Recent research

Pediatr Transplant. 2006 Dec ;10 (8):928-33 17096760 (P,S,E,B,D)

School re-entry of the pediatric heart transplant recipient.

Constance M Weil, S Rodgers, S Rubovits
Contact: Department of Child and Adolescent Psychiatry, Children's Memorial Hospital, Chicago, IL, USA.

Pediatric cardiac transplant has become increasingly frequent in the last decade and survival rates have improved remarkably. Outcome research on this population suggests that the majority of children have the capacity for healthy adaptation although 25-40% have been shown to have some type of psychiatric difficulties. As school plays a major role in these children's lives, early intervention and close liaison with schools is indicated to reduce psychological morbidity, enhance adaptation within the school environment and enhance overall adjustment. This paper proposes a model for a school re-entry program for this population. The school re-entry program is aimed at children who are undergoing cardiac transplant and will be entering or re-entering the school system. They may range in academic age from preschool to college level and have been attending private or public schools with placements in regular education programs, regular education programs with resource support, special education programs, and alternative school programs. Others may not have been attending school because of the severity of their medical condition and have been receiving in-home tutoring. Each child is offered school re-entry assistance by a multi-disciplinary team composed of members from the Cardiology Transplant Service. The re-entry program includes cognitive and psychosocial assessment, liaison with the child's school pre- and post-transplant, academic planning and provision of academic, emotional, and behavioral support before, during, and immediately after transplant, a school re-entry visit, and an ongoing school consultation. The goal is to address issues necessary for a successful school re-entry including appropriate academic placement and support, psychosocial adjustment, education of school personnel and ongoing health needs of the student. The next step is to formally evaluate the efficacy of this program in successful school re-entry.

Matern Child Nurs J.; 1988 Winter;17(4):217-302

School adjustment of children with congenital heart disease.

N M Youssef
Contact: Faculty of Nursing, High Institute of Nursing, Cairo University, Egypt.

A correlation study was conducted to investigate school adjustment of school-age children and adolescents with varying degrees of severity of congenital heart disease. School adjustment was inferred from scholastic achievement, scholastic grade average, social competence and behavior as assessed by mothers, and adaptive functioning and behavior as assessed by teachers. Forty-eight children with mild, moderate, and severe congenital heart disease (16 children in each group) and their mothers and teachers participated in the study. Data were obtained from: (a) the Coopersmith Self-Esteem Inventory, and the Children's Depression Inventory completed by the children; (b) the Child Behavior Checklist and demographic data sheet completed by the mothers; (c) the Child Behavior Checklist--Teacher's Report Form, and the information sheet on Absence Rate and Scholastic Grade Average completed by the teachers; and (d) the children's medical records. Two-way and three-way analysis of variance were performed between the mean scores for each of the variables. Statistical significance was found (a) between the means for absence rate, behavior as assessed by teachers, and depression with respect to severity of congenital heart disease; (b) between the means for scholastic achievement, scholastic grade average, and adaptive functions and behavior as assessed by teachers according to IQ level; (c) between the means for scholastic achievement, scholastic grade average, and social competence as assessed by mothers regarding level of self-esteem; (d) between the mean scores for social competence with respect to level of depression. There were also three significant interactions: between age and sex for the means of behavior as assessed by mothers; between severity of heart disease and I level for the means of behavior as assessed by teachers; and between severity of heart disease and depression level for the means of social competence. It was concluded that children with congenital heart disease with the added burdens of below average IQ, low self-esteem, and high depression were at particular risk for poor school adjustment.

The Journal of pediatrics 2008, vol. 152, no3, pp. 349-355.

Health-related quality of life in children and adolescents after open-heart surgery.

Landolt MA, Valsangiacomo Buechel ER, Latal B.
Contact: Department of Psychosomatics and Psychiatry, University Children's Hospital, Zurich, Switzerland.

