bluuebunny/crossref_metadata_2025_split huggingface dataset:
Physical activity in children and adolescents with congenital heart disease. Aspects of measurements with an activity monitor
Authors: Fredriksen P. M. , Ingjer E. , Thaulow E. 
Published: August 2008

AbstractThe aim of the present study was to evaluate different aspects of a device designed to monitor physical activity. Measurements of different axes and placement of the monitor were tested using a treadmill with ranging increments in incline or speed. The monitor was also used to assess the level of physical activity among children and adolescents with congenital heart disease and in healthy controls at the same age. The results indicate that the monitor is a valid and reproducible instrument for measurements of physical activity. The study revealed that the level of activity was higher for healthy boys than healthy girls (p<0.0001). Boys with congenital heart disease also displayed higher values compared to girls with congenital heart disease, although the difference was not significant (p=0.067). Healthy boys revealed a significantly higher level of activity than did boys with congenital heart disease (p=0.003), but no such difference was found in girls (p=0.757). Nor were any differences found between younger and older indi viduals among patients with congenital heart disease. Young healthy controls, however, showed significantly higher levels of activity than their older counterparts. There were differences in activity monitored during the week, with lower activity in the weekends, but the activity on the same day in different weeks seemed stable. Neither were there any differences between measurements over whole weeks. The results indicate that the Computer Science & Application monitor is a valid instrument for assessing physical activity. The monitor may also be used, therefore, to validate the levels of physical activity level in children with congenital heart disease after medical and surgical treatment.
Effect of physical training in children and adolescents with congenital heart disease
Authors: Fredriksen Per Morten , Kahrs N. , Blaasvaer S. , Sigurdsen E. , Gundersen O. , Roeksund O. , Norgaand G. , Vik J.T. , Soerbye O. , Ingjer F. , Thaulow E. 
Published: August 2008

AbstractIn order to test the effect of systematic supervised physical training, we divided a total of 129 children and adolescents with congenital heart disease into a group undergoing intervention and a control group. All patients underwent exercise tests, measurements of physical activity, and a survey of psychosocial factors. An improvement in uptake of peak level of oxygen was observed after intervention. There was also an improvement in physical activity in both groups measured by a monitor, although this was significant only in those with intervention. The psychosocial scales measured by the Child Behavior Checklist showed a decrease in internalizing scores for those subjected to intervention. This was decreased due to decreased withdrawal and somatic complaints. In conclusion, we recommend systematic supervised training, including testing of routine follow-ups, in patients with congenital heart disease.
Non-invasive determination of the systolic peak-to-peak gradient in children with aortic stenosis: validation of a mathematical model
Authors: Lima Valter C. , Zahn Evan , Houde Christine , Smallhorn Jeffrey , Freedom Robert M. , Benson Lee N. 
Published: August 2008

AbstractDoppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient=6.02+1.49*(mean gradient)−0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y=0.67 × + 11.11, r=0.87, SEE=6 mm Hg, p=0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y=0.66 × + 14.44, r=0.84, SEE=9 mm Hg, p=O.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.
Isolated origin of the left subclavian artery from the left pulmonary artery
Authors: Russell Jennifer L , Smallhorn Jeffrey F. , Black Michael D. , Hornberger Lisa K. 
Published: August 2008

AbstractWe describe two children with isolated origin of the left subclavian artery from the left pulmonary artery detected by echocardiography during the assessment of their congenital cardiac malformations. Both patients demonstrated pre-operative evidence of subclavian steal. This entity results from persistence of the dorsal segment of the sixth left arch, with regression of the left fourth arch and interruption of the left dorsal arch distal to the origin of the seventh left intersegmental artery. The significance of this finding relates to the potential for pulmonary overcirculation, which could have significant post-operative ramifications if not detected prior to surgical repair of an associated cardiac malformation. This entity differs from cases with a right aortic arch and aberrant left subclavian artery which has the potential to form a vascular ring, unlike cases with isolated origin of the left subclavian artery from the pulmonary artery that do not cause compression of the airway.
Left ventricular function in adolescents and adults with restrictive ventricular septal defect and moderate left-to-right shunting
Authors: Magee Alan G. , Fenn Lynn , Vellekoop Joric , Godman Michael J. 
Published: August 2008

