David R. DeMaso

Adolescents With d-Transposition of the Great Arteries Corrected With the Arterial Switch Procedure Neuropsychological Assessment and Structural Brain Imaging

Background— We report neuropsychological and structural brain imaging assessments in children 16 years of age with d-transposition of the great arteries who underwent the arterial switch operation as infants. Children were randomly assigned to a vital organ support method, deep hypothermia with either total circulatory arrest or continuous low-flow cardiopulmonary bypass. Methods and Results— Of 159 eligible adolescents, 139 (87%) participated. Academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition were assessed. Few significant treatment group differences were found. The occurrence of seizures in the postoperative period was the medical variable most consistently related to worse outcomes. The scores of both treatment groups tended to be lower than those of the test normative populations, with substantial proportions scoring ≥1 SDs below the expected mean. Although the test scores of most adolescents in this trial cohort are in the average range, a substantial proportion have received remedial academic or behavioral services (65%). Magnetic resonance imaging abnormalities were more frequent in the d-transposition of the great arteries group (33%) than in a referent group (4%). Conclusions— Adolescents with d-transposition of the great arteries who have undergone the arterial switch operation are at increased neurodevelopmental risk. These data suggest that children with congenital heart disease may benefit from ongoing surveillance to identify emerging difficulties. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00000470.

Adolescents With d-Transposition of the Great Arteries Corrected With the Arterial Switch Procedure

Background— We report neuropsychological and structural brain imaging assessments in children 16 years of age with d-transposition of the great arteries who underwent the arterial switch operation as infants. Children were randomly assigned to a vital organ support method, deep hypothermia with either total circulatory arrest or continuous low-flow cardiopulmonary bypass. Methods and Results— Of 159 eligible adolescents, 139 (87%) participated. Academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition were assessed. Few significant treatment group differences were found. The occurrence of seizures in the postoperative period was the medical variable most consistently related to worse outcomes. The scores of both treatment groups tended to be lower than those of the test normative populations, with substantial proportions scoring ≥1 SDs below the expected mean. Although the test scores of most adolescents in this trial cohort are in the average range, a substantial proportion have received remedial academic or behavioral services (65%). Magnetic resonance imaging abnormalities were more frequent in the d-transposition of the great arteries group (33%) than in a referent group (4%). Conclusions— Adolescents with d-transposition of the great arteries who have undergone the arterial switch operation are at increased neurodevelopmental risk. These data suggest that children with congenital heart disease may benefit from ongoing surveillance to identify emerging difficulties. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00000470.

NASPE expert consensus conference: Radiofrequency catheter ablation in children with and without congenital heart disease. Report of the writing committee

A Consensus Conference on Pediatric Radiofrequency Catheter Ablation took place at the 21 Annual Scientific Sessions of the North American Society of Pacing and Electrophysiology (NASPE). The participants included health professionals from the pediatric and adult electrophysiological communities, and involved physicians, nurses, and other allied professionals. This statement attempts to coalesce the information presented and is directed to all health professionals who are involved in the care of pediatric patients undergoing ablation. In an attempt to write such a document, the editors must try to represent what they believed was the general consensus of opinion amongst the participants. As generally understood in the medical world, “consensus” does not indicate complete harmony within a group but a substantial degree of agreement. It is hoped that the information presented falls within that definition and will serve as a foundation on which to build toward in the future. Even as this document was being prepared, new technologies were introduced that may expand the indications and possibly the type of personnel and training necessary to perform these procedures. It will be interesting to look back on this statement of the current state-of-the art over the next 5–10 years and see if some of the recommendations stand the test of time. As is true of many documents, only in retrospect can a judgement be made on the strength of what is stated in this statement. The goal of the Consensus Conference was to bring together pediatric and adult practitioners in electrophysiology for an all day discussion of catheter ablation in children and in patients with congenital heart disease (CHD). The organizers believed that, in choosing topics for discussion, the development of indications for catheter ablation should flow naturally from what is known concerning the natural history of the arrhythmias in question in the pediatric population and from the latest information available concerning outcomes from these procedures. Therefore, the initial part of this statement deals with the natural history issues of specific arrhythmias in children with and without CHD. Issues of radiation exposure and animal models of toxicity due to radiofrequency (RF) application are noted followed by outcomes and complications of RF ablation for various arrhythmias. Indications for ablation are then discussed. It is important to realize that these indications as presented are not necessarily “written in stone” and do not take the place of a “Policy Statement.” Rather, as mentioned above, this represents a general sense of agreement within the community of pediatric and adult electrophysiologists . The second half of the conference then dealt with the “nuts and bolts” of performing these proNASPE POSITION STATEMENT

