24-hour ambulatory ECGs in the detection and management of cardiac dysrhythmias in infants and children

Summary Sixty-four 24-hour ambulatory ECGs (cardioscans) were recorded in 47 patients, 5 days to 24 years of age, using a new commercially available miniature tape recorder and analysis system to evaluate known or suspected dysrhythmias. Only two of the 64 cardioscans were inadequate for interpretation, and the study was based on the 62 that could be interpreted. A dysrhythmia was found in 84% (52/62) and in 48% (30/62) a dysrhythmia was detected on the cardioscan, which had not been present on the previous standard 15-lead ECG.Of 25 cardioscans done to determine the cause of symptoms, symptoms coincided with dysrhythmia in only three; in 13 the symptoms did not occur during the cardioscan although dysrhythmia was recorded; in nine the symptoms did occur but no dysrhythmia was recorded, excluding dysrhythmia as their cause.Twenty-five of 44 cardioscans done to judge the adequacy of medical therapy for a dysrhythmia suggested that the therapy was suboptimal. Changes in therapy based on the cardioscan resulted in improvement in 21 patients and partial or no improvement in four patients.Useful information was detected in five of 13 postoperative cardioscans and in one of three cardioscans performed on children with artificial pacemakers.Of 25 cardioscans showing potentially life-threatening dysrhythmias, 12 showed an increase during sleep of frequency or duration of dysrhythmic episodes, five showed no change during sleep, and eight showed a decrease during sleep.The 24-hour cardioscan is a useful means of detecting and managing dysrhythmias in the pediatric age group.Supported in part by grant HL-07190 from the National Institutes of Health and by Public Health Service grant RR-00188 from the General Clinical Research Branch, National Institutes of Health. Dr. Gillette is recipient of a National Institutes of Health Research Career Development Award HL00571.Presented in part at the 26th Annual Scientific Session of the American College of Cardiology, Las Vegas, Nevada, March 1977     

Unusual roentgenographic presentation of a congenital cystic malformation of the lung

We describe here an infant with a large, solitary, fluid-filled lung cyst and hyperinflation of adjacent lung tissue in the same lobe. The combination of a fluid-filled cyst and ectatic emphysema in the same lobe suggests bronchial collapse and airway obstruction as a contributory mechanism for this unusual roentgenographic presentation of a congenital cystic malformation of the lung.This investigation was supported in part by a grant (RR-81) from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health and by Grant HD-00049 from the National Institutes of Health     

Transseptal left heart catheterization: Experience with a new technique in 520 pediatric and adult patients

Summary A new technique for transseptal left heart catheterization utilizing the Transseptal Introducer Set® in 520 patients is described in detail. The age range of these patients was 3 months to 40 years with 30% of the patients less than 2 years old and 28% less than 10 kilograms in weight. The safety and advantages of the technique are emphasized. The introducer set and new technique make transseptal left heart catheterization a safe, versatile, and dependable approach to all areas of the left heart, particularly in infants and children with complex congenital heart lesions.Supported in part by grant HL-07190 from the National Institutes of Health, United States Public Health Service and by grant RR-00188 from General Clinical Research Branch, National Institutes of Health.Presented in part at 52nd Scientific Session of American Heart Association, November 12–15, 1979.     

Comparison of the cardiovascular action of isoproterenol,dopamine, and dobutamine in the neonatal and mature dog

Summary To determine if there are age-related differences of cardiovascular responses to isoproterenol, dopamine hydrochloride, and dobutamine hydrochloride, we recorded cardiac output, renal artery blood flow, central aortic blood pressure, and heart rate in 11 anesthetized puppies ranging in age from 0 to 10 days and in five adult dogs during incremental infusion of isoproterenol (0.05 to 1.25μg/kg/min), dopamine (2 to 50μg/kg/min), and dobutamine (2 to 50μg/kg/min). Isoproterenol decreased systemic arterial mean blood pressure and renal blood flow more in adult dogs than in puppies. Cardiac output was increased in both age groups, but the increase was more marked in adults than in puppies. Heart rate increased similarly in puppies and adults. Dopamine increased systemic arterial mean blood pressure, heart rate, renal blood flow, and cardiac output in both puppies and adult dogs, but the increase of cardiac output was more marked in adult dogs than in puppies. Dobutamine significantly increased systemic arterial mean blood pressure, cardiac output, and renal blood flow in adult dogs but not in puppies. Heart rate increased in both groups of dogs. This study demonstrates age-related differences in the response of the cardiovascular system of anesthetized dogs to isoproterenol, dopamine, and dobutamine. This material was developed by the Section of Myocardial Biology of the National Heart and Blood Vessel Research and Demonstration Center, Baylor College of Medicine, a grant-supported research project of the National Heart, Lung, and Blood Institute, National Institutes of Health, grant HL 17269 and contract HV-52998. Supported in part by grant HL-5756 from the National Institutes of Health. Dr. Driscoll is recipient of Young Investigator's Award 1-R23 H622309-01 from the National Heart, Lung, and Blood Institute. Dr. Gillette is recipient of National Institutes of Health Research Career Development Award HL00571. Presented at the Scientific Sessions of the American Academy of Pediatrics, Section of Cardiology, New York, New York, November 1977     

