bluuebunny/crossref_metadata_2025 huggingface dataset:
Abbreviated combined anatomical/electrophysiological approach for catheter ablation of atrioventricular nodal reentrant tachycardia in children
Authors: Bertram Harald , Windhagen-Mahnert Britta , Bökenkamp Regina , Kriebel Thomas , Peuster Matthias , Hausdorf Gerd , Paul Thomas
Published: August 2006

Atrioventricular nodal reentrant tachycardia was proven during electrophysiologic study in 41 children, aged from 3.7 to 16 years, who were referred for catheter ablation of symptomatic supraventricular tachycardia. Using an abbreviated combined anatomical and electrogram-guided approach for selective ablation of the slow pathway, a steerable ablation catheter was placed at the inferior region of the vestibule of the tricuspid valve close to the orifice of the coronary sinus, with the intention of recording a multicomponent local atrial electrogramm during sinus rhythm. If application of radiofrequency current of 500 kHz at 70°C at this site did not result in a slowly accelerated junctional rhythm, at a rate of less than 120 beats per minute, the catheter was stepwise advanced up to a position midway towards the apex of the triangle of Koch for additional applications of energy. Ablation was achieved in 35 of the patients. In 6 patients, the slow pathway was modulated such that the tachycardia could no longer be induced. The number of applications of energy ranged from 1 to 19, with a median of 6 applications. The mean period of fluoroscopy was reduced to 15.6 (4.3 to 39.8) minutes, while the overall duration of the catheterization procedures ranged from 88 to 280 (mean 173.2) minutes. In none of the patients did we observe permanent high grade atrioventricular block. During follow-up over a mean of 4.1 years, two patients had recurrence of tachycardia, corresponding to a 95% rate of success in the midterm. We conclude that selective radiofrequency ablation of the slow pathway using the abbreviated anatomical and electrophysiological approach is a safe and curative therapeutic approach in children with atrioventricular nodal reentrant tachycardia. Periods required for fluoroscopy can be significantly reduced, and mid-term results are excellent.
Transcatheter closure of various types of defects within the oval fossa using the double umbrella device (CardioSEAL) – feasibility and echocardiographic follow-up
Authors: Kaulitz Renate , Peuster Matthias , Jux Christian , Paul Thomas , Hausdorf Gerd
Published: August 2006

Data on long-term follow-up for closure of so-called secundum type“ atrial septal defects within the oval fossa using recently developed devices are limited, and results focused on presence of residual shunting. The purpose of our study was to report the experience from a single center establishing the effectiveness of transcatheter closure in patients with various types of defect other than those located centrally within the oval fossa.

A total of 72 patients was included in this study. On transesophageal echocardiography, the size of the defects varied from 6 to 18 mm, with estimation of the stretched diameter from 11 to 21 mm. The ratio of stretched diameter to the extent of the residual septum ranged from 0.28 to 0.54. Mean follow-up was 30.5+ 7.4 months, with a range from 13 to 42 months. The rate of closure using devices with diameters from 28 to 40 mm increased from 80% immediately after implantation to 93% in the 57 patients examined 24 months after implantation. For further analysis, we compared the 44 patients with a solitary, centrally located, defect to 28 having morphological variations, including superiorly located defects with deficient superior and aortic rims, multifenestrated and aneurysmal defects, or isolated additional defects. There was no incidence of formation of thrombus, sustained atrial arrhythmia, or infective endocarditis. Residual shunting was not influenced by location or morphology of the defects, but increased with size, stretched diameter, and the ratio of pulmonary to systemic flows. Serial transthoracic echocardiographic findings revealed malposition of one right-sided superior arm of the device in 8 patients, while protrusion of one leftsided arm onto the right atrial aspect was observed in 3 patients. Fluoroscopy showed fatigue fracture of a single arm in 7 patients (9.7%) within the first 6 months after implantation.

These results demonstrate that transcatheter closure with the non self-centering double umbrella device was effective and safe on medium-term follow-up, and could be extended to defects within the oval fossa having various morphologies. Residual shunting resolved with time, and was not related to either morphology or the position of the device.
Ectopic atrial tachycardia due to aneurysm of the right atrial appendage
Authors: Mizui Sawa , Mori Kazuhiro , Kuroda Yasuhiro
Published: August 2006

We report an infant with ectopic atrial tachycardia, due to an aneurysm of the right atrial appendage, who developed congestive heart failure. Although catheter ablation was transiently successful, tachycardia recurred 2 days later. The aneurysm of the right atrial appendage was resected successfully by surgery, and thereafter she did well, reverting to normal sinus rhythm.
Late cardiac tamponade after transcatheter closure of atrial septal defect with Cardioseal® device
Authors: Pinto Fátima F. , Sousa Lídia , Fragata José
Published: August 2006

Cardiac tamponade occurring late after interventional closure of defects within the oval fossa is a very rare but life-threatening complication. We describe such an occurrence after use of a Cardioseal device to close an interatrial communication. Two arms of the device had perforated left atrial wall. The device was removed at surgery, and the defect closed uneventfully. All available means should be used to identify this complication.
Takayasu disease masquarading as interruption of the aortic arch in a 2-year-old child
Authors: Ladhani S. , Tulloh R. , Anderson D.
Published: August 2006

