General health status of children with D-transposition of the great arteries after the arterial switch operation.

BACKGROUND: To study the long-term impact on general health status of D-transposition of the great arteries (D-TGA) after the arterial switch operation (ASO) during infancy, we asked parents to complete the Child Health Questionnaire, Parent Form-50 when their children were 8 years old. METHODS AND RESULTS: Of 160 eligible patients, questionnaires were completed for 155 subjects (96%). Median age at surgery was 6 days (range 1 to 67 days), and median age at completion of the Child Health Questionnaire was 8.1 years (7.6 to 10.0 years). Subsequent to questionnaire completion, children underwent psychometric testing. Mean Physical Health Summary and Psychosocial Summary scores were 54.0+/-6.1 and 49.7+/-9.9, respectively, which were similar to those of normal subjects. Compared with the normative sample, parents of D-TGA patients reported more problems with attention, learning, and speech, as well as greater frequency of developmental delay (P<0.001 for each). Worse Psychosocial Summary scores were significantly associated with lower full-scale IQ (P=0.001) and lower achievement in reading (P=0.005) and math (P=0.007). Worse Physical Health Summary scores were associated with longer hospital stay after the ASO (P=0.02). General health status scores were not significantly related to presence of ventricular septal defect, age at surgery, perfusion variables during the ASO, sex, or history of cardiac reoperation. CONCLUSIONS: At age 8 years, children with D-TGA after ASO have an overall physical and psychosocial health status similar to that of the general population. Lower IQ and academic achievement are associated with worse psychosocial health status, whereas longer hospital course after initial surgery is associated with worse physical health status.

Developmental and neurologic effects of alpha-stat versus pH-stat strategies for deep hypothermic cardiopulmonary bypass in infants.

OBJECTIVES: In a randomized single-center trial, we compared developmental and neurologic outcomes at 1 and 2 to 4 years of age in children who underwent reparative cardiac operations at less than 9 months of age after use of the alpha-stat versus pH-stat strategy during deep hypothermic cardiopulmonary bypass. METHODS: Among 168 children eligible for follow-up, 1-year developmental evaluations were carried out on 111, neurologic evaluations on 110, and electroencephalographic evaluations on 102. Parents of 122 children completed questionnaires on behavior and development when children were 2 to 4 years of age. RESULTS: The Psychomotor Development Index scores of the alpha-stat and pH-stat groups did not differ significantly (P =.97). For Mental Development Index scores, the treatment group effect differed according to diagnosis (P =.007). In the D -transposition of the great arteries (n = 59) and tetralogy of Fallot (n = 36) subgroups, the pH-stat group had slightly higher Mental Development Index scores than the alpha-stat group, although these differences were not statistically significant. In the ventricular septal defect subgroup (n = 16), the alpha-stat group had significantly higher scores. Psychomotor Development Index and Mental Development Index scores were significantly higher in the group with D -transposition of the great arteries than in the other 2 groups (P =.03 and P =.01, respectively). Across all diagnoses, Mental Development Index scores were significantly higher than Psychomotor Development Index scores (P <.001). Treatment group assignment was not significantly associated with abnormalities on neurologic examination (P =.70) or electroencephalographic examination (P =.77) at 1 year or with parents' ratings of children's development (P =.99) or behavior (P =.27) at age 2 to 4 years. CONCLUSIONS: Use of alpha-stat versus pH-stat acid-base management strategy during reparative infant cardiac operations with deep hypothermic cardiopulmonary bypass was not consistently related to either improved or impaired early neurodevelopmental outcomes.

An evaluation of an innovative multimedia educational software program for asthma management: report of a randomized, controlled trial.

BACKGROUND: Asthma continues to be a substantial cause of morbidity in pediatric populations. New strategies are needed to provide cost-effective educational interventions for children with asthma, particularly those in the inner city. OBJECTIVE: To assess the effectiveness of a multimedia educational software program about asthma. SETTING: A hospital-based primary care clinic and an affiliated neighborhood health center. DESIGN: Randomized, controlled trial. POPULATION: Children 3 to 12 years old with physician-diagnosed asthma. INTERVENTION: An interactive educational computer program, Asthma Control, designed to teach children about asthma and its management. Using a graphic display of a child going through simulated daily events, the game emphasizes: 1) monitoring; 2) allergen identification; 3) use of medications; 4) use of health services; and 5) maintenance of normal activity. Control group participants reviewed printed educational materials with a research assistant. OUTCOMES: Acute health care use (emergency department and outpatient) was the primary outcome. Secondary outcome measures included maternal report of asthma symptom severity, child functional status and school absences, satisfaction with care, and parental and child knowledge of asthma. RESULTS: A total of 137 families were enrolled in the study (76 intervention, 61 control). Both intervention and control groups showed substantial improvement in all outcomes during the 12-month follow-up period. Aside from improvement in knowledge after use of the computer program, no differences were demonstrated between the 2 groups in primary or secondary outcome measures. Children reported enjoyment of program use. CONCLUSIONS: This trial of an educational software program found that it did not produce greater improvement than occurred with review of traditional written materials. Because both groups showed substantial improvement over baseline, computer-based education may be more cost-effective. Alternatively, improvements in illness severity over time may overshadow the effects of such interventions. Rigorous comprehensive evaluations such as this are necessary to assess new interventions intended to improve management and outcomes of asthma.

Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass.

BACKGROUND: It is not known whether developmental and neurological outcomes in the preschool period differ depending on whether the predominant vital organ support strategy used in infant heart surgery was total circulatory arrest (CA) or low-flow cardiopulmonary bypass. METHODS AND RESULTS: Infants with D-transposition of the great arteries who underwent an arterial-switch operation were randomly assigned to a support method consisting predominantly of CA or low-flow cardiopulmonary bypass. Developmental and neurological status were evaluated blindly at 4 years of age in 158 of 163 eligible children (97%). Neither IQ scores nor overall neurological status were significantly associated with either treatment group or duration of CA. The CA group scored lower on tests of motor function (gross motor, P=0.01; fine motor, P=0.03) and had more severe speech abnormalities (oromotor apraxia, P=0.007). Seizures in the perioperative period, detected either clinically or by continuous electroencephalographic monitoring, were associated with lower mean IQ scores (12.6 and 7.7 points, respectively) and increased risk of neurological abnormalities (odds ratios, 8.4 and 5.6, respectively). The performance of the full cohort was below expectations in several domains, including IQ, expressive language, visual-motor integration, motor function, and oromotor control. CONCLUSIONS: Use of CA to support vital organs during open heart surgery in infancy is associated, at the age of 4 years, with worse motor coordination and planning but not with lower IQ or with worse overall neurological status.

Addressing sexual abuse in the primary care setting.

OBJECTIVE: To describe factors that prompt pediatric practitioners to suspect child sexual abuse, the barriers to inquiry, and the approach to management of cases of possible abuse. DESIGN: Qualitative, descriptive, and case-based. METHODS: Six focus group interviews were conducted. Maternal and Child Health Bureau-sponsored collaborative office rounds groups nationwide participated in discussions of five vignettes. Each group interview lasted 1.5 hours and had 7 to 16 participants (n = 65). Audiotaped data were transcribed and analyzed independently for themes by two reviewers. RESULTS: Five themes emerged from the group interviews: anticipatory guidance, red flags, approach to management, terminology used in discussions, and barriers to inquiry. All groups discussed giving anticipatory guidance about sexual abuse. Half (3/6) believed girls were more likely to be victimized, and some (2/6) gave more anticipatory guidance to girls for this reason. Although some groups reported giving anticipatory guidance about sexual abuse, many reported inconsistencies in their practice. All groups identified historical, behavioral, and physical red flags for sexual abuse but believed that they were not trained in residency to recognize these signs. There was no consensus regarding the approach to management of cases of possible sexual abuse, and many participants did not know the types of questions that they should be asking children when they suspect abuse. Members of all groups reported using imprecise terms when they discuss sexual issues with families. Most (4/6) believed that it was a practitioner's responsibility to inquire about abuse but believed that their discomfort with sexual topics was a barrier to inquiry. All believed that the most significant barrier to inquiry was inadequate training in the area of sexual abuse and that cases are missed because of lack of training. CONCLUSIONS: Highly motivated pediatric practitioners reported that they give anticipatory guidance about sexual abuse inconsistently, that they were not trained to recognize red flags for sexual abuse, and that they do not have a consistent approach to cases of suspected abuse. Additionally, they reported that they are not comfortable discussing sexual issues and that they miss cases of sexual abuse primarily because of lack of training. Educational interventions that target these themes are essential to improve the ability of pediatricians to screen children and to intervene when sexual abuse is identified.

Relation of seizures after cardiac surgery in early infancy to neurodevelopmental outcome. Boston Circulatory Arrest Study Group.