Demonstration of vestibular implantation of ectopic ureters on an excretory urogram

The authors describe a patient in whom they demonstrated ectopic vestibular ureteral implantation on excretory urography. They indicate that this simple rechnique should be fully exploited before doing vaginograms or other more exotic examinations.Academic Trainee in Diagnostic Radiology. Supported by grant GM 1707 of the National Institute of General Medical Sciences, National Institutes of Health, USPHS.     

Intestinal tuberculosis in a child in an affluent society

A rare case of intestinal tuberculosis occurring in a child in an affluent society is described. The radiographic appearances of the terminal ileum resembled those previously stated to be pathognomonic of Crohn's Disease.Academic Trainee in Diagnostic Radiology. Supported by grant GM 1707 of the National Institute of General Medical Sciences, National Institutes of Health, USPHS.     

Involvement of the aortic valve cusps in discrete subaortic stenosis

Summary A review of 18 cases with discrete suboartic stenosis (DSS) revealed that involvement of the aortic valve cusps in the basic process was present in 16. In these 16 cases, extensions of fibroelastic tissue from the site of the DSS toward the base or superior to the base of one or more cusps were seen. In seven of the involved cases, deformity of a cusp was associated with these extensions. Valvular aortic insufficiency in cases with DSS might be caused by extensions of the fibroelastic tissue to the aortic cusps. This study was supported by research grant 5 RO1 HL05694 from the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, and by the Dwan Family Fund.     

Developmental delay in infants with congenital heart disease

Summary The mental and motor development of 173 infants with congenital heart disease was assessed by means of the Bayley Scales of Infant Development and clinical neurological examinations. The relationship between age, sex, congestive heart failure, hypoxemia, hospitalization, and test results was evaluated. The presence of congestive heart failure was found to be significantly associated with both mental and motor developmental delay. Hypoxemia and hospitalization were associated with delayed motor development. Developmental delay could be recognized as early as 2 months of age. Supported in part by training grant HL 05855 and program project grant HL 10436 from the National Institutes of Health, Bethesda, Maryland     

Inducible polymorphous ventricular tachycardia following Mustard operation for transposition of the great arteries

Summary A 22-year-old woman with chronic atrial tachycardia following Mustard's operation for transposition of the great arteries presented with dizziness and ventricular tachycardia documented with dynamic 24-h electrocardiogram. During intracardiac electrophysiology study, programmed ventricular extrastimulation induced polymorphous ventricular tachycardia (torsades de pointes). This was prevented by intravenous administration of procainamide. We postulate that polymorphous ventricular tachycardia is a possible cause of death in patients with Mustard's operation. Postoperative electrophysiologic study may define those patients at risk to develop this potentially fatal arrhythmia.This work was supported in part by Institutional Training grant HL 07387 and research grants HL 18794 and HL 23566 from the National Institutes of Health, Bethesda, Maryland, and a grant from the Bane Estate, Chicago, Illinois.     

Recurrent Kawasaki disease

Summary This case report describes a boy who had Kawasaki disease (KD) at age 12 months and had a recurrence one year later. The coronary arteries were normal following the initial episode; however, during the second episode he developed coronary aneurysms. Gallium-67 radionuclide imaging, echocardiography, and angiography were used to diagnose the coronary abnormalities.This work was supported in part by grant RR-00188 from the General Clinical Research Branch of the National Institutes of Health, Bethesda, Maryland.     

Direct entry of the right superior vena cava into the left atrium with aneurysmal dilatation and stenosis at its entry into the right atrium with stenosis of the pulmonary veins: A rare case

Summary This is the first autopsied case reported in the English language literature wherein the right superior vena cava entered both atria with obstruction of the entry into the right atrium, aneurysmal dilatation of the proximal part of the superior vena cava, and entry of the stenosed right upper pulmonary vein into the aneurysmal sac. The aneurysmally dilated right superior vena cava communicated directly with the left atrium. In addition, the remainder of the pulmonary veins, all of which were markedly stenosed, entered the left atrium. The embryogenesis of this unique malformation is briefly discussed. Aided by grant HL 30558-01 from the National Heart, Lung and Blood Institute of the National Institutes of Health, Bethesda, Maryland.     