AbstractBackgroundThe long-term haemodynamic effects of a restrictive ventricular septal defect permitting moderate left-to-right shunting are not known.Patients and methodsEchocardiographic measure ments of left heart dimensions and function were compared between a group of 9 older children and adults (median age 21 years, range 12–24.5 years) having restrictive ventricular septal defects (median Qp/Qs 1.7, range 1.4–2.1) and a group of 10 age matched controls.ResultsLeft ventricular mass indexed to body surface area was significantly greater in subjects than in controls (102±29 vs. 75±13 g/m2, p=0.02), although there was no significant difference in the ratio of mass to volume. There were no significant differ ences between indexes of left ventricular systolic function. Ratios of peak early to late diastolic ventricular filling were lower in those with septal defects (1.5±0.3 vs. 1.8±0.3, p=0.013), but there were no differences in other indexes of diastolic function.ConclusionsResting left ventricular function does not appear to have deteriorated by early adult life in patients with restrictive ventricular septal defects and moderate volume loading. This would support a continued conservative approach to management in these patients.
Neonatal pulmonary hypertension during extracorporeal membrane oxygenation
Authors: Tanke Ronald B. , Daniëls Otto , van Lier Henk J. , van Heyst Arno F. , Festen Cees 
Published: August 2008

AbstractObjectivesThis prospective study was designed to monitor severe pulmonary hypertension during extra corporeal membrane oxygenation using echo Doppler variables.BackgroundAll neonates treated with extracorporeal membrane oxygenation also have severe pulmonary hypertension. A study which monitors the reaction of the pre-existing pulmonary hypertension during extracorporeal oxygenation by frequent sampling of those variables related to pulmonary pressure is still lacking. Such a study is necessary to analyze the complex haemodynamic changes in patients undergoing extracorporeal membrane oxygenation.MethodIn 29 neonates, we estimated pulmonary arterial pressure using peakflow velocity of regurgitation across the tricuspid- and pulmonary valve, peakflow velocity of shunting across persistent arterial ductus, and systolic time intervals of the right ventricle. Correlation between the several estimations of pulmonary arterial pressure were analysed with the Spearman correlation coefficient.ResultsSystolic pulmonary arterial pressure measured by the velocity of tricuspid regurgitation illustrated severe pulmonary hypertension prior to extra corporeal membrane oxygenation (mean 63 mmHg, sd 20). Similar levels for the systolic pulmonary arterial pressure could be derived (mean 73 mmHg, sd 17) from ductal shunting. A fair correlation of 0.76 (p< 0.002) could be demonstrated. Pulmonary hypertension responded well and quickly to treatment by extra corporeal membrane oxygenation, with reductions within 24 hours to mean systolic levels of 35 mmHg, sd 23. This very early reaction has not previously been demonstrated and could be of importance in defining parameters for weaning from cardiopulmonary bypass. Diastolic pulmonary arterial pressure was investigated because of its relation to vascular resistance. It proved more difficult to measure because of the low incidence of pulmonary regurgitation. Derived diastolic pressures did not show any good correlations.ConclusionPulmonary hypertension is well documentated prior to extra corporeal membrane oxygenation and respons very quickly to the institution of treatment. Ultra sound techniques are indicated at the bedsite, and prove useful in monitoring pulmonary blood pressure during the procedure.
Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases.
Authors: Gerlis L. M. , Durà-Vilà G. , Ho S. Y. 
Published: August 2008

AbstractWe present two cases in which there was isomeric arrangement oi the left atrial appendages in association with multiple spleens and complete heart block. In both of these, the venous connections were normal. In one case the arrangement of the bronchial tree and the lungs was normal, the liver was left-sided, and the stomach was right-sided. In the other case, there was left isomeric broncho-pulmonary morphology, and both liver and stomach were right-sided. These unusual combinations show the need for full description of the morphology of other organs, and the venoatrial connections, in cases with isomeric arrangement of the atrial appendages. They also demonstrate that connection of the inferior caval vein cannot be taken as a reliable marker of an atrium having a morphologically right appendage.
Anomalous origin of the main stem of the left coronary artery from the non-facing sinus of valsalva associated with sudden death in a young athlete
Authors: Hamamichi Yuji , Okada Eikichi , Ichida Fukiko 
Published: August 2008

AbstractA young female athlete is described with anomalous origin of the main stem of the left coronary artery from the non-facing sinus of Valsalva who sustained myocardial infarction and died suddenly after physical exertion. Autopsy findings illustrated the mechanistic importance of acute angle take-off of the left main coronary artery and a slit-like orifice, which was likely compressed and obstructed by acute expansion of the aortic wall. This rare type of coronary anomaly has been regarded as having little clinical significance, but it can lead to sudden cardiac death under physical exertion.
Dependence on a respiratory ventilator due to an atrial septal defect
Authors: Motz Reinald , Grässl Gerhard , Trawöger Rudolf 
Published: August 2008

AbstractAn atrial septal defect is commonly thought of as a benign cardiac lesion especially in infancy. The haemodynamic consequences for the lungs, nonetheless, can be comparable to that produced by patency of the arterial duct. In a preterm boy, this lesion led to the development of broncho-pulmonary dysplasia, and dependence on a respiratory ventilator. He could be extubated shortly after surgical closure of the septal defect.
Aortic vasculopathy with aneurysm: a rare cause of fetal hydrops
Authors: Nissen Teo , Silverman Norman H. , Ursell Philip C. 
Published: August 2008

