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.     

Evaluation of recent research grant applications to the National Institutes of Health in the area of pediatric nephrology

To assess the National Institutes of Health extramural activity in the area of pediatric nephrology research, the rate of submission of grant applications to the National Institutes of Health and available indicators of the quality of these applications were analyzed. This was accomplished by utilizing the grant application files of the Division of Research Grants' Statistics and Analysis Branch with the help of the Reports, Analysis and Presentation Section. Applications reviewed during the period 1980-1983 (May 1980 to January 1984 Council dates) were evaluated, and data from the 10 Study Sections most frequently involved with the review of nephrology applications were tabulated. Results showed that the number of pediatric nephrology applications submitted was quite low, whereas the approval rate was good for those that were submitted.     

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)     

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.     

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     

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     

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.     

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     

Developing a career in academic paediatrics

Many trainees think about research towards the end of clinical training, but at that stage, it may be too late to get the most from the limited opportunities that remain. Ideally, trainees should consider research early in their career. For some, a career in academic paediatrics may be attractive, in which case following the clinical academic pathway as outlined by the National Institutes of Health Research may be appropriate. For others, research may be part of a wider clinical training. This article discusses a variety of research opportunities available to trainees, and offers advice on a career in academic paediatrics.     

NIH Consensus Development Conference statement: inhaled nitric-oxide therapy for premature infants

Premature birth is a major public health problem in the United States and internationally. Infants born at or before 32 weeks' gestation (2% of all births in the United States in 2007) are at extremely high risk for death in the neonatal period or for pulmonary, visual, and neurodevelopmental morbidities with lifelong consequences including bronchopulmonary dysplasia, retinopathy of prematurity, and brain injury. Risks for adverse outcomes increase with decreasing gestational age. The economic costs to care for these infants are also substantial (estimated at $26 billion in 2005 in the United States). It is clear that the need for strategies to improve outcomes for this high-risk population is great, and this need has prompted testing of new therapies with the potential to decrease pulmonary and other complications of prematurity. Inhaled nitric oxide (iNO) emerged as one such therapy. To provide health care professionals, families, and the general public with a responsible assessment of currently available data regarding the benefits and risks of iNO in premature infants, the Eunice Kennedy Shriver National Institute of Child Health and Human Development, the National Heart, Lung, and Blood Institute, and the Office of Medical Applications of Research of the National Institutes of Health convened a consensus-development conference. Findings from a substantial body of experimental work in developing animals and other model systems suggest that nitric oxide may enhance lung growth and reduce lung inflammation independently of its effects on blood vessel resistance. Although this work demonstrates biological plausibility and the results of randomized controlled trials in term and near-term infants were positive, combined evidence from the 14 randomized controlled trials of iNO treatment in premature infants of ≤ 34 weeks' gestation shows equivocal effects on pulmonary outcomes, survival, and neurodevelopmental outcomes.     

Bronchogenic cyst in a 14-month old boy (a case report).

A patient with a bronchogenic cyst in the mediastinum is presented. This rare cyst was found by chance in a 14-month-old boy suffering from respiratory distress due to partial compression of a bronchus. Ultrasonographic and tomographic examinations revealed a cystic mass measuring 3 x 3 cm in diameter located within the apex of the right lobe of the lung. Bronchogenic cyst should be considered when these signs present in early childhood.     

Duplication cyst of the stomach presenting as hemoptysis.

We report a rare case of a 2-year-old boy with a gastric duplication cyst located in the stomach, presenting with vague abdominal pain, recurrent cough, hemoptysis and a lesion in the left lower lobe of the lung reported as a sequestration. The duplication cyst was attached to the diaphragm and lungs by a narrow tract. The cyst was excised. Subsequent scans of the chest showed regression of the lung lesion. We believe this to be the first case in the literature where the hemoptysis was cured and the pulmonary lesion regressed completely after excision of the duplication cyst of the stomach alone. Pulmonary resection was thus avoided.     

Bronchogenic cyst of the lung mimicking a pulmonary hydatid cyst in a child

Lung bronchogenic cyst may be infected and present with symptomatology and chest radiological findings compatible with an infected lung cyst, including pulmonary hydatid cyst. We describe a case of a 5-year-old girl with a symptomatic solitary mass in the right lower lobe that was initially treated as a hydatid cyst. Eventually, a lobectomy was performed, and a final diagnosis done on pathology showed an infected congenital bronchogenic cyst.     

