A Preliminary Study of One Year Safety and Tolerability of Attention-Deficit/Hyperactivity Disorder Medications in Youth with Fontan Palliation or Heart Transplant.

There are no published studies that examine the safety and tolerability of medication to treat attention-deficit/hyperactivity disorder (ADHD) in children with histories of Fontan palliation (Fontan) or heart transplant (HT), despite the high prevalence of ADHD in these populations. To address this gap, we examined the cardiac course, somatic growth, and incidence of side effects for one year after medication initiation amongst children with Fontan or HT and comorbid ADHD. The final sample comprised 24 children with Fontan (12 medication-treated, 12 control) and 20 children with HT (10 medication-treated, 10 control). Demographic, somatic growth (height and weight percentile-for age), and cardiac data (blood pressure, heart rate, results of 24 h Holter monitoring, electrocardiograms) were extracted from electronic medical records. Medication-treated and control subjects were matched by cardiac diagnosis (Fontan or HT), age, and sex. Nonparametric statistical tests were utilized to compare between- and within-group differences prior to, and one year post, medication initiation. There were no differences in somatic growth or cardiac data when comparing medication-treated participants to matched controls, regardless of cardiac diagnosis. Within the medication group, a statistically significant increase in blood pressure was observed, though the group average remained within clinically acceptable limits. While results are preliminary in nature due to our very limited sample size, our findings suggest that ADHD medications can be tolerated with minimal cardiac or somatic growth effects amongst complex cardiac patients. Our preliminary results favor treating ADHD with medication, which has considerable implications for long-term academic/employment outcomes and quality of life for this population. Close collaboration between pediatricians, psychologists, and cardiologists is essential to individualizing and optimizing interventions and outcomes for children with Fontan or HT.

Specific patterns of executive functioning weaknesses among children after heart transplant.

BACKGROUND: Children with a history of heart transplant (HT) are at risk of executive functioning weaknesses secondary to heart disease and associated morbidity. However, specific executive functioning weaknesses have not been identified. METHOD: The present study, anchored in Anderson's (2002) Developmental Model of Executive Functioning, provides a detailed, retrospective analysis of executive functioning in the areas of goal setting, cognitive flexibility, attentional control, and information processing for a clinically referred sample of 53 pediatric HT recipients who underwent neuropsychological evaluations as part of typical clinical care. RESULTS: Broadly, the sample demonstrated mild-to-moderate deficits across cognitive, adaptive behavior, executive functioning, and academic domains, as well as elevated parent-reported concerns for depression and anxiety. Executive functioning weaknesses, while global, persisted after controlling for the effects of depression and anxiety and were most prominent in cognitive flexibility. In addition, poor cognitive flexibility predicted lower adaptive behavior, IQ, and academic outcomes among this population, placing them at considerable risk of extensive impairment in several domains of their lives. CONCLUSIONS: Taken together, children with a history of HT demonstrated broad difficulties across several areas of functioning, with particular concerns for working memory. As such, interventions and accommodations specifically targeting working memory may help provide the most optimal outcomes for this population.

Implications of attention and executive functioning weaknesses in youth with Fontan circulation.

Youth with Fontan circulation (Fontan) are at-risk for impairments in attention and executive functioning (EF) due to a confluence of genetic, prenatal, surgical, and medical risk factors. We sought to describe attention and EF in this population, measured via standardized performance-based tests and caregiver rating scales. We then examined how weaknesses in attention and EF were related to outcomes in other neurobehavioral domains, including adaptive behavior and academic achievement. Our sample included 93 youth with Fontan who were referred for neuropsychological evaluations as part of standard clinical care. The cohort as a whole measured between 0.18 to 0.99 standard deviations below normative means across domains of attention, EF, academic achievement, and intellectual ability. In addition, caregiver-reported concerns for attention, EF, anxiety, and depression were elevated, and approximately 0.35 to 0.85 standard deviations above normative means. Lastly, caregiver-reported adaptive behavior measured 0.93 to 1.24 standard deviations below normative values. Academic outcomes were differentially affected by demographic and attention/EF variables, while depression and caregiver-reported EF predicted adaptive behavior. Findings from this study underscore the importance of routine neuropsychological evaluation as part of comprehensive, multidisciplinary care for individuals with Fontan, with the goal of enhancing neurobehavioral and functional outcomes across the lifespan.