AbstractAn hydropic fetus seen at 28-weeks gestation had a saccular aortic aneurysm in the descending thoracic aorta. Histology disclosed marked fibrointimal hyperplasia, thrombus, and attenuation of the tunica media. The remainder of the descending thoracic aorta showed fibrointimal hyperplasia. We speculate that the narrowed lumen and rigid aortic wall resulting from this vasculopathy provided an increased afterload leading to cardiac failure.
Intermittent complete closure of the arterial duct
Authors: Chessa Massimo , Vettukattil Joseph J , Stümper Oliver 
Published: August 2008

AbstractIntermittent functional closure of a patent duct has been reported both clinically and echocardio-graphically. We describe the case of a 6-year-old with intermittent complete closure of a patent duct occurring during attempted transcatheter closure. Recurrence of clinical and echocardiographic signs prompted restudy. A patent duct of moderate size was demonstrated and successfully occluded by coil embolization. The interventional aspects of this unusual condition are discussed.
Fetal hydrops due to a tachyarrhythmia progressing to organic pulmonary stenosis
Authors: Fesslova V. , Villa L. , Nicolini U. 
Published: August 2008

AbstractWe discovered supraventricular tachycardia with advanced hydrops in the setting of normal cardiac anatomy at 26 weeks of gestation which resolved successfully following administration of digoxin and flecainide to the mother. A female baby was born after a premature rupture of the membranes at 30.6 weeks. The neonate was in sinus rhythm, showed a progressive regression of right ventricular insufficiency, but developed signs of acquired pulmonary valvar and subvalvar stenosis at 2 months of age.
Diagnosis and natural history of isolated congenital pulmonary regurgitation in fetal life
Authors: Mori Kazuhiro , Hayabuchi Yasunobu , Kuroda Yasuhiro 
Published: August 2008

AbstractWe describe a rare instance of isolated pulmonary regurgitation caused by a dysplastic pulmonary valve which was detected prenatally. Fetal echocardiography demonstrated severe pulmonary regurgitation, and progressive cardiomegaly because of right ventricular volume overload. After birth, conservative therapy was successful in alleviating the pulmonary vascular resistance, and the pulmonary regurgitation gradually decreased.
Pulmonary and systemic arteriovenous fistulas in patients with left isomerism
Authors: Kawata Hiroaki , Kishimoto Hidefumi , Ikawa Seiichiro , Ueno Takayoshi , Nakajima Tohru , Kayatani Futoshi , Inamura Noboru , Nakada Takeshi 
Published: August 2008

AbstractAbstract Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in 1 of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow, was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.
Early clinical experience with use of the ‘Amplatzer Septal Occluder’ device for atrial septal defect
Authors: Wilkinson James L , Goh Tiow Hoe 
Published: August 2008

AbstractDevice closure of oval fossa atrial scptal defects with the Amplatzer Septal Occluder was performed in 26 patients ranging in age from 0.89 to 60.44 years. In eight additional patients no device implant was performed because of the presence of multiple defects or because the defect was of a size unsuitable for closure with the devices currently available. The strectched diameter of the defects that were closed ranged from 4 to 23 mm (mean 14±5.4 mm) and device sizes ranged from 4 to 24 mm. Two devices were unstable, of which one embolized to the right atrium after release. Both devices were retrived at the same procedure. One of these parients subsequently underwent a successful device closure of his defect using a larger (24-mm) device. Three patients had multiple defects, which were successfully closed with a single device. At 1-month follow-up 23/26 (88%) and at 3-month follow-up 22/24 (92%) patients had complete closure of their defects, while two had residual shunts. One further patient who had complete closure of his defect at 1-month post-implant had his device removed and his atrial septal defect patched surgically 8 weeks after device closure. This was done as a result of the development of a vegetation affecting the device after an episode of septicaemia, which was not relate to the cardiac problems. There was no procedure-related morbidty or martality and all patients remain well at the present time.
Growing up with congenital heart disease: the dilemmas of adolescents and young adults
Authors: Tong Elizabeth M. , Sparacino Patricia S.A. , Messias DeAnne K.H. , Foote Dru , Chesla Catherine A. , Gilliss Cartherine L. 
Published: August 2008

