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The PedsQL 4.0 (Pediatric Quality of Life Inventory) Generic Core Scales are child self-report and parent proxy-report scales developed to measure health-related quality of life (HRQOL) in children and adolescents ages 2-18. The PedsQL 4.0 Generic Core Scales consist of 23 items applicable for healthy school and community populations and pediatric populations with acute and chronic health conditions. The 4 PedsQL 4.0 Generic Core Scales (Physical, Emotional, Social, School) were administered to 209 children and 269 parents (289 subjects accrued overall) recruited from pediatric cardiology, orthopedics, and rheumatology clinics. Sensitivity, responsiveness, and the impact on clinical decision-making were determined. The PedsQL was differentially sensitive to increasing degrees of cardiac disease severity in the cardiology clinic setting and responsive to clinical change over time in the pediatric orthopedics clinic setting. In the pediatric rheumatology clinic setting, the PedsQL demonstrated an impact on clinical decision-making, resulting in subsequent increases in HRQOL.

In recent years, enormous technological advances have occurred that allow intervention for many congenital heart defects in the pediatric cardiac catheterization laboratory. Therapeutic catheterization procedures, including valvuloplasty, angioplasty, stent implantation, coil embolization, and device occlusion, are employed to treat heart defects in infants and children. This report describes these interventional procedures, outcomes, potential complications, and implications for nursing care., (Copyright 2001 W.B. Saunders Company)

Objectives: The purpose of this study was to assess the neurodevelopmental status of children after Fontan repair of functional single ventricle and to examine the relationship between cognitive function and selected patient characteristics., Study Design: Neurodevelopmental tests including the Stanford-Binet Intelligence (IQ) scale and the Developmental Test of Visual Motor Integration (VMI) were administered to 32 children (26 months to 16 years of age) with complex single ventricle. The mean and distribution of IQ and VMI scores were compared with population norms. The relationship between test scores and patient characteristics was examined utilizing analysis of variance and correlational methods., Results: The majority of children had intellectual function within the normal range (mean, 97.5 +/- 12.1). Below average VMI scores were found in 21.4% of children. There were no significant correlations between intellectual function or visual motor integration ability and preoperative oxygen saturation or age at Fontan. Children who had deep hypothermic circulatory arrest during a prior Norwood procedure tended to have a lower IQ score., Conclusions: Intellectual development in children with Fontan repair of complex heart defects is essentially within the normal range. Visual motor integration deficits may be more prevalent in these children. In our population, the duration and degree of preoperative hypoxemia had no apparent effect on cognitive function.

Pleural effusions after the modified Fontan procedure are unpredictable, increase morbidity, and prolong hospital stay. To assess the relation between preoperative characteristics and postoperative pleural drainage, we performed a retrospective study of 71 patients who underwent Fontan procedures. Analyses revealed no significant relation between duration of effusion and age at Fontan, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, type of Fontan, or prior cavopulmonary anastomosis. Patients with significant aortopulmonary collateral vessels evidenced by angiographic opacification of the pulmonary arteries or veins had more prolonged pleural drainage. The duration of the pleural drainage was significantly less in patients who had aortopulmonary collateral occlusion.

Background: The modified Fontan procedure has become the treatment of choice for patients born with a univentricular heart. Although the operative mortality has steadily decreased in recent years, the hospital stay is still prolonged in many patients due to fluid retention and pleural effusions., Methods: We retrospectively analyzed subsets of patients undergoing the bidirectional cavopulmonary shunt (BDCPS) and modified Fontan procedure in an attempt to define factors influencing operative mortality and morbidity., Results: Multivariate analysis of 64 patients undergoing BDCPS revealed that age 6 months or less, concomitant operation, mean pulmonary pressure of 15 mm Hg or less, and mean pulmonary artery ratio of 1.8:1 or less were not statistically significant indicators of risk. Abnormal pulmonary artery architecture was a significant predictor of early and late death (p < or = 0.01). Retrospective analysis of 71 patients undergoing the modified Fontan procedure revealed no significant relationship between duration of pleural effusions and age at operation, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, or prior BDCPS: Patients with important aortopulmonary collateral vessels defined by angiography had prolonged pleural drainage. Selective use of the total extracardiac conduit and the fenestrated Fontan resulted in low overall mortality with no statistical differences between subsets of patients undergoing different modifications of the Fontan procedure., Conclusions: These data indicate that the operative risk for BDCPS or modified Fontan procedure is quite low if a procedure appropriate for the patient is selected. Abnormal pulmonary artery architecture is an important risk factor for death after the BDCPS: Aortopulmonary collateral vessels are associated with fluid retention and pleural effusions after a modified Fontan procedure. Important aortopulmonary collateral vessels should be occluded before or during the modified Fontan procedure.

