Transseptal left heart catheterization as an aid in the diagnosis of cor triatriatum

Summary A case of cor triatriatum is described in which transseptal left heart catheterization was useful in making the diagnosis by allowing easy access to the accessory chamber for pressure measurements and angiography.     

Transseptal Left Heart Catheterization for a Patient With a Prosthetic Mitral Valve Using Live Three-Dimensional Transesophageal Echocardiography

The images and videos presented in this article illustrate a diagnostic transseptal left heart catheterization for a patient with a prosthetic mitral valve guided by live three-dimensional (3D) transesophageal echocardiography. This method provided high-quality 3D imaging that was useful in guiding transseptal puncture and demonstrating prosthetic valve function during this evaluation. Electronic Supplementary Material The online version of this article (doi: ) contains supplementary material, which is available to authorized users.     

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.     

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     

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     

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     

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     

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     

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.     

Sedation for cardiac catheterization: A controlled study

Summary In a double-blind study we compared the effectiveness of a meperidine-promethazine-chlorpromazine combination (drug A) and a fentanyl citrate-droperidol combination (drug B) as sedatives for cardiac catheterization and angiography. The doses for drug A were meperidine, 1.84 mg/kg; promethazine, 0.46 mg/kg; and chlorpromazine, 0.46 mg/kg; for drug B they were fentanyl citrate, 1.25μg/kg; and droperidol, 62.5μg/kg. Drug A or B was assigned at random to each of 94 patients aged 3 to 34 years admitted for cardiac catheterization and was given intramuscularly 30 minutes before catheterization. Each patient who required additional sedation was given intravenously (IV) one fourth of the original dose of the same medication used for initial sedation. If still further sedation was required, diazepam, 1 to 2 mg IV, was administered. The effectiveness of sedation or need for additional medication in the group who received drug A were not significantly different from those in the group who received drug B: supplemental sedation was required in 8 of 47 (17%) with drug A and 8 of 47 (17%) with drug B. No significant differences between the two groups were noted for the mean values of heart rate, respiratory rate, oxygen consumption, cardiac index, left ventricular end-diastolic pressure, arterial oxygen saturation, pH,Po ₂, andPco ₂ measured during catheterization. No side effects were observed in either group, and all patients had a steady, uneventful recovery. We conclude that the meperidine-promethazine-chlorpromazine and the fentanyl-droperidol combinations were equally effective for precatheterization sedation with the dose used. Supported in part by grants HLB 5885 and HLB 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.     

Cardiac abnormalities in children with hyperthyroidism

Summary The cardiac status of 18 hyperthyroid (HT) children (9 black and 9 white) was evaluated by echocardiography. Mitral regurgitation (MR) was diagnosed clinically in 33% (6 of the 9 blacks). None of the 9 white children had MR. Left ventricular end-diastolic diameter (LVEDD) and volume (LVEDV) did not differ from the predicted normal (PN) based on body surface area and heart rate, except in those with MR where increased LVEDD and LVEDV were noted (p<0.02). LV mass was +1.75 standard deviations (σ) of the PN (p<0.01), due to increased wall thickness or LVEDV. Left ventricular output (LVO) was +0.35σ PN (p=ns); however, when compared to that of normal children, LVO of HT was higher (p<0.001) due to the increased heart rate. Enhanced left ventricular contractility was suggested by increased rate of dimensional change during ejection (peak dD/dt-syst), with a mean value of −11.39 cm/sec as compared to the normal of −9.54 cm/sec (p<0.01). A linear multivariate regression equation differentiated the cardiac status of HT from that of normal children. Following treatment to euthyroid state, MR disappeared in 2 and became less in 4 patients. LVO, LV mass, and peak dD/dt-syst also became less. Significant cardiac changes occur in children with hyperthyroidism, which may be reversible in part after euthyroidism is restored. Supported in part by a grant (RR 305) from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health.     

