Richard F. Ittenbach

Risk factors for mortality after the Norwood procedure

OBJECTIVES Recent studies have suggested that survival following the Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS), particularly aortic atresia (AA), as compared to other forms of functional single ventricle and systemic outflow tract obstruction. The current study was undertaken to evaluate our recent experience with the Norwood procedure and to evaluate potential predictors of operative and 1-year mortality. METHODS A retrospective study of risk factors for operative and 1-year mortality in 158 patients undergoing the Norwood procedure between January 1, 1998 and June 30, 2001. RESULTS HLHS was present in 102 patients (70 with AA) and other forms of functional single ventricle with systemic outflow tract obstruction in the remaining 56. Operative survival was 77% (122/158), 78% for patients with HLHS and 75% for patients with other diagnoses. Multivariable analysis identified birth weight (odds ratio (OR) 0.18/kg, 95% confidence limit (CL) 0.08-0.42, P<0.001), associated cardiac anomalies (OR 4.45, 95% CL 1.50-13.2, P=0.001), total support time (OR 1.02/min, 95% CL 1.01-1.03, P=0.004), and extracorporeal membrane oxygenation (ECMO) or ventricular assist device (VAD) support (OR 17.8, 95% CL 4.40-71.0, P<0.001) as predictors of operative mortality. The anatomic diagnosis (HLHS versus non-HLHS) was not a predictor of mortality, P=0.6). The Kaplan-Meier survival estimate at 1 year was 66% (95% CL 58-73%) and was not different for patients with HLHS compared to non-HLHS, P=0.5. For patients who have survived the Norwood procedure, survival to 1 year was 86% (95% CL 78-91%). Presence of an extra-cardiac anomaly or genetic syndrome (OR 2.70, 95% CL 0.98-7.41%, P=0.05) and presence of an additional cardiac defect (OR 3.99, 95% CL 1.67-9.57, P=0.002) were predictors of worse survival in the first year of life. CONCLUSIONS The Norwood procedure is currently being applied to a heterogeneous group of patients. Operative and 1-year survival are equivalent for patients with HLHS and those with other cardiac defects. The presence of additional cardiac or extra-cardiac anomalies are predictors of poor outcome.

Inattention, hyperactivity, and school performance in a population of school-age children with complex congenital heart disease.

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 childs 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.

Use of complementary and alternative medicine among children recently diagnosed with autistic spectrum disorder.

ABSTRACT. This study examined the prevalence and correlates of use of different types of complementary and alternative medicine (CAM) among a sample of children with suspected or recently diagnosed autism. The authors’ review of 284 charts of children seen at the Regional Autism Center of The Children’s Hospital of Philadelphia, Pennsylvania, found that more than 30% of children were using some CAM, and that 9% were using potentially harmful CAM. Having an additional diagnosis was protective against CAM use and being Latino was associated with CAM use. Having seen a prior provider regarding the child’s health condition was predictive of potentially harmful CAM use. Further research is required on cultural differences in treatment decisions about CAM, as well as the reasons for the association between the use of prior providers and CAM. The high prevalence of CAM use among a recently diagnosed sample indicates that clinicians should discuss CAM early in the assessment process.

Relationship of Dietary Intake to Gastrointestinal Symptoms in Children with Autistic Spectrum Disorders

BACKGROUND Gastrointestinal (GI) symptoms and abnormalities in stool consistency are frequently reported by parents of children with autism spectrum disorders (ASD). The purpose of this study was to 1) describe dietary intake of a cohort of children with ASD compared with normative data and 2) determine whether GI symptoms and stool consistency are related to dietary intake. METHODS Data from diet diaries of children (3-8 years) with ASD (n = 62) were analyzed by a registered pediatric dietician to compare to RDA standards for total calories, protein, carbohydrate, and fat. Dietary intake was correlated with descriptors of stool consistency using cumulative logistic regression methods. RESULTS Intake of calories, carbohydrates, and fat were in the average range; protein intake was increased (211% of RDA). Reported frequency of GI abnormalities, including abnormal stool consistency (e.g., bulky or loose), was increased (54%). No statistically significant relationships between stool consistency and dietary intake were observed. CONCLUSIONS In this sample, there was a high rate of reported gastrointestinal symptoms, despite lack of medical causes. Intake was adequate for calories and carbohydrates and increased for protein. The children did not exhibit excessive carbohydrate intake. There was no association of nutrient intake to changes in stool consistency.

Classifying the intensity of pediatric cancer treatment protocols: The intensity of treatment rating scale 2.0 (ITR-2)

To develop and validate a method of classifying the intensity of pediatric oncology treatments using four operationally defined categories of treatment intensity.