Takayasu disease is a chronic, progressive inflammatory vasculitis of large and medium-sized vessels, which commonly presents in adulthood. This case report describes a 2 year old girl who presented with acute heart failure and complete occlusion of the aortic arch and was subsequently diagnosed with Takayasu disease. As far as we can determine, this is the first report of such an acute and atypical presentation of Takayasu disease at such a young age.
Feasibility of transcatheter closure of multiple defects within the oval fossa
Authors: Ewert Peter , Berger Felix , Kretschmar Oliver , Abdul-Khaliq Hashim , Stiller Brigitte , Lange Peter E
Published: August 2006

Background: Multiple perforations in the floor of the oval fossa may be an obstacle for transcatheter closure. Thus, we analyzed the interventions in 33 patients with more than one interatrial communication in comparison with 370 procedures with a single defect. Methods and Results: A diagnostic catheterization, which included a balloon-sizing maneuver, was performed. We implanted a total of 46 occluders, made up of 42 Amplatzers and 4 CardioSEALs. In 20 patients, the defects were closed with a single occluder, namely 18 Amplatzer and 2 CardioSEAL devices. Complete closure was achieved in 15 patients, while a tiny residual shunt remained in 5 patients. In 13 patients, two devices were implanted, without any residual shunt being found immediately after implantation. In 3 patients, the occluders did not touch each other. In 10 patients, their rims overlapped. In comparison with the control group, the group with multiple defects did not differ in the distribution of age, gender, and indications for device closure. The mean time of the procedure, and the time required for fluoroscopy, however, were significant longer (P< 0.001). These times ranged from 45 to 250 minutes with a median of 140 minutes, and from 0.0 to 39.2 minutes, with a median of 12.0 minutes, respectively. Also, the association with an atrial septal aneurysm was significantly more frequent 61 vs. 17%; P< 0.001). The times taken during insertion of double devices were also significantly longer than those needed for insertion of a single device (P< 0.001). Conclusions: Transcatheter closure of multiple defects within the oval fossa is feasible with currently available occluders, albeit than, in selected cases it is necessary to implant two devices.
The bronchial cast syndrome after the Fontan procedure: further evidence of its etiology
Authors: McMahon Colin J. , Nihill Michael R. , Reber Adele
Published: August 2006

We describe a three-year-old boy who presented with recurrent expectoration of bronchial casts six months following creation of a fenestrated lateral tunnel Fontan circulation for pulmonary atresia with intact ventricular septum. Cardiac catheterization demonstrated elevated central venous pressure with two areas of stenosis within the Fontan circuit, specifically at the junction of the right superior caval vein and the right pulmonary artery, and between the atrial baffle and the right superior caval vein. Insertion of Palmaz stents in these areas resulted in a reduction in central venous pressure, and a transient reduction in production of casts. Eight weeks after catheterization, however, he produced further casts, which resulted in hypoxia, respiratory arrest and death. We reviewed the autopsied specimens obtained from patients with the Fontan circulation over an eleven-year period at our institution in order to ascertain the prevalence of subclinical production of bronchial casts. We found no casts in the thirteen patients examined. Hemodynamic assessment is vital in all patients who develop this syndrome, and should be the primary focus of investigation, rather than solely directing efforts at lysis of casts.
The role of tissue plasminogen activator in the successful treatment of infected cardiac thrombus in children
Authors: Zalzstein Eli , Zucker Nili , Levitas Aviva
Published: August 2006

Infected cardiac thrombus is rare in children, with antibiotic or antifungal agents used as the first line of treatment. Persistence is an indication for surgical intervention. We describe two children who were treated successfully with a combination of antibiotic and antithrombotic agents. Use of antithrombotic agents promotes degeneration of fibrin, thus reducing the mass and facilitating the diffusion of the antibiotic and/or antifungal agents.
Patterns of pulmonary venous flow in the fetus with disease of the left heart
Authors: Crowe David A. , Allan Lindsey D.
Published: August 2006

A restrictive atrial septal defect is a risk factor for mortality in some forms of left heart disease, and can be predicted from the patterns of flow in the pulmonary veins in fetal life. Our objective was to document the patterns of pulmonary venous flow of blood in different forms of left heart disease in the fetus. The pulmonary venous flow profile was documented in 61 cases. The systolic, diastolic, and reverse waves were measured and compared to normal. Profiles of venous flow suggestive of restriction at the atrial septum were found in 32 of 61 cases using the ratio of systolic to diastolic waves, and 26 cases using the height of the reverse wave.