Psychological functioning in children with cyanotic heart defects

The relationship between congenital heart disease (CHD) and psychological functioning was studied in 63 children with transposition of the great arteries (TGA) and 77 children with tetralogy of Fallot (TF). These youngsters were compared with a group of 36 children originally diagnosed with CHD, but who spontaneously recovered (SR) without medical intervention. All children were under one year of age when diagnosed and were given psychological testing between 5.5 and 6.3 years. Children with TGA and TF had poorer overall psychological functioning and significantly greater central nervous system (CNS) impairment than the SR children. However when the effects of IQ and CNS impairment were controlled, there were no differences between groups. The diagnosis of a severe cyanotic heart defect does not appear to make a child more likely to have emotional disorder in the absence of other factors.

The longitudinal impact of psychological functioning, medical severity, and family functioning in pediatric heart transplantation

BACKGROUND Few data are available on the longitudinal psychological functioning of patients after pediatric heart transplantation. The objective of this study was to determine whether pre-transplant psychological functioning, post-transplant medical severity, and family functioning relate to the psychological functioning of pediatric patients after heart transplantation. METHODS The study included 23 patients who underwent heart transplantation between ages 3 and 20 years, survived at least 1 year after transplantation, and had been assessed previously after transplantation between 1993 and 1995. This study reports a second post-transplant assessment between 1999 and 2000. We assessed psychological functioning using the Childrens Global Assessment Scale before and after heart transplantation. We assessed medical severity using the number of outpatient visits, hospitalizations, and biopsies and using the Side Effect Severity Scale. We used the Global Assessment of Family Relational Functioning Scale to rate family functioning. RESULTS The majority of patients (15/23) were alive at the second follow-up. They had survived a median of 9.6 (6.1-12.9) years after transplantation. Similar to their first follow-up assessments, 73% demonstrated good psychological functioning after heart transplantation. Although we found no correlation between medical severity and post-transplant psychological functioning, we did find a significant correlation between family functioning during the first 2 years of transplantation and post-transplant emotional adjustment. CONCLUSIONS The majority of children and adolescents have the capacity for healthy psychological functioning after heart transplantation. Nevertheless, ongoing psychological assessment and intervention is necessary for patients and their families who face pediatric heart transplantation because >25% probably will have emotional adjustment difficulties.

Psychiatric disorders and behavioral characteristics of pediatric patients with both epilepsy and attention-deficit hyperactivity disorder.

OBJECTIVE Attention-deficit hyperactivity disorder (ADHD) coexisting with epilepsy is poorly understood; thus, we compared the clinical correlates and psychiatric comorbid conditions of 36 children with epilepsy and ADHD aged 6 to 17 years enrolled in an ADHD treatment trial, with those reported in the literature on children with ADHD without epilepsy. METHODS Measures included the Kiddie Schedule for Affective Disorders and Schizophrenia for School-Age Children (KSADS), the Wechsler Abbreviated Scale of Intelligence (WASI), and the Scales for Independent Behavior-Revised (SIB-R). RESULTS Mean IQ was 86+/-19, and SIB-R Standard Score was 72+/-26. The ADHD-Combined subtype, composed of both inattentive and hyperactive symptoms, was most frequent (58%). Sixty-one percent exhibited a comorbid disorder, including anxiety disorders (36%) and oppositional defiant disorder (31%). CONCLUSIONS Comorbidity in ADHD with epilepsy is similar to that in ADHD without epilepsy reported in the literature. These preliminary data argue that the pathophysiology of ADHD has common components in both populations.