Study of the conduction system in a population of patients with sudden infant death syndrome

Summary The conduction system of 23 infant hearts, 15 of sudden infant death syndrome (SIDS) and eight of those dying from known cause, was serially sectioned. A left-sided His bundle was found more commonly in (SIDS) (eight of 15) than in the controls (two of eight). Taking into account a previous study in which a left-sided His bundle was found in only four of 32 hearts from all age groups, this is statistically significant and may be a factor promoting SIDS.This research was aided by grant HL-30558-02 from the National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland.     

Congenital aortico-left atrial tunnel

Summary A newborn baby with heart failure had ventricular septal defect and aorto-left atrial communication. Aortography demonstrated a large funnel-shaped vessel originating from a dilated left aortic sinus and opening into the base of the left atrial appendage. Open heart surgery was unsuccessful. Histological examination (750 serial sections) of the vessel including the adjoining aorta and left atrial appendage showed no evidence of coronary artery structures. To our knowledge, this represents the first case of congenital aortico-left atrial tunnel.Supported in part by grant RR-305 from the General Clinical Research Centers Program of the Division of Research Resources and by grant HE-07605-17 from the National Heart Institute, National Institutes of Health, Bethesda, MarylandDr. Lev is a Career Investigator and Educator of the Chicago Heart Association.     

Distance of upper pole calyx to spine and lower pole calyx to ureter as indicators of parenchymal loss in children

The authors analyzed the excretory urograms of 177 female children whose ages ranged from infancy to eleven years. The purpose of the study was to discover whether the upper pole calyx moves closer to the spine and the lower pole calyx moves closer to the ureter in the presence of calyceal clubbing and parenchymal scarring. This study confirms these observations but shows that considerable overlap occurs between normals and abnormals. This overlap is greatest in the younger age group and in the lower pole calyx to ureter measurements.Academic Trainee in Diagnostic Radiology. Supported by grant GM 1707 of the National Institute of General Medical Sciences, National Institutes of Health, USPHS.     

Increasing pediatric obesity in the United States

Data from four national surveys indicate pronounced increases in the prevalence of pediatric obesity in the United States. Obese children are defined as those with triceps skinfolds greater than or equal to the 85th percentile of children of the same age and sex in 1963 to 1970. Superobese children are those with skinfolds greater than or equal to the 95th percentile. Compared with skinfold data from the 1963 to 1965 National Health Examination Survey, cycle 2, skinfold data from the second National Health and Nutrition Examination Survey, conducted from 1976 to 1980, indicate a 54% increase in the prevalence of obesity among children 6 to 11 years old and a 98% increase in the prevalence of superobesity. Compared with skinfold data from the 1966 to 1970 National Health Examination Survey, cycle 3, skinfold data from the second National Health and Nutrition Examination Survey indicate a 39% increase in the prevalence of obesity among children 12 to 17 years old and a 64% increase in the prevalence of superobesity. Increases occurred among children of all ages and both sexes and for both blacks and whites. Blood pressure data from the four surveys suggest that the share of pediatric hypertension associated with obesity has increased. Such rapid increases in obesity indicate that environmental causes are likely responsible.     

Systemic to pulmonary arterial shunts for patients with cyanotic congenital heart disease: Current status

The various central vascular shunting operations used for palliation of patients with congenital heart disease plus decreased pulmonary blood flow are discussed. The status of each in current practice is outlined in order to provide an up-to-data reference for the pediatric radiologist. In general, the Waterston shunt is preferred for infants under six months and the Blalock-Taussig anastomosis for patients older than this.Supported by: Grant 260, Children's Bureau, Maternal and Child Health Service, Department of Health, Education and Welfare, Washington, D. C.; and grants from the National Heart and Lung Institute of the National Institutes of Health, the John A. Hartford Foundation, Inc., and the Max Kade Foundation, Inc.     

