K. T. Kip

Prenatal Detection of Congenital Heart Disease in Southern Nevada The Need for Universal Fetal Cardiac Evaluation

Congenital cardiac malformations are common developmental anomalies. In the United States, congenital heart disease is the number one cause of infant mortality from congenital malformations. Prenatal diagnosis of congenital heart defects aids treatment coordination. Our aim was to evaluate prenatal detection of serious congenital heart malformations in Clark County, Nevada.

Diagnosis of Absent Ductus Venosus in a Population Referred for Fetal Echocardiography Association With a Persistent Portosystemic Shunt Requiring Postnatal Device Occlusion

The purpose of this series was to assess the incidence, anatomic variants, and implications of an absent ductus venosus (ADV) in patients referred for fetal echocardiography.

Dextrocardia: practical clinical points and comments on terminology.

Dextrocardia is defined by the authors as a right-sided heart with a base–apex axis directed rightward, resulting from a variation in cardiac development, and not used as a general term indicating any heart in the right chest. Dextrocardia occurs in approximately 0.01% of live births and can be discovered in various clinical settings and at various patient ages. The authors review their experience with dextrocardia, discuss useful clinical points that aid in evaluating complex anatomy, recount the history of dextrocardia terminology, and note the current inconsistent nomenclature.

Hair-Grooming Syncope in Children

Objectives. This report describes children undergoing pediatric cardiology evaluation for syncope in which a hair-grooming trigger was determined as the stimulus. Methods. A review of our database revealed 1525 patients with syncope seen by our program of whom 111 had a hair-grooming trigger determined as a cause. Results. Of the 111 patients, 78% were girls. We found characteristic difference between boys and girls with boys experiencing syncope more during hair cutting whereas girls experienced syncope more during hair combing and brushing. Electrocardiograms and echocardiograms were performed as part of syncope evaluation and no significant abnormalities were found in either test in this patient group. Conclusions. This is the largest reported group of children presenting with syncope that had a hair-grooming trigger. Our data also include the first series of boys with the condition. The hair-grooming trigger appears to stimulate a benign form of neurocardiogenic reflex syncope.

FETAL BRADYCARDIA. A PRACTICAL APPROACH

Fetal bradycardia may herald fetal demise. This article highlights arrhythmic fetal bradycardia rather than bradycardia caused by perinatal distress. We briefly examine the embryonic conduction systems development and physiology and we review the classification, aetiology, evaluation, and approach to fetal bradycardia. Our aim is to provide the clinician with practical information about fetal bradycardia that enlightens causative conditions and aids management.

Simplified Pediatric Electrocardiogram Interpretation

We describe a simplified method for interpreting a pediatric electrocardiogram (EKG). The method uses 4 steps and requires only a few memorized rules, and it can aid health care providers who do not have immediate access to pediatric cardiology services. Most pediatric EKGs are normal. However, both abnormal and normal EKGs should be sent to a pediatric cardiologist for later, confirmatory interpretation.

172 BRAIN NATRIURETIC PEPTIDE: A SENSITIVE MONITOR IN PATIENTS WITH HYPOPLASTIC LEFT HEART SYNDROME.

Introduction Amplified cardiac B-natriuretic peptide (BNP) expression results from ventricular volume or pressure overload. The literature reports limited experience with serial BNP levels in pediatric patients. We report a small series of patients with hypoplastic left heart syndrome (HLH) undergoing serial outpatient BNP monitoring. Objective To identify the clinical usefulness of rapid, serial BNP-level monitoring in patients with complex congenital heart disease. Methods Rapidly available fingerstick BNP levels were obtained in our in-office certified outpatient clinical laboratory. Results take less than 20 minutes. We analyzed 36 BNP values obtained in 12 patients with HLH. Results Age ranged from 2 months to 8 years. Patients were status post stage I, II, or III HLH Norwood palliation, and all were on combination medical therapy, including angiotensin-converting enzyme inhibitors, digoxin, diuretics, and aspirin or Coumadin. Serial BNP levels were normal (20 ± 6 pg/dL) in 8 patients > 4 weeks after cardiac surgery. BNP levels were elevated (range 103-839 pg/dL) during serial evaluation in four patients, two with systemic pressure overload (one with systemic hypertension and one with residual coarctation) and two with volume overload (atrioventricular valve regurgitation [AVR]). Elevated BNP values decreased with adjustments to medical therapy in the patients with hypertension and AVR and by interventional cardiac catheterization in the patient with residual coarctation. Conclusions BNP levels were normal in hemodynamically stable HLH patients. Abnormal BNP levels were a guide to clinical management. Prolonged pathologic loading conditions, especially in those patients with single right ventricles, may lead to ventricular dysfunction, congestive heart failure, and death. Predicting the potential for ventricular dysfunction is preferable to detecting its presence. Validation of the clinical usefulness of rapid, serial BNP level monitoring in patients with complex congenital heart disease warrants further study.