A restrictive atrial septal defect was found most commonly in fetuses with critical aortic stenosis or the hypoplastic left heart syndrome, and was less frequently seen in the setting of mitral atresia or coarctation. In order to plan appropriate postnatal management, examination of the profiles of pulmonary venous flow is an essential part of the evaluation of any form of left heart disease detected in the fetus.
Elevations of Troponin I after interventional cardiac catheterization
Authors: Kannankeril Prince J. , Wax David F. , Pahl Elfriede
Published: August 2006

Background: Elevation of cardiac troponin I in the serum is a specific marker for myocardial injury. We measured levels of troponin I in the serum in children before and after cardiac catheterization to determine if this procedure was associated with an increase in levels of troponin. Methods: We enrolled patients under 21 years of age undergoing cardiac catheterization at our institution. A baseline sample of serum was drawn at the start of the procedure. Repeat samples were obtained immediately after, and six hours subsequent to the procedure. All samples were analyzed for cardiac troponin I using the Abbott AxSYM microparticle immunoassay system. Levels were considered normal (0–0.4 ng/ml) or elevated (>0.4ng/ml). Patients were excluded if the baseline level was elevated. Results: Levels of cardiac troponin I were elevated in the serum from 11 of 14 (79%) cases immediately after the procedure (p < 0.0001), and in 12 of 14 (86%) six hours later (p < 0.0001). Only 2 patients had recognized complications potentially causing myocardial injury. Conclusion: Levels of cardiac troponin I increase in the serum in a high proportion of children after cardiac catheterization. These elevations can be observed immediately, and are maintained for at least six hours. Our study suggests that cardiac catheterization, predominantly intervention, is associated with myocardial injury, even in the absence of complications.
Optimal surgical approach for repair of aortopulmonary window
Authors: Hew Chee-Chin , Bacha Emile A. , Zurakowski David , del Nido Pedro J. , Mayer John E. , Jonas Richard A.
Published: August 2006

Background: This is a review of the experience over 26 year in a single institution with surgical repair of aortopulmonary window. Methods: Between July 1973 and March 1999, 38 patients underwent surgery for aortopulmonary window at a median age of 5 weeks, and with a median weight of 3.9 kg. Median follow-up was 6.6 years, with a range from 0.8 to 26 years. Additional defects were present in 25 (65%) patients, including interruption of the aortic arch in 7, tetralogy of Fallot in 7, ventricular septal defect in 5, functionally univentricular anatomy in 3, aortic coarctation in 2, and anomalous origin of a coronary artery in 1. We approached via an aortotomy in 45%, an incision through the defect in 31%, and using a pulmonary arteriotomy in 24% of patients. Closure was achieved using a single patch in 30 patients (79%). Results: There were 3 (7.9%) in-hospital deaths. Actuarial patient survival was 88% at 10 years. Three patients required reinterventions for stenoses of the great arteries. Freedom from any reintervention was 70% at 10 years. By multivariate analysis, the approach through a pulmonary arteriotomy was shown to result in a higher need for reintervention (p = 0.01). Conclusions: Repair of aortopulmonary window can be done with excellent results. A pulmonary arteriotomy should be avoided.
Repair of double outlet right ventricle with doubly-committed ventricular septal defect
Authors: Uemura Hideki , Yagihara Toshikatsu , Kadohama Takayuki , Kawahira Youichi , Yoshikawa Yoshiro
Published: August 2006

Objective: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. Methods: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. Results: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117 ± 68 months. Catheterization carried out 16 ± 6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16 ± 7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Conclusion: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.
The pulmonary vasculature in a neonatal porcine model with increased pulmonary blood flow and pressure
Authors: Stenbøg Elisabeth V. , Steinbrüchel Daniel A. , Thomsen Anne Bloch , Baandrup Ulrik , Heickendorff Lene , Ingerslev Jørgen , Andersen Niels Trolle , Emmertsen Kristian
Published: August 2006

Introduction: Hypertension and hyperperfusion of the pulmonary vascular bed in the setting of congenital cardiac malformations may lead to progressive pulmonary vascular disease. To improve the understanding of the basic mechanisms of this disease, there is a need for clinically relevant animal models which reflect the disease process. Material and Results: We randomly allocated 45 newborn pigs, at the age of 48 hrs, to groups in which there was either construction of a 3 mm central aorto-pulmonary shunt, undertaken in 9, or ligation of the left pulmonary artery, achieved in 13. Controls included sham operations in 13, or no operations in 10 pigs. Follow-up was continued for three months. The interventions were compatible with survival in most pigs. The shunts resulted in an acute 85% increase in systolic pulmonary arterial pressure, and a more than twofold increase in pulmonary blood flow. By three months of age, nearly all shunts had closed spontaneously, and haemodynamics were normal. Ligation of the left pulmonary artery resulted in a normal total pulmonary blood flow, despite only the right lung being perfused, and a 33% increase in systolic pulmonary arterial pressure. These haemodynamic changes were maintained throughout the period of study. In both groups, histomorphometry revealed markedly increased muscularity of the intra-acinar pulmonary arteries. Circulating levels of endothelin were normal in the shunted animals, and elevated in those with ligation of the left pulmonary artery. Conclusion: In neonatal porcine models of pulmonary vascular disease, created by construction of 3 mm central aorto-pulmonary shunts and ligation of one pulmonary artery, we observed histopathological changes of the pulmonary vasculature similar to early hypertensive pulmonary vascular disease in humans. Elevated circulating levels of endothelin were associated with abnormal haemodynamics rather than abnormal pathology. These findings could be valuable for future studies on the pathogenesis of hypertensive pulmonary vascular disease associated with congenital cardiac malformations.
Factors associated with self-perceived state of health in adolescents with congenital cardiac disease attending paediatric cardiologic clinics
Authors: Kendall Lynne , Lewin Robert J. P. , Parsons Jonathan M. , Veldtman Gruschen R. , Quirk Joanne , Hardman Geoffrey F.
Published: August 2006