Pulmonary artery distortion is a risk factor among candidates for the Fontan procedure. In 57 patients evaluated by catheterization after successful cavopulmonary anastomosis, 8 had proximal left pulmonary artery (LPA) stenosis, either discrete (4 patients) or long segment (4 patients). Median age was 27 months (range 19 to 60 months). Median weight was 11.4 kg (range 9.1 to 20.0). Mean diameter at LPA stenosis was 4.4 +/- 0.4. Proximal right pulmonary artery mean diameter was 10.4 +/- 1.0 mm. After angiographic and hemodynamic assessment, short 11F sheaths were placed in the right internal jugular (6 patients) or subclavian veins (2 patients). Pulmonary artery angioplasty and stent placement were performed. LPA stenoses were enlarged using 10 Palmaz stents dilated to 10 mm (7 patients) or to 12 mm (3 patients). Poststent angiograms showed that narrowest LPA dimensions were significantly enlarged to 9.9 mm +/- 1.0 mm, p < 001). There were no complications. Follow-up studies (catheterizations in 4 patients, echocardiograms in 8 patients) were performed 4 to 9 months after stent implantation. No restenosis was observed. Five patients had completion of their Fontan procedures; three patients are pending Fontan completion. This study demonstrates the efficacy and safety of the percutaneous use of Palmaz stents to correct pulmonary artery stenosis in young children after cavopulmonary anastomosis.

Background: The bidirectional Glenn (BDG) is frequently used in the staged surgical management of single ventricle patients. Controversy exists whether accessory pulmonary blood flow (APBF) sources should be left at the time of the BDG to augment systemic saturation or should be eliminated to reduce volume load of the ventricle. The present study was a retrospective review to assess the influence of APBF on outcome after the BDG., Methods and Results: Ninety-two patients have undergone BDG at our institute during the interval from 1986 through 1994. At the time of BDG, 40 patients had either a systemic-to-pulmonary artery shunt or patent right ventricular outflow tract as an additional source of pulmonary blood flow. Fifty-two patients had elimination of APBF. There were three operative deaths (two with and one without APBF) and four procedures (two in each group) that failed and required subsequent revision. Thus, there were 85 patients who underwent successful operation. Effusions (defined as chest tube drainage exceeding 7 days' duration) occurred in 8 of 85 patients; this complication was seen in 7 of 36 patients (19%) with APBF and 1 of 49 patients (2%) without APBF (P < .05). There were 11 deaths, including 6 patients (17%) with APBF, 2 patients (4%) without APBF, and 3 of the patients (75%) who had a failed BDG., Conclusions: The data suggest that morbidity and mortality are lower in patients in whom APBF is eliminated at the time of the BDG.

Objectives: This report summarizes our experience with the use of occluding spring coils to close the small patent ductus arteriosus., Background: Several patent ductus arteriosus occluders (most notably the Rashkind device) have been developed and studied. Occluding spring coils have been used to close abnormal vessels and vascular connections. We previously reported the use of occluding spring coils to close the small patent ductus arteriosus in a small group of patients. This report describes our series of patients having patent ductus arteriosus closure with occluding spring coils., Methods: Between June 1990 and June 1993, 30 patients underwent cardiac catheterization to have patent ductus arteriosus closure by occluding spring coils. Selection criteria were age > 6 months and narrowest patent ductus arteriosus internal dimension < or = 3.0 mm by color flow imaging. Definitive selection was based on review of aortograms performed at catheterization. A 5.2F coronary catheter was used to deliver one or two standard occluding spring coils. A loop was delivered in the main pulmonary artery, and the remainder of the coil was delivered across the patent ductus arteriosus and into the aortic diverticulum. Patent ductus arteriosus closure was confirmed by aortography or color flow imaging, or both. Follow-up after coil placement occurred at 6 weeks and 6 months and included two-view chest radiography, echocardiography and color flow imaging., Results: Of the 30 patients, 29 had successful implantation by one (27 patients) or two (2 patients) occluding spring coils. Of these 29 patients, 19 had a clinically apparent and 10 had a silent patent ductus arteriosus. Average ductus minimal internal dimension was 1.7 mm (range 1.0 to 3.0). Complete closure of the ductus was confirmed in 27 patients by aortography or color flow imaging or both (in 24 within 4 h, in 2 after 6 weeks and in 1 after 6 months). Six weeks after implantation, two patients had a tiny residual patent ductus arteriosus noted on color flow imaging. One patient did not have successful implantation. This patient had a 3.2-mm ductus, and two coils migrated to the distal left pulmonary artery and could not be retrieved. There were no deaths or any significant complications noted during early or late follow-up in these patients., Conclusions: Occluding spring coils may have additional application in closing the small patent ductus arteriosus.