Aortic morphometry and microcephaly in hypoplastic left heart syndrome

Microcephaly is a marker of abnormal fetal cerebral development, and a known risk factor for cognitive dysfunction. Patients with hypoplastic left heart syndrome have been found to have an increased incidence of abnormal neurodevelopmental outcomes. We hypothesized that reduced cerebral blood flow from the diminutive ascending aorta and transverse aortic arch in the setting of hypoplastic left heart syndrome may influence fetal growth of the brain. The purpose of our study, therefore, was to define the prevalence of microcephaly in full-term infants with hypoplastic left heart syndrome, and to investigate potential cardiac risk factors for microcephaly. We carried out a retrospective review of full-term neonates with hypoplastic left heart syndrome. Eligible patients had documented indexes of birth weight, and measurements of length, and head circumference, as well as adequate echocardiographic images for measurement of the diameters of the ascending aorta and transverse aortic arch. We used logistic regression for analysis of the data. A total of 129 neonates met the criteria for inclusion, with 15 (12%) proving to have microcephaly. The sizes of their heads were disproportionately smaller than their weights (p less than 0.001) and lengths (p less than 0.001) at birth. Microcephaly was associated with lower birth weight (p less than 0.001), lower birth length (p equal to 0.007), and a smaller diameter of the ascending aorta (p equal to 0.034), but not a smaller transverse aortic arch (p equal to 0.619), or aortic atresia (p equal to 0.969). We conclude that microcephaly was common in this cohort of neonates with hypoplastic left heart syndrome, with the size of the head being disproportionately smaller than weight and length at birth. Microcephaly was associated with a small ascending aorta, but not a small transverse aortic arch. Impairment of somatic growth may be an additional factor in the development of microcephaly in these neonates.

Preoperative pulmonary venous obstruction affects long-term outcome for survivors of total anomalous pulmonary venous connection repair

BACKGROUND Early outcomes after repair of total anomalous pulmonary venous connection continue to improve; however, little information is available concerning long-term functional status and quality of life. METHODS One hundred patients underwent isolated total anomalous pulmonary venous connection repair at The Childrens Hospital of Philadelphia between 1983 and 2001. Medical records were reviewed and a standardized questionnaire was administered to guardians of survivors. RESULTS Median age at repair was 15.5 days (range, 1 to 563 days). Overall hospital mortality was 14%, decreasing from 19% before 1995 to 5% after 1995. At 15 years after repair, actuarial survival was 84% and freedom from late death or reintervention for hospital survivors was 85%. At a median follow-up of 5.9 years (range, 0 to 17.7 years) 64% of guardians described their childs overall health as excellent, 27% good, 9% fair, and 0% poor. With regard to school performance, 40% of children were characterized as above average, 29% average, 4% below average, and 27% were in special education classes or had repeated grades. By multivariable logistic regression, the presence of associated chromosomal or noncardiac syndromes and pulmonary venous obstruction were found to be significant factors with regard to parental assessment of both overall health and school performance. CONCLUSIONS The majority of children who undergo isolated total anomalous pulmonary venous connection repair can expect an excellent long-term functional outcome. Factors present before operation, such as pulmonary venous obstruction and associated anomalies, can influence overall health and school performance in the long term.

The Concept of Voluntary Consent

Our primary focus is on analysis of the concept of voluntariness, with a secondary focus on the implications of our analysis for the concept and the requirements of voluntary informed consent. We propose that two necessary and jointly sufficient conditions must be satisfied for an action to be voluntary: intentionality, and substantial freedom from controlling influences. We reject authenticity as a necessary condition of voluntary action, and we note that constraining situations may or may not undermine voluntariness, depending on the circumstances and the psychological capacities of agents. We compare and evaluate several accounts of voluntariness and argue that our view, unlike other treatments in bioethics, is not a value-laden theory. We also discuss the empirical assessment of individuals’ perceptions of the degrees of noncontrol and self-control. We propose use of a particular Decision Making Control Instrument. Empirical research using this instrument can provide data that will help establish appropriate policies and procedures for obtaining voluntary consent to research.

Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease

OBJECTIVES. Mortality rates in pediatric patients with heart disease have decreased dramatically in recent decades, resulting in an increasing number of survivors with morbidities that impact quality of life. The purpose of this study was to assess and compare how heart disease affects the quality of life of the pediatric cardiac patient from the perspectives of the patient, parent, and health care provider. METHOD. Individual focus groups were conducted with children (8–12 years of age) with heart disease, adolescents (13–18 years of age) with heart disease, parents of children with heart disease, parents of adolescents with heart disease, and health care providers of pediatric patients with heart disease. A structured focus group technique was used to develop a list of potential items that might affect quality of life. Participants chose the 5 most important items from the list. These items were then categorized into preidentified dimensions (physical, psychological, social, school, and other). The percentages of the total votes for all items were calculated and distributions of responses across dimensions within group and within dimension across groups were reported and compared qualitatively. RESULTS. Patient and parent groups identified similar items as important. Providers identified different items. The physical limitation item received the largest percentage of total votes in all groups (9%–20%). Analysis across dimension revealed that those items related to the physical dimension received the highest percentage of total votes among all groups (30.2%–51.2%). Analysis within dimension revealed that patients endorsed items in the physical dimension more frequently than parents or providers. Within the psychological dimension, the children selected the fewest items relative to all other groups (7.2% vs 21.3%–37.8%), whereas health care providers endorsed these items more frequently than patients or parents (child or parent of child groups 7.2%–21.3% vs health care provider group 28.8%; adolescent or parent of adolescent groups 29.6% vs health care provider group 37.8%). Differences were noted between the child and adolescent groups in the psychological (child versus adolescent: 7.2% vs 29.6%) and school (child versus adolescent: 11.2% vs 2.1%) dimensions. CONCLUSIONS. Patients and parents generally agreed on how heart disease affects the quality of life of children and adolescents, whereas health care providers had a different opinion.

A revision of the intensity of treatment rating scale: Classifying the intensity of pediatric cancer treatment

We previously developed a reliable and valid method for classifying the intensity of pediatric cancer treatment. The Intensity of Treatment Rating Scale (ITR‐2.0) 1 classifies treatments into four operationally defined levels of intensity and is completed by pediatric oncology specialists based on diagnosis, stage, and treatment data from the medical record. Experience with the ITR‐2.0 and recent changes in treatment protocols indicated the need for a minor revision and revalidation.