congenital heart defect
• Adolescence

Acknowledgements

The authors thank the adolescents and parents who participated in this study. The authors also wish to thank Madelyn Labella and George Sasha Taylor for their assistance with scoring, Jane Holmes Bernstein, Ph.D. for graciously donating her time and expertise with particularly challenging DSS-ROCF scoring questions, and Christian Stopp, M.S., for his assistance with data management.

Disclosure statement

No potential conflict of interest was reported by the authors.

Notes

1. There are many subtypes of CCHD which affect the structure of the heart and/or the surrounding blood vessels, as well as the functional integrity of the cardiovascular system. The three subtypes of congenital heart defects included in this study are d-transposition of the great arteries (TGA), Tetralogy of Fallot (TOF), and single-ventricle cardiac anatomy requiring the Fontan operation (SVF). All three are considered cyanotic conditions, meaning that the blood being circulated throughout the body is oxygen-deficient; all are considered CCHD, meaning that children are severely ill during the neonatal period and require surgical correction for survival (Hoffman & Kaplan, 2002; Miatton, De Wolf, Francois, Thiery, & Vingerhoets, 2006). For children born with TGA, the two main arteries carrying blood from the heart to the body are transposed; therefore, oxygenated blood continues to circulate to and from the lungs, while deoxygenated blood continues to circulate throughout the rest of the body (including the brain). Prior to surgery, survival is dependent on leakages (that allow some oxygenated blood to seep into the body through chambers in the heart) or catheterization until full surgical correction is possible, thus putting children in a state of suboptimal oxygenation until surgical correction is performed (arterial switch operation; American Heart Association, 2015). Children born with TOF have four co-occurring cardiac malformations: (1) ventricular septal defect, (2) pulmonary stenosis, (3) an overriding aorta, and (4) right ventricular hypertrophy. TOF is commonly associated with genetic conditions (e.g., 22q11.2 deletion syndrome) but can occur in isolation or in combination with other heart defects. TOF lesions result in some deoxygenated blood being pumped back into the body, thus reducing the overall concentration of oxygenated blood. Children with this condition require a single surgical correction or several staged procedures, depending on the severity of the cardiac lesions (American Heart Association, 2015). Children born with single-ventricle lesions have a rare disorder in which one of the lower chambers of the heart is malfunctioning or absent. Compared to the other children with CCHD, these children are considered to be among those at the highest risk, and they sustain a prolonged period of suboptimal cerebral oxygenation while completing staged surgical palliation (Fenton et al., 2007).

Additional information

Funding

This work was supported by the National Heart, Lung, and Blood Institute [grant numbers HL77681, HL74734, and HL096825]; the Farb Family Fund; and the National Center for Research Resources [grant number RR02172].