Today's health care environment prompted implementation of a case management model by pediatric nurse practitioner clinical nurse specialists to promote the organization of resources for optimal care of children with heart disease. Evaluation of this pilot program suggests that achievement of expected outcomes within an appropriate length of stay was facilitated, that parents were ready for discharge, and that readmissions were infrequent. In addition, patient and system variances resulting in delay of discharge and discharge preparation needs were identified. The pediatric nurse practitioner as case manager may have a significant impact on the quality and cost of care for hospitalized children.

Children with terminal heart disease experience a dramatic improvement in functional status after heart transplantation but may be at increased risk for problems in psychosocial adaptation. Selected psychosocial outcomes were assessed in 49 pediatric heart transplant recipients and their families from five heart transplantation centers. Heart transplant recipients did not appear significantly different from their peers on self-report measures of self-concept and anxiety, but they showed significantly less social competence and more behavior problems than a normative population. Behavior problems observed were most frequently suggestive of depression and were significantly associated with greater family stress and diminished family resources for managing stress. The study findings further suggest that the heart transplant recipients' ability to verbalize or ventilate their feelings and concerns to others seems to facilitate psychosocial adaptation. Assessment of stress, resources, and coping is imperative to enable health professionals to promote the psychosocial adaptation of pediatric heart transplant recipients and their families.

Organ transplantation is allowing the hope for survival and an improved quality of life to become a reality for many children with end-stage diseases. Most pediatric transplant recipients are active and able to participate in age-appropriate activities. Despite dramatic symptomatic improvements, however, children and families continue to face major psychosocial stresses. Family stresses are related to the uncertainty of the child's future health and well-being, role strain, social isolation, and financial burdens. Pediatric transplant recipients may have difficulties with psychosocial adjustment, especially related to dissatisfaction with the cosmetic side effects of immunosuppressive therapy and a lack of socialization skills or social competence. Psychosocial stresses faced by children following transplant may result in behavior problems, depression, poorer social adaptation, or noncompliance. The psychosocial adaptation of pediatric transplant recipients can be promoted through support and counseling from health professionals caring for these families. More research is needed to evaluate the psychosocial implications of pediatric organ transplantation and identify effective methods of providing psychological support and promoting adaptation in these children.

In order to evaluate the preventive health care practices in children with heart disease, 499 families were surveyed in outpatient settings. Data were collected on 215 children with heart disease and 284 control children without known chronic illness. There was no significant difference between the groups in the type of primary physician utilized or frequency of visits to the primary care physician. Immunizations were incomplete in 32.7% of the children with heart disease compared to only 2.5% in the control group (P less than 0.0001). Among the children over 3 years of age with heart disease, 29% had not received routine dental care within the past year compared with 23.4% in the controls (P = NS). The parents of children with heart disease were found to pay less money out-of-pocket for their child's health care than the parents of control children (P less than 0.0001). The data suggest that important aspects of primary health care were neglected in a large group of children with heart disease and that cost was not a major cause for the inadequate preventive care delivery. An educational program directed at health care professionals and parents is proposed.

To examine the psychosocial responses of children and adolescents with a cardiac pacemaker and compare their responses to those of their peers, we evaluated 30 pediatric pacemaker patients, aged 7 to 19 years, and two age- and sex-matched comparison groups, including 30 patients with similar heart disease but without pacemakers and 30 physically healthy children, using standardized psychometric tests and a specific interview format. We postulated that children with pacemakers would experience greater stress in psychosocial adaptation. No significant differences on standardized measures of trait anxiety, self-competence, or self-esteem were found between the pacemaker group and the comparison groups. In contrast, pacemaker subjects were significantly (p less than 0.05) more external in their locus-of-control orientation than were healthy subjects, suggesting a diminished sense of personal control and less autonomy. Pacemaker subjects, particularly the older ones, had significantly (p less than 0.05) greater knowledge of pacemaker systems than did subjects in the other two groups, facilitating the use of intellectualization as a coping mechanism. The pacemaker patients were likely to be as fearful of social rejection as of potential pacemaker failure. All three groups identified potential negative peer reactions toward an individual with a pacemaker. The patients with cardiac disease but without pacemakers and the healthy subjects perceived significant (p less than 0.05) social and emotional differences between patients with pacemakers and their peers, but the pacemaker patients did not view themselves as different from their peers. This study demonstrates healthy psychosocial adaptation of children with cardiac pacemakers. Although these children appear to cope effectively with the stress of their life situation through the use of denial and intellectualization, they may experience problems both in the development of autonomy and in social isolation and rejection.