AbstactAdvances in diagnosis, medical management and surgical intervention have improved the longevity and quality of life for children with congenital heart disease. Despite this, research studies specifically examining the psychosocial concerns of adolescents and young adults with congenital heart disease are few. To explore the subjective experiences and dilemmas of this population during the transition from adolescence to young adulthood, we interviewed, using a semi-structured protocol, a convenience sample of nine adolescents and young adults. Using analytic procedures inherent in Grounded Theory methodology, seven themes were identified: the dilemma of normality; dilemmas in disclosure; dilemmas in strategies for management of illness; the challenge of social integration versus social isolation; the challenge of dependence versus independence; the challenge of uncertainty; and strategies for coping. An understanding of these experiences by health professionals can be beneficial in helping this clinical population as they grow up and face the challenges of an uncertain, yet promising, future.
Verapamil therapy in infants with hypertrophic cardiomyopathy
Authors: Moran Adrian M. , Colan Steven D. 
Published: August 2008

AbstractWe sought to evaluate the saftey and efficacy of acure and chronic treatment with verapamil infanta with hypertrophic cardimyopathy. Prior studies have shown an improvement in adluts with hypertrophiuc cardiomyophathy who were treated with verapamil. Acutely, it reduced the degree of left ventricular outflow tract abstruction. Chronic therapy was associated with an improvement in sysmptoms ans incresed long-term survial. To date, no data are available on the efficacy of this drug in infants with hypertrophic cardiomyopathy. We evaluated prospectively the safety and efficacy of verapamil in infants. The acute and chronic effects of verapamil on infants with an echocardiographic diagnosis of hypertrophic cardiomyopathy were evaluated at a single institution between 1980 and 1994, with long-term follow-up available until 1996. Acute effects of an intravenous bolus of 0.1 mg/kg and infusion at 0.007 mg/kg/min were evaluated, where possible, in the cardiac catheterization laboratory. Oral verapamil at 3–5 mg/kg/day was started after catheterization. Follow-up included serial clinical, echocardiographic and Holter monitoring. A total of 22 patients were studied, 17 having a presumed diagnosis of primary hypertrophic cardiomyopathy including three with Noonan's syndrome. Acute infusion of the drug was well tolerated by all, without adverse electrophysiological effects. Haemodynamic effects were consistent with a negative inotropic action with significant falls in cardiac index (4.6±1.2 to 4.1±0.9 l/min/m2), systolic blood pressure (88±16 to 82±14 mmHg) and gradient across the left ventricular outflow tract (nine patients 48.2±30.4 to 28.4±24.1 mmHg). End-diastolic pressure was unchanged (14.0±6.8 to 13.9±4.7 mmHg). Three patients with primary hypertrophic cardiomyopathy died (two while being treated). In the group with primary hypertrophic cardiomyopathy continuing with long-term treatment, follow-up revealed regression in two, progression in three (two died) and stability in 10. For those treated, there was a trend towards improvement in clinical status. Verapamil is well tolerated acutely in infants with hypertrophic cardiomyopathy. The outcome was considerably better in these patients compared with prior reports, though careful long-term assessment is needed.
Epidemiology and diagnosis of ventricular septal defect in Malta
Authors: Grech Victor 
Published: August 2008

AbstractMalta is a small island with minimal changes in its population, making it an ideal location for epidemiological and historical studies dealing with congenital heart malformations. Ventricular septal defect was studied retrospectively from 1930 to 1994. A sharp and significant decline in age at diagnosis was found, predating echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence of this lesion as seen at birth, particularly of minor defects. The prevalence of ventricular septal defect from 1990 to 1994 was 3.85/1000 live births, with 3.O3/1OOO not needing surgery and 0.83/1000 requiring operative intervention. Half the defects closed spontaneously. The majority of ventricular septal defects overall, and those spontaneously closing, were muscular defects. The overall prevalence at birth was significantly higher than that reported in recent studies using similar methodologies, implying that the reported rate is more likely to be the true prevalence at birth of clinically detectable defects.
History, diagnosis, surgery and epidemiology of pulmonary stenosis in Malta
Authors: Grech Victor 
Published: August 2008

AbstractThe Maltese populartion constitutes an ideal location for epidemiological and historical studies dealing with congenital hearr malformations. Pulmonary stenosis was studied retrospectively from 1943 to 1994. A sharp, significant decline in age at diagnosis was found, which predates the introduction of echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence at birth of this lesion, patticularly of pulmonary stenosis not requiring intervention. The prevalence at birth of pulmonary stenosis from 1990 to 1994 was 1.65/1000 live births (95% CL: 1.21–2.24), with 1.11/1000 mild lesions (95% CI: 0.76–1.62) and 0.54/1000 lesions requiring intervention (95% CI: 0.31–0.92). The prevalence at birth overal was significantly higher than that reported in recent studies with similar methodologies due to the higher pickup of milder variants of pulmonary stenosis by echocardiography.Significantly more pulmonary more pulmonary stenosis was found in females than in males.