BACKGROUND: The outcome of infants who have transient seizures after open heart surgery has not been studied. Using the database of the Boston Circulatory Arrest Study involving 171 children with D-transposition of the great arteries, we explored the relationship between early postoperative clinical and EEG seizures and neurodevelopmental outcomes at ages 1 and 2 1/2 years. METHODS AND RESULTS: At 1 year, children returned for developmental and neurological evaluations and MRI. Parent-completed developmental questionnaires were collected at 2 1/2 years of age. At 1 year, children with early postoperative seizures had lower Psychomotor Development Index (motor function) scores (clinical seizures: 12.9 mean difference [MD]; 95% confidence interval [CI], 2.2 to 23.6; P=.02; EEG seizures: 13.3 MD; 95% CI, 6.8 to 19.7; P<.001). Mental Developmental Index scores of children with clinical or EEG seizures were also lower, but the differences were not statistically significant. Infants with seizures were more likely to have an abnormal neurological examination (clinical seizures: 78% versus 31%; P=.008; EEG seizures: 58% versus 34%; P=.04). Children with EEG seizures were more likely to have MRI abnormalities (43% versus 13%, P=.002). At age 2 1/2, children with EEG seizures had lower scores in several areas of function. CONCLUSIONS: In infants undergoing the arterial switch operation for correction of D-transposition of the great arteries, transient postoperative clinical and EEG seizures were associated with worse neurodevelopmental outcomes at ages 1 and 2 1/2 years as well as neurological and MRI abnormalities at 1 year of age. The occurrence of such seizures may provide an early sign of brain injury with neurological and developmental sequelae.

Patterns of developmental dysfunction after surgery during infancy to correct transposition of the great arteries.

A randomized clinical trial was conducted to compare the early development of children with d-transposition of the great arteries (N = 171) repaired by an arterial switch operation that used either predominantly total circulatory arrest or predominantly continuous low-flow cardiopulmonary bypass. The children assigned to the circulatory arrest group scored lower than those assigned to low-flow bypass on the Bayley Scales of Infant Development at 1 year of age. Responses to parental questionnaires completed when the children were 2.5 years old indicated that the children in the circulatory arrest group, especially those with a ventricular septal defect, also manifested poorer expressive language and were considered to display more internalizing and externalizing problem behaviors. The use of circulatory arrest to protect vital organs during open heart surgery might place children at increased risk of early developmental difficulties, especially in the domains of motor and language function.

Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass.

BACKGROUND: Deep hypothermia with either total circulatory arrest or low-flow cardiopulmonary bypass is used to support vital organs during heart surgery in infants. We compared the developmental and neurologic sequelae of these two strategies one year after surgery. METHODS: Infants with D-transposition of the great arteries who underwent an arterial-switch operation were randomly assigned to a method of support consisting predominantly of circulatory arrest or a method consisting predominantly of low-flow bypass. Developmental and neurologic evaluations and magnetic resonance imaging (MRI) were performed at one year of age. RESULTS: Of the 171 patients enrolled in the study, 155 were evaluated. After adjustment for the presence or absence of a ventricular septal defect, the infants assigned to circulatory arrest, as compared with those assigned to low-flow bypass, had a lower mean score on the Psychomotor Development Index of the Bayley Scales of Infant Development (a 6.5-point deficit, P = 0.01) and a higher proportion had scores < or = 80 (i.e., 2 SD or more below the population mean) (27 percent vs. 12 percent, P = 0.02). The score on the Psychomotor Development Index was inversely related to the duration of circulatory arrest (P = 0.02). The risk of neurologic abnormalities increased with the duration of circulatory arrest (P = 0.04). The method of support was not associated with the prevalence of abnormalities on MRI scans of the brain, scores on the Mental Development Index of the Bayley Scale, or scores on a test of visual-recognition memory. Perioperative electroencephalographic seizure activity was associated with lower scores on the Psychomotor Development Index (P = 0.002) and an increased likelihood of abnormalities on MRI scans of the brain (P < 0.001). CONCLUSIONS: Heart surgery performed with circulatory arrest as the predominant support strategy is associated with a higher risk of delayed motor development and neurologic abnormalities at the age of one year than is surgery with low-flow bypass as the predominant support strategy.

Infants tolerate spinal anesthesia with minimal overall autonomic changes: analysis of heart rate variability in former premature infants undergoing hernia repair.

Unlike adults, neonates tolerate high thoracic spinal anesthesia with minimal changes in heart rate (HR) and arterial blood pressure. To examine the potential autonomic regulatory mechanisms which may account for these findings, the relation between short-term heart rate variability (HRV) and respiratory activity was analyzed in a group of eight ASA grade II former premature infants undergoing high thoracic spinal anesthesia for inguinal hernia repairs. Quantitative measures of sympathetic (As) and parasympathetic (Ap) modulation of HR were derived. HR, arterial blood pressure, and a calibrated respiratory signal were recorded during 4.4-min stable epochs in eight subjects 1) preoperatively, 2) postincision after high thoracic spinal anesthesia, and 3) during an active sleep state in the postoperative period. Power spectral analysis of HRV and respiratory power yielded measures of low-frequency power (LFP: 0.02-0.15 Hz) and high-frequency power (HFP: 0.15-0.8 Hz). Transfer function analysis between respiratory activity and HR were used to quantify As and Ap. All subjects had successful high thoracic spinal anesthesia with highest levels ranging from C7-T4. Mean HR, blood pressure, and respiratory power did not change significantly with high thoracic spinal anesthesia. LFP and HFP both decreased significantly, whereas the LFP/HFP ratio remained stable. Group mean As and Ap both decreased, but the changes were not significant. Despite overall cardiovascular stability, HRV decreased with high thoracic spinal anesthesia, but the balance between LFP and HFP remained stable, suggesting that the reflex response to high thoracic spinal anesthesia was predominantly diminished parasympathetic modulation of cardiac function. The expected decrease in HR and blood pressure from the sympatholysis which results from high thoracic spinal anesthesia were apparently offset by withdrawal of cardiac vagal activity.