OBJECTIVE: To assess health-related quality of life (HRQOL) in children with congenital heart disease (CHD) after open-heart surgery and to identify medical, individual, and family-related predictors of outcome.
STUDY DESIGN: Cross-sectional cohort study. 155 children with CHD (40% cyanotic type) who underwent open-heart surgery between 1995 and 1998 were eligible. One hundred and ten patients (response rate 71%) were investigated at a mean age of 10.4 years. Mean age at operation was 2.3 years (range, 0-8.7 years), mean duration of cardiopulmonary bypass was 95 minutes (range, 5-206 minutes), and circulatory arrest was performed in 9% of the children. Child- and parent-rated HRQOL was evaluated by the TNO-AZL Child Quality of Life Questionnaire. Scores were compared with healthy references.
RESULTS: Most dimensions of self-reported HRQOL were impaired, including autonomy and motor, social, and emotional functioning. In addition to the children's ratings, parents rated cognitive functioning of their children as diminished. Multivariate analyses revealed that duration of cardiopulmonary bypass, length of hospitalization, need for current cardiac medication, and adverse family relationships had a negative impact on parent- but not on child-rated HRQOL.
CONCLUSIONS: HRQOL is impaired in children with CHD after open-heart surgery. Medical variables and the quality of family relationships are important determinants.

European Journal of Cardiovascular Prevention and Rehabilitation. (2005) Vol. 12. Issue. 2 pp. 138-143.

Quality of life and specific problems after cardiac surgery in adolescents and adults with congenital heart diseases.

Immer FF, Althaus SM, Berdat PA, Saner H, Carrel TP.
Contact: Department of Cardiovascular Surgery, University Hospital, 3010 Bern, Switzerland.

BACKGROUND: Grown-ups with congenital heart disease (GUCH) constitute an increasing population. Some of them reach adulthood without intervention and may present with symptoms, some require first intervention or re-operation for various reasons. Although interventions become more and more frequent in these patients, limited knowledge exists on their quality of life (QoL). The aim of the present study was to analyze QoL in GUCH patients who underwent cardiac surgery after the age of 14 years.
DESIGN: A total of 296 patients with a mean age of 35+/-16 years (range 14-72 years) were operated on at our institution between July 1987 and December 2000, mainly for atrial septal defect (ASD), outflow tract lesion, Marfan syndrome and co-arctation. Early mortality was 3.4%. During follow-up QoL was assessed with the short form 36 health survey questionnaire (SF-36) and an additional questionnaire focused on medical and psychosocial aspects. Results were analyzed for the total collective and in relation to the underlying congenital heart disease (CHD).
RESULTS: Quality of life was excellent and similar to an age- and gender-matched standard population, except in patients following repair of complete AV-canal. The main restrictions in this group were found in the emotional aspect (62.5+/-29.9) and physical role function (60.5+/-25.0) and reflected in limitations of daily activity.
CONCLUSION: Outcome and QoL in adolescents and adults with CHD is excellent and similar to that of an age- and gender-matched standard population. In the future special attention should be focused more strongly on medical follow-up and psychosocial problems in this increasing group of patients.

The Journal of pediatrics. 2007, vol. 151, no1, pp. 73-78

Neuropsychological performance in school-aged children with surgically corrected congenital heart disease.

Miatton M, De Wolf D, François K, Thiery E, Vingerhoets G.
Contact: Laboratory for Neuropsychology, Department of Internal Medicine, Ghent University, Ghent, Belgium.

OBJECTIVE: As surgical management of children with congenital heart disease (CHD) advanced, developmental outcome became the main focus of contemporary research. In this study, we specify the cognitive profile of children with CHD, 6 to 12 years postoperatively.
STUDY DESIGN: Patients with CHD (n = 43, mean age 8 years, 8 months) and healthy controls (n = 43, mean age 8 years, 11 months), were examined with an abbreviated intelligence scale (Wechsler Intelligence Scale for Children-3rd edition, Dutch version) and a developmental neuropsychological assessment battery (NEPSY [a developmental NEuroPSYchological assessment]).
RESULTS: We identified significantly lower scores for the CHD group on Estimated Full Scale IQ (P < .01). Neuropsychological assessment revealed lower scores for the CHD group on the cognitive domains of Sensorimotor Functioning (P < .001), Language (P < .001), Attention and Executive Functioning (P < .05), and Memory (P < .05). Children with CHD displayed more impulsive test behavior than healthy peers. No differences on IQ or cognitive domains were found between the cyanotic and the acyanotic CHD group.
CONCLUSIONS: Six to 12 years postoperatively, children with CHD display a neuropsychological profile with mainly mild motor deficits and subtle difficulties with language tasks. Attention/executive functioning and memory also appear involved but to a lesser degree. Long-term follow-up of children with surgically corrected CHD, even when hemodynamically successful, is warranted, as they are at risk for neurodevelopmental delay at school age.