Interactive storytelling environments: coping with cardiac illness at Boston's Children's Hospital

This paper describes exploration of uses of a computational storytelling environment on the Car- diology Unit of the ChildrenHospital in Boston, during the summer of 1997. Young cardiac patients ranging from age 7 to 16 used the SAGE environment to tell personal stories and cre- ate interactive characters, as a way of coping with cardiac illness, hospitalizations, and invasive medical procedures. This pilot study is part of a larger collaborative effort between Children ´ Hospital and MERL - A Mitsubishi Electric Research Laboratory, to develop a web-based appli- cation, the Experience Journal, to assist patients and their families in dealing with serious medical illness. The focus of the paper is on young patientsof SAGE, on SAGEaffordances in the context of the hospital, and on design recommendations for the development of future computa- tional play kits for expressing and exchanging feelings and ideas. Preliminary analysis of young patientsindicates that children used different modes of interaction-direct, mediated, and differed-, depending upon what personae the narrator chooses to take on. These modes seem to vary with the mindset and health condition of the child.

Postoperative recovery in children after minimum versus full-length sternotomy.

BACKGROUND Minimal access incisions for pediatric cardiac surgery have been reported to hasten postoperative recovery. This prospective study compared recovery after a minimum versus full-length sternotomy for repair of atrial septal defects in children. METHODS We studied 35 children undergoing atrial septal defect repair using a full-length sternotomy (n = 18) or ministernotomy (n = 17) according to the surgeons preference. All children were managed according to an established clinical practice guideline. Intraoperative comparisons included patient demographics, bypass and cross-clamp times, and, as a measure of stress response, epinephrine, norepinephrine, and lactate levels at six time intervals throughout the surgical procedure. Postoperative comparisons included pain scores at 6, 12, and 24 hours, frequency of emesis, analgesic requirements, respiratory rate and gas exchange, and length of intensive care unit and total hospital stay. Nurse and parent assessment scores of overall recovery were constructed using visual analog and Likert scales. RESULTS No significant differences between mini- versus full-length sternotomy were detected for the measured outcome variables. No adverse outcomes were detected. CONCLUSIONS In this prospective study, a ministernotomy did not enhance postoperative recovery, and the primary advantage appears to be an improved cosmetic result.

White matter microstructure and cognition in adolescents with congenital heart disease.

OBJECTIVE To describe the relationship between altered white matter microstructure and neurodevelopment in children with dextro-transposition of the great arteries (d-TGA). STUDY DESIGN We report correlations between regional white matter microstructure as measured by fractional anisotropy (FA) and cognitive outcome in a homogeneous group of adolescents with d-TGA. Subjects with d-TGA (n = 49) and controls (n = 29) underwent diffusion tensor imaging and neurocognitive testing. In the group with d-TGA, we correlated neurocognitive scores with FA in 14 composite regions of interest in which subjects with d-TGA had lower FA than controls. RESULTS Among the patients with d-TGA, mathematics achievement correlated with left parietal FA (r = 0.39; P = .006), inattention/hyperactivity symptoms correlated with right precentral FA (r = -0.39; P = .006) and left parietal FA (r = -0.30; P = .04), executive function correlated with right precentral FA (r = -0.30; P = .04), and visual-spatial skills correlated with right frontal FA (r = 0.30; P = .04). We also found an unanticipated correlation between memory and right posterior limb of the internal capsule FA (r = 0.29; P = .047). CONCLUSION Within the group with d-TGA, regions of reduced white matter microstructure are associated with cognitive performance in a pattern similar to that seen in healthy adolescents and adults. Diminished white matter microstructure may contribute to cognitive compromise in adolescents who underwent open-heart surgery in infancy.

Adaptive phase I study of OROS methylphenidate treatment of attention deficit hyperactivity disorder with epilepsy

OBJECTIVE The goal of this study was to pilot a randomized controlled trial of OROS methylphenidate (OROS-MPH) to treat attention deficit hyperactivity disorder (ADHD) plus epilepsy. METHODS Thirty-three patients, 6-18years of age, taking antiepileptic drugs and with a last seizure 1-60months prior were assigned to a maximum daily dose of 18, 36, or 54mg of OROS-MPH in a double-blind placebo-controlled crossover trial. RESULTS There were no serious adverse events and no carryover effects in the crossover trial. OROS-MPH reduced ADHD symptoms more than did placebo treatment. There were too few seizures during the active (5) and placebo arms (3) to confidently assess seizure risk; however, considering exposure time, we observed an increased daily risk of seizures with increasing dose of OROS-MPH, suggesting that potential safety concerns require further study. CONCLUSION A larger study to assess the effect of OROS-MPH on seizure risk is needed. A crossover design including subjects with frequent seizures could maximize power and address high patient heterogeneity and recruitment difficulties.