The purpose of our study was to determine the ways in which adolescents with congenital cardiac disease believed that the condition had affected their life, and how these views were related to their perceived health. Interviews were conducted with a series of 37 adolescents, 17 girls and 20 boys, aged from 11 to 18, as they attended the clinics of 4 paediatric cardiologists in a teaching hospital in the United Kingdom. Transcripts of the interviews were analysed for recurring themes. A questionnaire was formed consisting of a set of questions for each theme, and additional items eliciting “perceived health”, and administered to a second series of 74 adolescents, 40 boys and 34 girls, who were again aged from 11 to 18 years. Slightly less than half (46%) perceived their health as either “good” or “very good”, and one-third (33%) rated it as “average”. The majority (66%) felt themselves to be “the same” as, or only very slightly “different” from, their peers. The assessment of the seriousness of their condition by the adolescents, the degree to which they saw themselves as different from others, and their perceived health, were not related to the “complexity of the underlying medical condition” as rated by their physician. It was the psychosocial themes, such as exclusion from activities or the effect of the condition on relationships, that were most strongly related to the perception of their health by the adolescents. Improved education of parents, teachers and peers, and attendance at classes for cardiac rehabilitation, might help to ameliorate some of these problems.
Massive pericardial effusion with left-to-right intracardiac shunt
Authors: Edwards A. J. , Ring N. J. , Marshall A. J.
Published: August 2006

We describe a 31-year-old man with Down's syndrome who presented with severe chronic hypothyroidism and a massive pericardial effusion. Following partial aspiration of this effusion, he rapidly deteriorated and died. Findings at autopsy revealed him to have an atrioventricular septal defect with shunting at the atrial level. We postulate that, by releasing extrinsic pressure on his right heart by the aspiration, there was sudden shunting of blood from left-to-right, resulting in hypotension, shock, and subsequent death. We highlight the difficulties in management of such a case, and suggest alternative strategies.
Isolated noncompaction of ventricular myocardium
Authors: Elshershari Huda , Okutan Vedat , Çeliker Alpay
Published: August 2006

Isolated noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The diagnosis can be made echocardiographically, and the entity may be associated with problems of cardiac rhythm. We describe two illustrative cases, with Wolf-Parkinson-White syndrome in one, and left bundle branch block in the other. We emphasize that children with problems of rhythm and findings suggestive for left ventricular cardiomyopathy, either hypertrophic or dilated; should be investigated to exclude isolated noncompaction of the ventricular myocardium.
Hemorheology and renal function during cardiopulmonary bypass in infants
Authors: Dittrich Sven , Priesemann Max , Fischer Thomas , Boettcher Wolfgang , Müller Christian , Dähnert Ingo , Ewert Peter , Alexi-Meskishvili Vladimir , Hetzer Roland , Lange Peter E.
Published: August 2006

Background: Acute renal failure is an occasional complication after cardiopulmonary bypass in infants. Whereas it is well known that postoperative hemodynamics inflict acute renal failure, the influence of extra-corporeal circulation on the kidney is less clear. Moreover, changes in blood viscosity occur during and after surgery, which may influence renal dysfunction. For this reason, we investigated the impact of blood viscosity on renal function during cardiopulmonary bypass. Methods: In 34 patients weighting less than 10 kg, we performed repeated analysis of urine, blood, and plasma viscosity. Results: Polyuria and proteinuria that appeared during cardiopulmonary bypass indicated an elevated transglomerular filtration gradient, which recovered within 24 hours. The appearance of N-acetyl-β-D-glucosaminidase in the urine, and elevated excretion of sodium, were additionally indicative of mild tubular damage. Elevation of blood viscosity during hypothermic perfusion showed a statistical correlation with proteinuria and N-acetyl-β-D-glucosaminidaseuria. With hypothermia, the relation of blood viscosity to plasma viscosity became stronger, while the relation to the hematocrit decreased compared to normothermia. Conclusions: During cardiopulmonary bypass perfusion, the kidney can be stressed by proteinuria and mild tubular damage. Our data provide evidence that the kidneys can be protected by improved blood viscosity during cardioplegia, but this needs confirmation in a prospective interventional study.
The pathogenesis of heart failure in infants with congenital heart disease
Authors: Buchhorn Reiner , Hammersen Annette , Bartmus Dietmar , Bürsch Joachim
Published: August 2006