Heart 2001;85:687-691 (June)

Congenital heart disease and cardiac surgery in childhood: effects on cognitive function and academic ability.

Wray J, Sensky T.
Contact: Paediatric Surgical Unit, Harefield Hospital, Harefield, Middlesex UB9 6JH, UK.

OBJECTIVE: To evaluate changes in cognitive and academic functioning following cardiac surgery in children with congenital heart disease. DESIGN: A prospective cross sectional study in which patients were assessed immediately before treatment and 12 months later.
PATIENTS: Three groups of children aged 3.5-17 years: a group with congenital heart disease awaiting surgery, another awaiting bone marrow transplantation, and a healthy comparison group.
MAIN OUTCOME MEASURES: Intelligence quotient and measures of academic attainment, evaluated with the British Ability Scales.
RESULTS: Preoperatively, children with cyanotic lesions showed deficits in comparison with those with acyanotic lesions. Postoperatively, children with cyanotic lesions showed a deterioration in performance and achieved significantly lower scores than those with acyanotic lesions. While there were significant differences between the congenital heart disease and bone marrow transplantation groups preoperatively, these were no longer apparent at follow up.
CONCLUSIONS: In contrast to previously published findings, the present results suggest that cardiac surgery does not result in early postoperative improvements in cognitive function for children with congenital heart disease. The nature of the cardiac lesion continues to affect cognitive and academic performance, even after surgery.

Cardiology in the Young (2006), 16 : 157-164 Cambridge University Press

Health and well-being of children with congenital cardiac malformations, and their families, following open-heart surgery.

Majnemer A, Limperopoulos C, Shevell M, Rohlicek C, Rosenblatt B, Tchervenkov C.
Contact: Department of Neurology and Neurosurgery, School of Physical and Occupational Therapy, Montreal Children's Hospital-McGill University, Montreal, Quebec, Canada.

Infants who survive open-heart surgery are at risk for developmental disability, which may impact on the well-being not only of the child, but also the family. The objective of our prospective study, therefore, was to determine the long-term health-related quality of life of children with congenital cardiac malformations following open-heart surgery, and to describe the persisting level of stress in their families. To this end, 49 parents completed the Child Health Questionnaire, the Parenting Stress Index, and the Child Behaviour Checklist as part of a developmental follow-up protocol when their child was 5 years of age. Mean scores on the Child Health Questionnaire were in the normal range, with physical well-being equal to 53.5, psychosocial well-being 50.9, with only 6.4 percent and 8.5 percent of subjects, respectively, falling within the suboptimal range of less than 40. The distribution of scores on the Parenting Stress Index, however, were more variable, with over one-quarter of parents indicating a high level of stress, with almost one-fifth having low levels of stress, and just over half scoring in the normal range, with the group mean being 52.6 plus or minus 32.3. An abnormal neurologic examination before surgery was associated with lower physical health (beta equal to -5.5, p equal to 0.02, r2 equal to 0.18), whereas lower arterial saturations of oxygen, less than 85 percent preoperatively, was associated with lower psychosocial health (beta equal to -6.6, p equal to 0.01, and r(2) equal to 0.14). The internalizing and externalizing behaviours of the child were significantly correlated with psychosocial well being, with r ranging from -0.32 to -0.52, and p less than 0.05. Parental stress also correlated with psychosocial health (r equal to -0.48 and p equal to 0.0009). Overall, the perception by the parents of the health-related quality of life of their child is favourable 5 years following open-heart surgery during infancy. Many parents, nonetheless, continue to feel either stressed or defensive about their child, particularly if their child exhibits behavioural difficulties. Our findings suggest that strategies need to be considered to enhance family well-being in the planning and delivery of health services to this population at high risk.

Cardiology in the Young (2004), 14 : 373-379 Cambridge University Press

Follow-up in patients with congenital cardiac disease more complex than haemodynamic assessment.

Fredriksen PM, Mengshoel AM, Frydenlund A, Sørbye Ø, Thaulow E.
Contact: Physiotherapy Department, Rikshospitalet University Hospital, Oslo, Norway.