Each of seven infants 1 to 9 days old and weighing 2.1 to 3.3 kg with truncus arteriosus underwent repair of their defects with a right ventricular-to-pulmonary artery valveless conduit. Congestive heart failure and cyanosis were present before surgery in each. Five patients survived surgery and one of these subsequently died. Comparison between preoperative hemodynamics of the survivors and nonsurvivors disclosed similar peak systolic pulmonary arterial and aortic pressures, and pulmonary (Rp) or systemic resistance (Rs) in the groups. However, the mean Rp/Rs ratio in survivors (0.15) was significantly less than in nonsurvivors (0.63) (p = .001). The four remaining survivors are asymptomatic 7 months to 5.5 years after operation. Postoperative cardiac catheterization in three patients disclosed proximal conduit obstruction of 10 to 20 mm Hg in each, distal conduit obstruction of 35 mm Hg in two, and mild truncal valve stenosis and moderate truncal regurgitation in one each. We recommend the use of a valveless conduit for the symptomatic neonate with truncus arteriosus and a low Rp/Rs ratio (less than or equal to 0.03).

To help children with congenital heart disease, and their parents, cope with the stress of hospitalization for cardiac catheterization, a videotape was developed entitled "Leonard Z. Lion Presents ... Learning About Your Heart Catheterization." Fifty-three patients with congenital heart disease, 3 to 12 years of age, were randomly assigned to experimental (N = 31) or control (N = 22) groups. Each viewed the catheterization videotape or an unrelated control videotape before cardiac catheterization. The child's knowledge, anxiety, and adjustment during and after hospitalization for catheterization were evaluated. The experimental group displayed a significantly greater knowledge of events and sensations experienced during hospitalization than controls (P less than .0001). Children in the experimental group tended to sleep more during the catheterization procedure (P = .05) and when awake to ask more questions (P = .02). After discharge, the experimental group also tended to demonstrate more independent behavior. We conclude that viewing this videotape on heart catheterization helped children with congenital heart disease to cope with the stress of hospitalization for the procedure.

Self-concept, health beliefs, health behaviors, health information sources, and perceived health information needs were assessed in 90 patients with congenital heart disease and 54 peers without chronic illness, ages 13 to 22 years. Data revealed that patients with congenital heart disease did not differ from their healthy counterparts in overall self-concept, health beliefs, use of preventive health services, and sources of health information. Patients with congenital heart disease reported greater perceived stress in comparison to peers. Significantly more patients with heart disease needed information regarding pregnancy and contraception, and older cardiac subjects were less likely to be sexually active. For these patients who currently seem to rely largely on peers for some critical health information, sensitive and comprehensive counseling by health professionals is imperative.

The study was designed to identify some of the psychosocial barriers to compliance in a hospital-based weight control intervention program for adolescents. Forty obese adolescents, 10 to 16 years of age, and their parents were surveyed prior to participation in a behavioral change weight control program at a major teaching hospital. Significant correlations were obtained between weight loss outcome and six factors. In obese adolescents, weight loss was significantly associated with their beliefs regarding: (1) personal control over weight, (2) barriers or difficulty of losing weight, (3) medical problems as a cause of their obesity, (4) family problems as a cause of their obesity, and (5) perceived willingness of family members to diet. It is suggested that greater weight loss in children who perceived more barriers/difficulty and less family willingness to diet may reflect the importance of having realistic expectations related to behavioral compliance. In addition, a positive parental attitude or expectation that the child was less likely to be overweight in the future was associated with greater weight loss compliance. Other parental health beliefs, however, did not generally predict the child's weight loss response to the intervention. The findings lend support to the significance of the adolescent's beliefs regarding weight and family support in explaining weight loss response to a behavioral change intervention program.

Parents of ten children who underwent cardiac transplantation at the authors' institution participated in this study to evaluate the effect of heart transplantation on the family. Parents completed the Hymovich Chronicity Impact and Coping Instrument (CICI) and/or the Feetham Family Function Survey (FFFS) 3 to 24 months (mean, 8.4 months) after the transplant. Results indicate that parents face significant psychological and social stresses after their child's heart transplantation related to (1) the uncertainty of the child's future health and well-being, (2) role strain, (3) social isolation, and (4) financial burdens. Recommendations for nursing practice are suggested.