Access to the Left Atrium for Delivery of Radiofrequency Ablation in Young Patients: Retrograde Aortic vs Transseptal Approach

Reported experience with the transseptal approach to the left atrium for delivery of radiofrequency energy in the young patient is limited. To compare two approaches for radiofrequency ablation (RFA) in the left atrium we reviewed our experience from January 1, 1991, through February 1, 1999, in 154 procedures performed on 136 patients (mean age 12.2 years). The patients were grouped by either the retrograde aortic route (R, n= 30) or the transseptal atrial route (T, n= 106). No significant differences were found in age, weight, height, supraventricular tachycardia cycle length, or electrocardiograph characteristics (manifest vs concealed accessory pathway) between the two approaches. Comparison of the transseptal group to the retrograde aortic group revealed a significant difference in the number of catheters (mean = 4 R vs 3 T, p < 0.0001), total fluoroscopic time (71.3 min R vs 43.0 min T, p= 0.0007), diagnostic fluoroscopic time (40.2 min R vs 16.6 min T, p < 0.0001), ablation fluoroscopic time (44.7 min R vs 25.3 min T, p= 0.019), and procedure time (5.0 hours R vs 4.1 hours T, p < 0.0001). No significant difference was found in success rate, number of radiofrequency applications, or major complication rate. These data suggest that although outcomes and major complication rates are similar for the two groups, the use of fewer catheters and shorter fluoroscopic times warrant consideration of the transseptal atrial approach in young patients.     

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.     

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.     

Bone-mineral density deficits from childhood cancer and its therapy

The growing population of childhood cancer survivors—currently estimated at 1 in 900 young adults aged 15–45 years—underscores the importance of studying long-term complications of oncotherapy. While these patients are returning to the mainstream of life, they carry with them toxicities from prior therapy that may compound or potentiate changes typically seen with the normal aging process. Skeletal toxicities such as scoliosis, craniofacial dysplasia, and limb-length discrepancy are readily apparent. However, others such as osteoporosis and osteonecrosis are silent until they reach advanced stages when attempts at amelioration may be unsuccessful. This review addresses bone-mineral density deficits that may predispose childhood cancer survivors to earlier onset and more severe osteopenia and osteoporosis than the normal population.This study was supported in part by grants P30 CA-21765 and P01 CA-20180 from the National Cancer Institute, a Center of Excellence grant from the state of Tennessee from the National Institutes of Health and by the American Lebanese Syrian Associated Charities (ALSAC)     

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.     

Iliac vein—inferior caval thrombosis after cardiac catheterization in infancy

Summary In a retrospective analysis of 300 children who underwent cardiac catheterization in the first six months of life, iliac vein-inferior caval thrombosis (IVICT) was diagnosed at a later study in 4% (12/300). IVICT occurred in only three (1.2%) of the 244 children catheterized by the cutdown technique but in nine (16%) of the 56 studied by the percutaneous technique. There is no significant relationship between the occurrence of IVICT and age, weight, duration of venous cannulation, systemic arterial oxygen saturation, use of balloon flow-directed and atrial septostomy catheters, or systemic heparinization at the first catheterization. Supported in part by Public Health Service grant T23-HL0719302, project No. 260, Maternal and Child Health Service, and 1-F32-HL05794-02 Presented in part at the Section on Cardiology, 48th Annual Meeting of the American Academy of Pediatrics, San Francisco, California, October 13–18, 1979     

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.     

Horizontal ventricular septum with dextroversion: Hearts with and without aortic atresia

Summary Two hearts with horizontal ventricular septum, dextroversion (situs solitus), ventricular septal defects, and malaligned great vessels are reported. One of the hearts had aortic atresia and the infant died; the other patient had a Fontan-type physiologic correction.Reviewing the literature, the following conclusions are drawn: (a) Hearts with horizontal ventricular septum and those with criss-cross atrioventricular connections may be the result of different degrees of rotation of the ventricular muscle mass. This rotation is not likely to be postseptational but preseptational. (b) Only those hearts with a complete 180° rotation should be called criss-cross hearts. (c) Partial rotation results in a horizontal septum such that the right ventricle is invariably superior, regardless of atrioventricular concordance or discordance, situs solitus or inversus, or dextroversion. (d) Physiologic surgical correction is often possible but has to be tailored to the details of each heart.This paper was supported in part by research grant HL30558-04 from the National Institutes of Health, National Heart, Lung and Blood Institute, Bethesda, Maryland.