Background: The clinical symptoms of heart failure in infants with left-to-right shunts are thought to be explained by well-known hemodynamic disturbances such as pulmonary hypertension and overcirculation, but previous studies have not, thus far, found the expected correlations with hemodynamic and clinical parameters. Based on the neurohormonal model of heart failure, we hypothesised that the clinical symptoms of infants with left-to-right shunts are also related to neurohormonal disorders. Methods: We compared various neurohormonal and hemodynamic parameters measured invasively in 70 infants with left-to-right shunts to the respiratory rate and gain in weight over a corresponding period of time. Heart rate correlated significantly with respiratory rate (r = 0.62***, p < 0.001) and gain in weight (r =−0.31*, p = 0.015), but more conventional measures of severity, such as the ratio of pulmonary to systemic flows, failed to show comparable correlations with clinical symptoms. Respiratory rate was related to levels of norepinephrine (r = 0.47***, p < 0.001) and plasma renin activity (r = 0.65***, p < 0.001). The important impact of autonomic imbalance on respiratory rate was underlined by an analysis of variability of heart rate in 26 infants that showed significantly reduced values for the domains of time and frequency. We were not able to find a conclusive multiple regression model with which to explain the symptom "failure to thrive". Conclusions: A increased heart rate, reduced variability in heart rate, and elevated levels of norepinephrine and renin are significant predictors of clinical symptoms such as tachypnea in infants with congenital cardiac malformations. The neurohormonal hypothesis, in which heart failure is interpreted not only as a hemodynamic derangement but also as a neurohormonal disorder, may be valid for infants with congenital cardiac malformations.
Influence of the introduction of Amplatzer device on the interventional closure of defects within the oval fossa in children
Authors: Rastegari Mahvash , Redington Andrew N. , Sullivan Ian D.
Published: August 2006

Since June 1998, we have used an Amplatzer device whenever considered appropriate in patients with isolated defects within the oval fossa. The aim of this study was to define the total cohort of patients with isolated defects in the oval fossa seen at this hospital, so as to assess the impact of this policy on contemporary management. In the two-year period commencing 1st June 1998, 116 patients older than 6 months were seen with an isolated septal defect within the oval fossa. Mean age at closure or last review was 5.8 years, with a range from 0.5 to 20 years. In total, 42 (36%) patients were assigned to surgical closure, 25 (22%) to closure using an Amplatzer device, and 49 (42%) remained under clinical follow up. Direct referral for surgical closure occurred in 24 (21%) patients, in whom transcatheter closure was considered not appropriate after transthoracic echocardiography. Transoesophageal echocardiography was performed in 45 (39%) patients to assess suitability for closure using the Amplatzer device. Of these, 20 (44% of the group undergoing transoesophageal echocardiography) were considered unsuitable for closure in this fashion. Of these, 8 were referred for surgery and 2 with small defects were considered not to require closure. Patients undergoing closure with the device were older than the group referred for surgical closure, having a median age of 7.8 versus 3.6 years, and stayed for a shorter period in hospital. Those closed using the device stayed for 2 days, as opposed to a median of 5 days, with a range from 4 to 10 days for those undergoing surgical closure. Closure was complete as assessed by echocardiography after follow up of 1–3 months in both groups. There were no recognised complications related to insertion of the device, whereas transient postoperative morbidity occurred in 38% of those closed surgically. Insertion of an Amplatzer device was considered to be appropriate in 37% of patients older than 6 months requiring closure of an atrial septal defect in the oval fossa.
Young Investigator's Prizewinner 2001 Direct visualization of the influence of normothermic as opposed to hypothermic cardiopulmonary bypass on the systemic microcirculation in neonatal piglets
Authors: Wagner Florian M.-J. , Schiller Wolfgang , Dilg Guido , Depner Christian , Welz Armin , Lacour-Gayet Francois
Published: August 2006

The direct visualization of systemic microcirculation using intravitalmicroscopy permits the classification of proinflammatory and ischemic microvascular alterations during normothermic and hypothermic cardiopulmonary bypass in neonates. We used seven newborn piglets, on average aged 9 days, and weighing 3200 g, as a control group. In addition, we studied nine piglets subjected to 60 minutes of constant non-pulsatile flow using hypothermic extracorporeal circulation at 28°C, and five piglets using normothermic conditions at 37°C. The microvascular network of the greater omentum and the subcutaneous tissue was directly visualized using intravitalmicroscopy. We analysed interactions between leukocytes and endothelial cells, microvascular morphology, and microrheological conditions, focussing on signs of ischemic and proinflammatory alterations. During normothermic cardiopulmonary bypass, the numbers of activated leukocytes were elevated compared to hypothermic cardiopulmonary bypass (p > 0.05). Arteriolar diameter decreased during hypothermia. Capillaries were markedly dilated during normothermia. Patterns of microvascular perfusion, for both types of cardiopulmonary bypass, showed signs of ischemic damage, revealed by a reduced functional capillary density. Perfusion dependent levels of lactate were higher during normothermic cardiopulmonary bypass (p > 0.05). This new experimental approach revealed that non-pulsatile cardiopulmonary bypass, independent of temperature, induces a proinflammatory and ischemic response compared to an unaltered control group. The markedly elevated numbers of activated adherent leukocytes, the reduced capillary density, and the high lactate levels in those undergoing bypass in normothermic conditions indicate a more pronounced inflammatory stimulus and tissue hypoperfusion compared to the possible protective effect of hypothermia for children undergoing cardiopulmonary bypass.
Discordances between the different types of atrial arrangement and the positions of the thoraco-abdominal organs
Authors: Machado-Atias Ivan , Anselmi Guillermo , Machado-Hernandez Ivan , Febres Cecilia
Published: August 2006