Instantaneous rates of left ventricular wall motion in infants and children

Summary To assess their usefulness as indexes of left ventricular (LV) function, peak rates of decrease and increase in LV diameter were determined from single-cycle echocardiograms in 136 infants and children using a digitizer linked to a computer. The subjects included 62 normal children, 15 patients with congestive cardiomyopathy (CCM), 29 with aortic stenosis (AS), and 30 with patent ductus arteriosus (PDA). The results were compared with the conventional echocardiographic measurements of fractional change in LV diameter (percent ΔLVD) and mean velocity of circumferential fiber shortening (Vcf). The peak shortening rate (PSR) was directly related to LV diameter (r=0.7; standard error of the estimate [SEE], 17.4 mm/sec, p<0.001). Peak Vcf was directly related to heart rate (r=0.48, SEE 0.48 circumferences per second [circ/sec], p<0.001) and ranged from 0.87 to 3.57 circ/sec. Peak Vcf was decreased in CCM (p<0.001) and was normal in AS and PDA. The peak relaxation rate (PRR) was not significantly correlated with diastolic diameter or heart rate. It was decreased in CCM (p<0.001) and normal in AS and PDA. All patients with CCM had decreased percentages of ΔLVD and eight of ten had decreased mean Vcf. The single-cycle peak rates of shortening and relaxation of the LV wall are influenced by heart rate and LV diameter. We did not find them superior to conventional echographic indexes in distinguishing normals from patients with CCM, AS, or PDA. Supported in part by Grant HL-07190 from the National Institutes of Health, United States Public Health Service, by USPH Grant RR-00188 from the General Clinical Research Branch, National Institutes of Health, and by the Ryan O'Neal Connelly Memorial Fund.     

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.     

Human embryo research

In 1996, a ban on the use of US Department of Health and Human Services funds for research on the creation of human embryos and research that involved the injury or destruction of human embryos was signed into law. This ban was partially reversed in 2000 when the National Institutes of Health announced it would fund selective research on human pluripotent stem cells. Given the potential benefits to society, research using human embryos is an issue that deserves additional consideration. The American Academy of Pediatrics believes that, under certain conditions, research using human embryos and pluripotent stem cells is of sufficient scientific importance that the National Institutes of Health should fund it and that federal oversight is morally preferable to the currently unregulated private sector approach.     

The role of the primary care provider in preventing and treating alcohol problems in adolescents.

Adolescents use alcohol more frequently and heavily than all other illicit drugs combined. Given the myriad health, developmental, and social problems associated with alcohol use, it is not surprising that the American Medical Association's Guidelines for Adolescent Preventive Services recommends that adolescents be asked annually about their use of alcohol, and those who report any use during the past year should be assessed further. However, routine alcohol screening of adolescents in primary care and emergency medical settings is not universally applied. In March 2000, the National Institute on Alcohol Abuse and Alcoholism of the National Institutes of Health and the Agency for Health care Research and Quality jointly sponsored a meeting entitled The Expanding Role of Primary Care in the Prevention and Treatment of Alcohol-Use Disorders. The purpose of the meeting was to bring together researchers, policymakers, clinicians, insurance providers, and medical education specialists to determine the best approaches to increase the involvement of primary care physicians and other health care professionals in screening and intervening for alcohol problems in their patients. The National Institute on Alcohol Abuse and Alcoholism and Agency for Health care Research and Quality believe that the evidence of efficacy for primary care involvement is compelling and are working together to promote the translation of these findings into clinical practice. The following article summarizes what is currently known about adolescent alcohol use and how it can be addressed in primary care settings. It provided the background for the meeting's focus on adolescent issues.     

Research in Perinatal and Neonatal Medicine— A Scientific Vision for Future Decades

In this article, a brief overview is presented concerning potential opportunities for and challenges in developing a neonatal-perinatal research agenda. Over a two year period, the Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, in the USA developed NICHD-Scientific Vision—The Next Decade. An outline of the Vision process and some of the recommendations originating from this are also described.     

Why should there be an NICHD?

In its nearly 5 decades of existence, the Eunice Kennedy Shriver National Institute of Child Health and Human Development has expended $23 billion in conducting and supporting research and translating discoveries to practice. The resulting dramatic impact on peoples' lives and improved health for children and families, chronicled herein, are a testament to the benefits of having this institute at the National Institutes of Health.     

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.