Relation of pH strategy and developmental outcome after hypothermic circulatory arrest.

To examine whether pH management during core cooling is a risk factor for adverse developmental outcome, we studied 16 children with transposition of the great arteries and intact ventricular septum who underwent a Senning procedure in infancy (median age 32 days, range 2 to 154 days) between 1983 and 1988. Information was collected retrospectively on many aspects of perfusion, including lowest carbon dioxide tension during core cooling, duration of core cooling, and duration of circulatory arrest. The pH strategy changed from pH-stat to alpha-stat in 1985, resulting in a wide range of pH values and carbon dioxide tension (34 to 76 mm Hg) during the study period. All children had rapid core cooling to a rectal temperature of 19.8 degrees +/- 2.7 degrees C (mean +/- standard deviation) and a tympanic temperature of 16.6 degrees +/- 3.0 degrees C. Development was assessed at median age 48.0 (11 to 79) months with the Bayley Scales (n = 4, children younger than 30 months) or the McCarthy Scales (n = 12, children older than 30 months). The mean core-cooling duration was 14.5 +/- 6.2 minutes, circulatory arrest time was 43.4 +/- 6.6 minutes, and total bypass plus circulatory arrest time was 89.7 +/- 12.7 minutes. Lower carbon dioxide tension (alpha-stat) before onset of circulatory arrest was associated with worse developmental outcome (r = 0.71, p = 0.002). This relationship remained highly significant when we controlled for sociodemographic and intraoperative variables. including core-cooling time, circulatory arrest time, and total elapsed time. Duration of circulatory arrest was not associated with developmental outcome. We conclude that when relatively rapid core cooling is used to achieve hypothermia before circulatory arrest in young infants, a more alkaline pH strategy such as alpha-stat may result in less effective cerebral protection.

Recurrent abdominal pain during childhood.

RAP offers a complex and often confusing array of symptoms and diagnostic possibilities. This may be due to its unique age of presentation, its inherent somatic and cognitive developmental issues, or the physiology of abdominal pain itself. A careful examination of the historic and physical findings should produce a therapeutic plan that addresses somatic, psychological, and environmental aspects of the child. This process will avoid overly simplistic and premature misdiagnosis or potentially unnecessary investigations that convey a sense of disinterest, haste, and disbelief in the problem. The successful management of RAP lies in the recognition that serious underlying disease frequently is not present and that time usually is on our side. It is the process of continued and thoughtful evaluation and reassurance over time that counts.

Cognitive development of children following early repair of transposition of the great arteries using deep hypothermic circulatory arrest.

Twenty-eight children who underwent corrective cardiac surgery in early infancy had developmental evaluations to explore whether cardiopulmonary bypass perfusion variables are associated with later cognitive function. All had transposition of the great arteries repaired by the arterial switch operation using deep hypothermic circulatory arrest. The mean duration of deep hypothermic circulatory arrest was 64 +/- 10 minutes (mean +/- SD). Median age at repair was 4 days (range 1 to 125 days). Tests of development were administered at age 7 to 53 months: Bayley Scales for children younger than 30 months of age (n = 18) and McCarthy Scales for older children (n = 10). Overall cognitive development score was 101.2 +/- 11.1. Duration of deep hypothermic circulatory arrest was not associated with performance. However, for core cooling periods of less than 20 minutes' duration, shorter cooling periods were associated with lower scores (r = .85, n = 11, P less than .001). These data suggest that patients undergoing relatively long periods of deep hypothermic circulatory arrest may require some minimum time of cardiopulmonary bypass cooling to avoid central nervous system injury.

The prevention of constipation and encopresis: a developmental model and approach.

This article proposes a developmental model for understanding the evolution and treatment of bowel problems in infancy and childhood. It focuses on anticipatory guidance to maximize parental understanding of the normal variations of bowel function and specific interventions to limit the long-term effects of mild bowel problems.

Recurrent abdominal pain in school children: the loneliness of the long-distance physician.

The recurring of abdominal pain can be a taxing clinical ordeal for the primary care physician or consultant. It often is a test of stamina, of diagnostic self-confidence, of fiscal constraint, and of vigilance for rare conditions. The clinician is apt to be haunted by the lingering question: "Am I missing something?" That which might or might not be "missed" comprises the content as well as the rationale for this review.