Numerous investigations have addressed the most appropriate anatomical structures to be used for the identification of segmental arrangement (“situs”). Variations from normal have been termed “heterotaxy syndromes”. The assumption is frequently made that, in such instances, all the organs in a given individual case follow the same rules for segmental morphology. This leads to confusion. The starting point for cardiac analysis is the structure of the atriums. On this basis, we can define viscero-atrial discordance when, in usual arrangement, mirror imagery, and heterotaxy, the atriums and their appendages are no longer concordant with the remaining thoracic and abdominal organs. With this in mind, we examined a large number of autopsied specimens to identify all possible variations of thoracic structures and abdominal organs relative to atrial arrangement. We analyzed 1100 malformed hearts. Of these, 1046 had usual arrangement with normally situated heart (95%); 10 cases had usual arrangement with right-sided heart (0.90%); 25 showed mirror imagery (2.27%), and 19 were found with heterotaxy (1.72%). There was a total of 54 hearts of usual arrangement with right-sided heart, mirror imagery and heterotaxy, (4.90%) and, amongst them, 17 showed viscero-atrial discordances (31.48%). In those having usual arrangement with right-sided heart, there were 2 cases (20%); 8 in those with mirror imagery (32%); and 7 in heterotaxy (36.8%). In the 1046 cases of usual arrangement with normally situated heart, there were 12 examples of viscero-atrial discordances (1.14%). In all, we found a total of 29 cases of viscero-atrial discordances. Taken together, the discordances are best dealt with by describing separately, in every case of normal or abnormal atrial arrangement, the thoracic structures, the atrial appendages, and the abdominal organs. In the final analysis, the diagnosis of the cardiac arrangement rests on the location and morphology of the atriums and their appendages.
Tricuspid valvar endocarditis caused by penicillin resistant pneumococcus
Authors: Bothwell Janice E. , Casey Frank , Webb C. Hugh
Published: August 2006

A 6-year-old boy presented with endocarditis of the tricuspid valve caused by penicillin resistant pneumococcus. He had no history of congenital cardiac disease. The likely portal of entry for the organism was the middle ear.
Treatment of rheumatic carditis with intravenous gammaglobulin: is there a beneficial effect?
Authors: Diab Karim A. , Timani Majd Ariss , Bitar Fadi F.
Published: August 2006

Rheumatic carditis is a major manifestation of acute rheumatic fever. Conventional therapy includes the use of salicylates and steroids. To date, however, such therapy has not been proven to have a clear benefit in reducing valvar heart disease. We report the use of high-dose intravenous immunoglobulin in two chidlren with acute rheumatic carditis in whom we have been able to document the beneficial effect.
Surgical repair of naturally palliated anomalous origin of the left coronary artery from the right pulmonary artery
Authors: Giardini Alessandro , Gargiulo Gaetano , Picchio Fernando M.
Published: August 2006

Anomalous origin of the left coronary artery from the pulmonary arteries is a rare and life-threatening defect, usually needing prompt surgical correction during infancy. We describe the case of a young asymptomatic patient with this defect who underwent surgical reimplantation despite the absence of signs of myocardial ischemia, due to the presence of proximal stenosis of the anomalous coronary artery.
Fetal myocardial hypertrophy in an experimental model of gestational diabetes
Authors: Menezes Honório S. , Barra Marinez , Belló André R. , Martins Cristiano B. , Zielinsky Paulo
Published: August 2006

Introduction: The frequent occurrence of prenatal hypertrophy of the muscular ventricular septum has been widely reported in fetuses of diabetic mothers. Objectives: This experimental study was carried out to test the hypothesis that the weight of the heart, the ratio of the cardiac weight to that of the body, the thickness of the muscular ventricular septum, and the myocytic profile within the ventricular septum are all increased in fetuses of diabetic rats in comparison to fetuses of normal rats. Methods: Diabetes was induced in 5 pregnant Wistar rats, bearing 30 fetuses, on the eighth day after conception, by intraperitoneal injection of 50mg/kg of streptozotocin. Five normal pregnant Wistar rats, bearing 20 fetuses, made up the control group. Morphometric data were obtained by a computer-assisted method applied to the measurements of the thickness of the ventricular septum, and myocytic nuclear area. Statistical analysis utilized Student's t-test and Kruskal-Wallis test. Results: The mean thickness of the septum was 675.56 μm (± 159) in the control fetuses, and 904.39 μm (± 262) in the fetuses carried by diabetic mothers (p < 0.001). The cardiac weight was 0.016 g (± 0.004) in the control group, and 0.023 g (± 0.005) in the group of diabetic fetuses (p < 0.001). The ratio of cardiac to body weight was 0.294% (± 0.079) in the control group, and 0.514% (± 0.073) in the diabetic group (p < 0.001). The myocytic nuclear area was 14.70 μm2 in the control group, and 21.43 μm2 in the diabetic group (p < 0.001). Conclusions: The presence of cellular and morphologic cardiac hypertrophy in fetuses of diabetic rats was demonstrated by the significant difference between the two groups for each analyzed feature.
Normal ranges for the variability in heart rate in young infants while sleeping
Authors: Massin Martial M. , Withofs Nadia , Maeyns Kristel , Ravet Françoise , Gérard Paul
Published: August 2006