The objective of the study was to assess behavioural and emotional problems, as well as physical capacity, in children and adolescents with congenital cardiac disease. From the database of Paediatric Heart Section, Children's Clinic, Rikshospitalet University Hospital, Oslo, Norway, we identified 430 patients whose parents received questionnaires using the Child Behaviour Check-List. The response rate was 75.8%. In addition, the parents received a questionnaire focusing on special issues with regard to physical activity. Parents of children and adolescents with congenital cardiac diseases reported significantly more behavioural problems than did a reference population and boys were scored higher compared to girls. Analysis showed a significant impact of physical capacity on the score representing total problems, as well as scores for externalising and internalising behaviour. Compared to a reference population, parents of children and adolescents with congenital cardiac disease score their children higher on most scales when rated using the Child Behaviour Check-List. The type of diagnosis did not affect the scores reflecting the total problem. The main factor of impact on behavioural problems was, as evaluated by the parents, the physical capacity of the children.

European Heart Journal 1998 19(5):801-807

Prediction of behavioural and emotional problems in children and adolescents with operated congenital heart disease.

Utens EM, Verhulst FC, Duivenvoorden HJ, Meijboom FJ, Erdman RA, Hess J.
Contact: Department of Child and Adolescent Psychiatry, Sophia Children's Hospital, Rotterdam, The Netherlands.

AIMS: The aim of the present study was to determine which medical variables were predictors of long-term behavioural/emotional outcome after surgical correction for congenital heart disease in infancy and childhood.
METHODS: The Child Behavior Checklist (CBCL) was used to predict parent-reported behavioural/emotional problems in 125 10-15 year-old congenital heart disease children from: (1) biographical status (2) medical history (3) heart surgery (4) short-term post-operative course and (5) number of heart operations and (6) extra cardiac concomitant anomalies.
RESULTS: Higher CBCL total problem scores at follow-up were associated with a greater number of heart operations and deep hypothermic circulatory arrest (< 22 degrees). 'Internalizing problems' were associated with a greater number of heart operations, deep hypothermic circulatory arrest, a short gestational age, low systemic oxygen saturation, and older age at surgical repair. 'Externalizing problems' were associated with a greater number of heart operations only.
CONCLUSION: Several medical variables were significant predictors and can be used to identify those congenital heart disease children who are at risk of long-term behavioural/emotional maladjustment.

Pediatrics Vol. 121 No. 4 April 2008, pp. e759-e767

Inattention, Hyperactivity, and School Performance in a Population of School-Age Children With Complex Congenital Heart Disease

Amanda J. Shillingford, MD, Marianne M. Glanzman, MD, Richard F. Ittenbach, PhD, Robert R. Clancy, MD, J. William Gaynor, MD and Gil Wernovsky, MD

INTRODUCTION: There is a growing interest in characterizing the neurodevelopmental outcomes of school-age survivors of cardiac surgery. The purpose of this study was to examine a population of 5- to 10-year-old children who underwent newborn cardiac surgery for complex congenital heart disease to characterize and assess risk factors for problems with inattention and hyperactivity, as well as the use of remedial school services.
PATIENTS AND METHODS: This study was a cross-sectional analysis of patients who underwent newborn cardiac surgery and were enrolled in a neuroprotection trial conducted at our institution between 1992 and 1997. Parents and teachers completed questionnaires for the school-age child to elicit information pertaining to the child's general health and academic performance. The severity of hyperactivity and inattention were assessed by using 2 standardized questionnaires (Attention-Deficit/Hyperactivity Disorder Rating Scale-IV and Behavior Assessment System for Children). In addition to calculating descriptive estimates of their occurrence, single-covariate logistic regression models were specified and tested by using 3 different outcomes (inattention, hyperactivity, and use of remedial school services) and 14 different covariates representing preoperative, intraoperative, and postoperative factors.
RESULTS: Data were obtained from parents and/or teachers for 109 children. Fifty-three (49%) were receiving some form of remedial academic services, and 15% were assigned to a special-education classroom. The number of children receiving clinically significant scores for inattention and hyperactivity on the Behavior Assessment System for Children was 3 to 4 times higher than observed in the general population. On the Attention-Deficit/Hyperactivity Disorder Rating Scale-IV, 30% of the parents reported high-risk scores for inattention and 29% reported high-risk scores for hyperactivity. No perioperative factors were statistically associated with adverse outcomes.
CONCLUSION: In this cohort of children with complex congenital heart disease, a significant proportion of the children were at risk for inattention and hyperactivity, and nearly half were using remedial school services. We did not identify any perioperative risk factors, which correlated with high-risk scores or the use of remedial school services. Ongoing neurodevelopmental follow-up and screening are recommended in this vulnerable population.