Paradoxical relationship between mitral valve prolapse and left ventricular function in Marfan's syndrome

Summary A child with Marfan's syndrome had cardiac failure during infancy. Aortic sinus aneurysms, mitral prolapse with severe regurgitation, and atrial septal defect were present. After myocardial infarction at age 4 years, the mitral prolapse became less and the regurgitation disappeared. After recovery of left ventricular function, mitral regurgitation reappeared. The unusual relationship between mitral valve and left ventricular function could be related to redundant chordae and leaflet. Myocardial infarction occurs in Marfan's syndrome even during childhood.Supported by grant RR-305 from the General Clinic Research Centers Program of the Division of Research Resources, National Institutes of Health, Bethesda, Maryland     

Hypoplastic left heart syndrome with dysplastic pulmonary valve with stenosis

Summary A dysplastic pulmonic valve in hypoplasia of aortic tract complex has not been dealt with in the literature. The recent attempted surgical correction for hypoplasia of aortic tract complex has given some hope of survival in this entity. The integrity of the tricuspid and pulmonic valves is important while considering total surgical correction. We describe three hearts of hypoplasia of aortic tract complex with dysplastic pulmonic valve. The echocardiographic recognition and the significance of the dysplastic nature of the pulmonary valve in the light of surgery are emphasized. Aided by grant HL 30558-02 from the National Heart Lung and Blood Institute of the National Institutes of Health, Bethesda, Maryland.     

Pulmonary excretion of carbon monoxide in the human infant as an index of bilirubin production

A total of 45 infants, including 20 appropriate-size-for-gestational-age infants (AGAs), 19 large-size-for-gestational-age infants (LGAs) and 6 infants of diabetic mothers (IDMs), had determinations of their pulmonary excretion rate of carbon monoxide (Ve_CO) in the first postnatal week as an index of bilirubin production. We calculated a ratio (Rw) of birth weight to ideal weight (50th percentile for gestational age) as a relative measure of infant size. We also measured maternal glycosylated hemoglobin (Hb A_Ic) in the postpartum period as a reflection of the time-integrated blood glucose level over the weeks preceding delivery. Mean values for maternal Hb A_Ic in the postpartum period, infant Rw, and Ve_CO were all significantly increased for the LGAs and IDMs compared to the normal AGAs. Nine LGAs had mothers whose Hb A_Ic levels were >2 S.D. higher than the mean Hb A_Ic level for mothers of normal AGAs. The infants whose mothers had the highest Hb A_Ic levels were not always the ones with the highest bilirubin production rates. These findings suggest that maternal Hb A_Ic in the postpartum period, infant size, and bilirubin production are associated phenomena, but that a postpartum time-integrated measure of blood glucose level over the weeks preceding parturition may not reflect changes in other associated factors which can affect infant erythropoiesis. The LGAs are not a homogeneous group, and some may have mothers with missed abnormalities of gestational glucose metaoblism.This investigation was supported in part by grants from the General Clinical Research Program of the Division of Research Resources, National Institutes of Health (RR-00081), the Thrasher Research Fund, the United States Public Health Services (AM-25603), the National Institutes of Health (HD-14426), and by the National Institutes of Health Biomedical Research Support Grant (5S01RR05583)     

Clinical pharmacology research in the pediatric patient: the challenge continues

For most of the 20th century, most drugs labeled by the United States Food and Drug Administration (USFDA) have not been adequately studied in the pediatric population. This lack of data has necessitated the continued dependence of practitioners on sub-optimal prescribing data placing pediatric patients at great risk of serious therapeutic misadventures. Recently, the USFDA has enacted and begun to enforce the Final Rule of 1997 which became effective on 1 April 1999. This rule is the culmination of the persistent efforts of numerous professional organizations, clinicians, academicians, the USFDA and others, to ensure the ready availability of appropriate data for medications intended for or that will be used in children. Unlike the 1994 Rule which voluntarily required pharmaceutical manufacturers to submit pediatric data, the Final Rule mandates submission of such data and, most importantly, empowers the USFDA to afford incentives and penalties for non-compliance including possible removal of already marketed products. This overview addresses many of the important components which must be included in the performance of a comprehensive clinical pharmacologic evaluation serving as the foundation for optimal dosing across the broad age range encompassing pediatric practice. Furthermore, the possible risk and/or benefits of the study must be reasonably defined prior to undertaking the study and clearly shared with the patient's caregivers. Consent should always be obtained from the caregiver and, when appropriate, assent obtained from the underage child. To facilitate such clinical investigations and to foster collaborative efforts with innovators and clinical research programs, the National Institutes of Health through the National Institute of Child Health and Human Development of the NIH established a network of Pediatric Pharmacology Research Units. These units have worked closely together and with other pediatric research centers to facilitate USFDA labeling of a number of commonly used medications. All of these very positive efforts highlight the many challenges that remain for the pediatric investigator and practitioner while underscoring the very positive environment in support of these efforts.