IMPROVEMENT IN EXERCISE CAPACITY IN OVERWEIGHT CHILDREN TREATED IN A LIFESTYLE MODIFICATION PROGRAM.: 299

Background Physical inactivity and decreased exercise capacity have been associated with long-term poor prognosis in terms of morbidity and mortality in overweight children. Objectives To assess changes in exercise capacity in overweight children attending a 12-week lifestyle modification program. Methods This study includes data from 121 children with body mass index (BMI) ≥ 95th percentile who complete a 12-week lifestyle modification program. The program consists of nutrition counseling and medically supervised exercise. BMIs were assessed using the 2000 CDC growth charts. Exercise capacity was estimated by the amount of exercise load (Rockport walking test) and the energy cost of exercise (metabolic equivalents [METs]). All measurements were taken at entry and at the end of the program. Each individual served as his or her own control. Results The group was composed of 50 girls and 73 boys, mean age 11.2 years (range 6-17 years) and ethnic distribution of Hispanic, 43%; Caucasian, 33%; African American, 7%; and other races, 13%. By the end of the program there was significant increase in the Rockport test (19.5 ± 11.9 vs 29.2 ± 9.3, p Conclusions This group of children was successful in achieving a significant improvement in their exercise capacity and BMI Z-score, after participation in a 12-week lifestyle modification program.

157 FAMILIAL HISTORY OF HYPERCHOLESTEROLEMIA AND ALTERED LIPID PANEL IN OVERWEIGHT CHILDREN AND ADOLESCENTS.

Background Serum lipid levels follow familial patterns. Obesity is associated with unfavorable lipid profile and increased cardiovascular risk. Our aim was to assess the relationship of familial history of hypercholesterolemia with the lipid profile in a group of overweight children and adolescents. Methods This study included 104 children and adolescents, with body mass index (BMI) ≥ 95th percentile, whose parents answered a questionnaire to address the familial history of hypercholesterolemia in first- and second-degree relatives. As part of the program, all participants received nutrition counseling and performed exercise under medical supervision over 12 weeks. Fasting blood samples were drawn at the first visit and at the end of the program. T-test was used for statistical analysis. Results There were 50 females and 54 males, mean age: 11.3 years (range 7-17 years), mean BMI Z-score: 2.36 ± 0.32, Hispanic: 52%, Caucasian: 30%, African American: 16%, and other races: 2%. At entry there were 44 patients who have one or more relatives with a positive familial history (Group A) and 60 with a negative history (Group B). Comparison in the lipid panel between groups is shown in the table. Conclusions At entry, the group with a positive familial history showed significantly higher serum levels in the lipid panel than those with a negative history. At the 12th week, initial differences in lipid panel still remained; this could mean that those individuals with a positive familial history require adding to nutrition counseling and medically supervised exercise, another type of intervention to lower their abnormal serum lipid levels.

462 USE OF QUANTITATIVE INSULIN SENSITIVITY CHECK INDEX (QUICKI) IN THE SCREENING OF HYPERINSULINEMIA IN OVERWEIGHT CHILDREN AND ADOLESCENTS.

Background Obesity in children and adolescents is a risk factor for development of type 2 diabetes later in life. Early detection of hyperinsulinemia with a simple but reliable clinical test is highly desirable. Objective To evaluate the Quantitative Insulin Sensitivity Check Index (QUICKI) as a screening tool for hyperinsulinemia in a group of non-diabetic overweight children and adolescents in a clinical setting. Methods This study included 598 children and adolescents with a body mass index (BMI) ≥ 95% percentile, referred to our risk factor reduction program for nutrition and healthy habits counseling. Fasting blood samples, for determination of glucose and insulin levels, were obtained early in the morning, during the first visit to the program. QUICKI was calculate as follows: 1/(log [insulin mIU/L] + log [glucose mg/dL]). The receiver operating characteristic (ROC) curve was constructed using the upper value of the normal range for clinical laboratory values of insulin (17 mIU/L). T test and binary nominal correlation was used for statistical analysis. Results There were 358 boys and 240 girls, mean age: 10.7 years (range: 2-18), mean BMI Z-score: 2.52 ± 0.58, ethnic distribution: Hispanic: 50%, Caucasian: 33%, African American: 11% and other races: 6%. Area under ROC curve was 99% (95% confident interval: 0.987-0.999). The best cutoff value of QUICKI for diagnosis of hyperinsulinemia was 0.317, sensitivity: 97.7%, specificity: 93.0%, positive likelihood ratio: 14.04. Those patients with a QUICKI ≤ 0.317 had statistically significant higher mean fasting insulin serum values than those with a QUICKI > 0.317 (31.8 ± 15.9 vs 10.8 ± 4.4 mIU/L, p < .001). Clinical diagnosis of hyperinsulinemia was highly correlated with hyperinsulinemia diagnosed by QUICKI (Phi coefficient correlation: 0.27, p < .0001). Conclusions In this group of overweight children and adolescents QUICKI showed a high correlation with the clinical diagnosis of hyperinsulinemia. At a cutoff value of ≤ 0.317 in this group of subjects the index had high predictive diagnostic values.