Objective: Measurements of the variability in heart rate are increasingly used as markers of cardiac autonomic activity. We sought to establish the development this variability in healthy young infants while sleeping. Patients: We carried out polygraphic studies with electrocardiographic recording in 587 healthy infants aged from 5 to 26 weeks. Methods: We determined several variables over a period of 400 minutes sleeping: mean RR interval, 5 time-domain (SDNN, SDNNi, SDANNi, RMSSD, and pNN50) and 5 frequency-domain indexes (spectral power over 3 regions of interest, total power and low-to-high frequency ratio). Frequency-domain indexes were also assessed separately for the periods of quiet sleep and those of rapid eye movement sleep. Results: Our data showed a significant correlation between the indexes of heart rate variability and the mean RR interval, the breathing rate, and the corrected age of the infants. We also demonstrated the importance of the maturation of the sleeping patterns. Conclusion: These data in a large cohort of healthy infants confirm a progressive maturation of the autonomic nervous system during sleep, and may be used to examine the influence of physiological and pathophysiological factors on autonomic control during polygraphic studies.
Can fibrotic bands in the aortic arch cause innocent murmurs in childhood?
Authors: Nothroff Joerg , Suemenicht Sybille Gundula , Wessel Armin
Published: August 2006

Children with innocent murmurs are often referred to a paediatric cardiologist for diagnosis. The most common murmurs of early childhood are the so-called Still's murmurs, followed by ejection murmurs across the pulmonary or aortic vessels, and the venous hum. There also exists a high coincidence of murmurs with the presence of tendinous structures traversing the cavity of the left ventricle. In this report, we describe 6 patients who presented to our outpatient clinic with cardiac murmurs. None of them had abnormalities on the clinical examination, electrocardiographic, or echocardiographic investigation. They presented a similar murmur that was also audible over the back. On closer examination of the aorta with cross-sectional echocardiography, we discovered echogenic, tendinous structures crossing the lumen of the descending aorta or the aortic arch. Whilst we are not yet able to prove that the cords produced the innocent murmurs, the association is highly suggestive.
An epicardial cyst in a child
Authors: Debus Volker , Krasemann Thomas , Semik Michael
Published: August 2006

An 8-year-old girl, without any previous medical history, presented with a first short syncope. Physical examination was unremarkable. Transthoracic echocardiography revealed a thin-walled, echo-free cystic structure adjacent to the posterior wall of the left ventricle, and compressing it moderately. Other echocardiographic findings were normal. Both computed tomography and magnetic resonance imaging suggested a simple pericardial cyst, but during surgery we found an epicardial cyst with partial involvement of the circumflex branch of the left coronary artery. Cardiopulmonary bypass was necessary for successful resection of the cyst, leaving behind only the small area in continuity with the coronary artery.
Cardiac rhabdomyoma: a report of alternative strategies to surgical resection
Authors: McMahon Colin J. , Ayres Nancy A. , Lewin Mark B.
Published: August 2006

Cardiac rhabdomyoma represents the commonest primary cardiac tumour. Its natural history is generally favourable, with resolution in the majority of cases. Surgical resection has been described for intractable arrhythmia, severe obstruction of the inflow or outflow tracts sufficient to compromise cardiac output, and systemic embolization. We describe an alternative palliative strategy, which was associated with regression of the tumour.
Changes in 99m Technegas ventilation lung scan in a newborn with absent pulmonary valve syndrome
Authors: Takahashi Kazuhiro , Kuwahara Takashi , Nagatsu Masayoshi
Published: August 2006

A newborn infant with tetralogy of Fallot and absent pulmonary valve was successfully corrected in two stages. Absent pulmonary valve syndrome presenting in early infancy manifests severe respiratory symptoms that still make challenging both management and surgical treatment. This is ascribed to tracheobronchial compression by the extremely dilated pulmonary arteries, and to the resultant pulmonary obstructive lesions. We report herein the first findings of 99m Technegas ventilation lung scanning in an infant with the syndrome to assess the pulmonary obstructive lesions.
Interrupted right aortic arch and origin of the left pulmonary artery from the aorta in DiGeorge syndrome
Authors: Sett Suvro S. , Sandor George G. S. , Mawson John B.
Published: August 2006

We describe a neonate with DiGeorge syndrome undergoing diagnosis and successful repair of interrupted right aortic arch and origin of the left pulmonary artery from the aorta. We discuss a link between this lesion and persistence of a left fifth arch.
Spectrum and outcome of atrioventricular septal defect in fetal life
Authors: Fesslova Vlasta , Villa Laura , Nava Simona , Boschetto Chiara , Redaelli Carla , Mannarino Savina
Published: August 2006

Objectives of the study: to analyse the features and outcomes of different types of atrioventricular septal defects, detected during fetal life, as compared to postnatal data. Material and methods: We analysed retrospectively the data concerning 82 fetuses with atrioventricular septal defect, diagnosed from 19 through 37 weeks gestation with a median of 26 weeks. In 46 cases (56.1%), the diagnosis has been made before the age of 24 weeks. Results: Characteristics of the series – in 44 fetuses the atrioventricular septal defect was not associated with other cardiac anomalies, while 38 fetuses had a more complex form. Chromosomal anomalies were present in 33 of the fetuses (40.2%), more frequently in cases without associated intracardiac defects (56.8%). Trisomy 21 occurred in just over one quarter the series, and in 43.2% of cases without associated defects. In addition, 11% of fetuses had trisomy 18, and one had trisomy 13. Extracardiac anomalies were present in 12 of the fetuses (14.6%), more frequently in cases without associated abnormalities. Of fetuses with more complex defects, 46.4% had hypoplasia of the left ventricle and aorta. Complete atrioventricular block was present in 10 of the fetuses (12.2%), mainly in fetuses with other malformations, and particularly with left isomerism. Recurrence of congenital heart disease was observed in 5 of the fetuses (6.1%). Outcome: In 25 instances (30.5%) the parents opted for termination of pregnancy. Of 57 cases that continued through pregnancy, 9 fetuses died prior to term (15.8%), 32 died postnatally (56.13%) and only 16 fetuses (28.1%) survived. Overall, the mortality was higher in cases with associated malformations, in those with heart failure or those with atrioventricular block. Cardiac surgery was performed in 19 infants, with 5 dying postoperatively, and one late. Conclusions: Our data show a high prevalence of atrioventricular septal defect associated with other malformations when diagnosed during fetal life. This combination is less frequently associated with chromosomal and extracardiac anomalies, but more often with obstructive lesions of the left heart and with atrioventricular block. The association results in a less favourable outcome.
Identification of gender differences in the thickness of the left ventricular wall by echocardiography in children
Authors: Nagasawa Hiroyuki , Arakaki Yoshio
Published: August 2006

We assessed the thickness of the normal left ventricular wall according to gender by performing twodimensional echocardiography in children. While a few studies have evaluated left ventricular wall thicknesses in children using two-dimensional echocardiography, there is little information related to the influence of gender throughout childhood. We evaluated 251 patients (128 males and 123 females) with innocent murmurs but without congenital heart anomalies, or coronary artery lesions or arrhythmias, as determined by repeated echocardiographic, and electrocardiographic examinations. Intra-ventricular septal thicknesses at end-diastole and end-systole, and left ventricular posterior thicknesses at end-diastole and end-systole were determined in the parasternal short-axis view. Significant gender differences were observed in the thickness of all four measurements. There was a linear relationship between the thickness of the left ventricular wall and height. Height was the most appropriate and practical index for assessing the thickness of the wall of the left ventricle in children.
Three-dimensional computed tomography in children with compression of the central airways complicating congenital heart disease
Authors: Kim Yang Min , Yoo Shi-Joon , Kim Tae Hoon , Park In Seung , Kim Woong Han , Lee Jae Young , Han Mi Young
Published: August 2006

Purpose: We investigated the quality and usefulness of spiral computed tomography and threedimensional reconstruction in children with obstruction of the central airways as a complication of congenital heart disease. Materials and methods: Spiral computed tomography with three-dimensional reconstruction was performed in 49 children with obstruction of the central airways seen as a complication of congenital heart disease. Spiral scanning was performed during breathing in 40, and after sedation with chloral hydrate in 38. Contrast medium was administered through a pedal venous route in 42. We analyzed the motion artifact, additional information provided by, and clinical usefulness, of the three-dimensional images. We also investigated the factors influencing the quality of the images. Results: Stenoses were seen in the trachea in 21 patients, and in bronchuses in 28. Their causes were an anomaly of the aortic arch in 6, posterior displacement of the aortic arch in 7, posterior displacement of the ascending aorta in 5, compression of the brachiocephalic artery in 5, absent pulmonary valve syndrome in 6, displaced or dilated cardiovascular structure in 17, and pulmonary arterial sling in 2. Motion artifact caused mild or negligible degradation of images in all patients except 6. Breath-holding in non-sedated children produced more severe motion artifact than did cardiovascular pulsation. Threedimensional images provided additional information over two-dimensional images in 11, and provided clinically useful information in 10. Contrast injection via the pedal route was better for the quality of threedimensional images than brachiocephalic injection (p = 0.013). Conclusions: Three-dimensional computed tomography is useful in evaluation of obstruction of the central airways in children with congenital heart disease. Despite the fact that motion artifact is unavoidable, the quality of three-dimensional images is acceptable for making a proper and accurate diagnosis. A pedal route is recommended for injection of contrast medium.
Multiple valvar replacements for hypereosinophilic syndrome
Authors: Radford Dorothy J. , Garlick R. Bruce , Pohlner Peter G.
Published: August 2006

A boy with familial eosinophilia had the hypereosinophilic syndrome, with involvement of mitral and tricuspid valves. Between the ages of 11 and 20 years, he underwent eight surgical procedures on his atrioventricular valves. The pathology included recurrent thrombotic vegetative masses related to hypereosinophilia. Initial repair of the mitral valve was shortlived, but recurrent repairs of the tricuspid valve were helpful. Mechanical prostheses inserted in the mitral position thrombosed despite anticoagulant therapy, and bioprosthetic valves deteriorated with thrombus, fibrosis, or tearing. The hypereosinophilic syndrome is unusual in children, and produces additional problems with valvar surgery.