bluuebunny/crossref_metadata_2025_split huggingface dataset:
Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children
Authors: Daubeney P. E. F. , Blackstone E. H. , Weintraub R. G. , Slavik Z. , Scanlon J. , Webber S. A. 
Published: August 2008

AbstractNormalization of the dimensions of cardiac structures to the size of the body, using so-called Z scores, is becoming increasingly common in the management of infants and children with congenital heart disease. Current published nomograms for the ascertainment of Z scores for cardiac structures in childhood are based largely on normal data obtained in formalin-fixed hearts. Since decisions concerning management are frequently based on the findings of cross-sectional echocardiograms, the dimensions of 15 cardiac structures were measured using cross-sectional echocardiography in 125 normal infants and children. Regression equations were derived relating cardiac dimensions to the size of the body. The expression of size with the highest correlation to cardiac dimensions was body surface area. Nomograms were then developed from which the Z score of a cardiac structure could be estimated from a knowledge of the body surface area and the echocardiographically derived measurement.
Double-outlet right ventricle with intact ventricular septum in a foetus with trisomy-18
Authors: Patel Chandrakant R. , Muise Kevin L. , Redline Raymond W 
Published: August 2008

AbstractA rare case of double-outlet right ventricle with intact ventricular septum diagnosed by foetal echocardiography at 21 weeks of gestation is described. Amniocentesis revealed trisomy–18. The cardiac diagnosis was confirmed at autopsy.
Divided left atrium associated with supravalvar mitral ring
Authors: Álvares Silvia , Melo António Sá , Antunes Manuel 
Published: August 2008

AbstractReported is a case with a rare association of divided left atrium, supramitral stenosing ring of the left atrium, connection of the left superior caval vein to the roof of the left atrium, unroofed coronary sinus with an interatrial communication at the mouth of the unroofed sinus and ventricular septal defect. The need for a complete echocardiographic examination in the presence of pulmonary venous obstruction is emphasized. Surgery was successful in spite of significant preoperative pulmonary hypertension.
Divided right atrium. Diagnosis by echocardiography, and considerations on the functional role of the Eustachian valve
Authors: Corno Antonio F. , Bron Cédric , von Segesser Ludwig K. 
Published: August 2008

AbstractA child presented at birth with severe cyanosis. Echocardiography showed hypoplasia of the right heart with a right-to-left shunt at atrial level. A conservative approach was adopted initially, and the situation improved over a few months, with reversal of the atrial shunt. Surgery was successfully performed at 4 years of age after further echocardiography revealed a congenitally large Eustachian valve and an atrial septal defect.
Sequential segmental approach to fetal congenital heart disease
Authors: Yoo Shi-Joon , Lee Young Ho , Cho Kyoung Sik , Kim Dae-Young 
Published: August 2008

AbstractThe sequential segmental approach is now universally used in the diagnosis of congenital heart disease. Its utilization during fetal sonography has not been well described. In this review, we show how the fetal heart can be approached in a sequential segmental manner by using six basic sonographic views. The transverse view of the fetal upper abdomen is obtained to determine the arrangement of the abdominal organs, which, in most cases, provides the important clues to the determination of the atrial arrangement. The four-chamber view is obtained to evaluate the atrioventricular junctions. The views of the left and right ventricular outflow tracts are obtained to evaluate the ventriculoarterial junctions. The three-vessel view and the aortic arch view are obtained for the evaluation of the arrangement and size of the great arteries, which provides the additional clues to the diagnosis of the abnormalities involving the ventriculoarterial junctions and the great arteries. The standard protocol to acquire these six basic views is also introduced.
Interruption of the aortic arch associated with deletion of chromosome 22q11 is associated with a subarterial and doubly committed ventricular septal defect in Japanese patients
Authors: Momma Kazuo , Ando Masahiko , Matsuoka Rumiko , Joo Kunitaka 
Published: August 2008

AbstractObjectivesThe purpose of this study was to clarify the clinical characteristics of interruption of the aortic arch associated with chromosome 22q11 deletion.BackgroundAbout half of patients with interruption of the aortic arch between the left common carotid and the left subclavian artery have deletion of chromosome 22q11.MethodsIn total, 20 patients with interruption of the aortic arch were studied with fluorescence in situ hybridization using peripheral lymphocytes and a DiGeorge syndrome chromosomal probe (Oncor N25). Cardiovascular anomalies in these patients were diagnosed by cross-sectional echocardiography and angiocardiography, and were confirmed at intracardiac repair.ResultsOf 13 patients with interruption between the left common carotid artery and the left subclavian artery, seven had the deletion. All 7 also showed thymic hypoplasia and hypocalcemia, together with a nasal voice and peculiar facies. Six of the seven patients had complete deficiency of the muscular outlet septum, with the defect extending to the perimembranous area. Such complete absence of the muscular outlet septum was not present in any of the patients without the deletion.ConclusionsInterruption of the aortic arch between the left common carotid and the left subclavian artery, absence of the thymus, and complete absence of the muscular outlet septum, were characteristic in Japanese patients with interruption of the aortic arch associated with deletion of chromosome 22q11.
Transcatheter closure as standard treatment for most interatrial defects: experience in 200 patients treated with the Amplatzer ™ Septal Occluder
Authors: Berger Felix , Ewert Peter , Boöjrnstad Per G. , Dähnert Ingo , Krings Gregor , Brilla-Austenat Ira , Vogel Michael , Lange Peter E. 
Published: August 2008

AbstractTo judge whether an Amplatzer™ Septal Occluder can be used as standard therapy instead of surgery for closure of atrial septal defects we report our experiences in 200 patients. Of these patients, 127 had an atrial septal defect with haemodynamically significant left-to-right shunt, 68 patients a persistent oval foramen after presumed paradoxical embolism, and 5 had a fenestration after Fontan-repair. Mean age was 29.8 years (0.8 to 77.7 years). Body weight ranged from 6.9 to 120.0 kg (mean 51.5 kg). After diagnostic cardiac catheterization, and balloon-sizing of the defect, we implanted Amplatzer™ Septal Occluders with stents of 4 to 28 mm diameter. Follow-up studies were obtained after 48hours, and one, six, and twelve months. Transcatheter closure of the atrial septal defect proved successful in all without any relevant residual shunts. In particular, complete closure was achieved in all patients after presumed paradoxical embolism. The mean period of follow-up is 9–5 months, with a range from 0.4 to 23.5 months, giving a total of 1898 patient months. The occlusion rate after three month was 98.1°. A trivial haemodynamically insignificant residual shunt remained in 1.9° of the patients. Fluoroscopy times ranged from 0 to 43.5 minutes, with a median of 8.7 minutes. The excellent results in the short and medium term in children and adults have resulted in using this device routinely at the present time for closure of central atrial septal defects up to a diameter of 28 mm. Final judgement, however, is only possible after long-term follow-up.
Reduced frequency of occlusion of aorto-pulmonary shunts in infants receiving Aspirin
Authors: Motz Reinald , Wessel Armin , Ruschewski Wofgang , Bürsch Jochen 
Published: August 2008

AbstractObjectiveInfants with severely reduced pulmonary perfusion due to complex congenital cardiac malformations are in need of an improved flow of blood to the lungs. One option for treatment is to construct a systemic-to-pulmonary arterial shunt. Although such shunts have been used since 1945, their spontaneous occlusion remains a major problem in the long-term.DesignWe studied all infants in whom a systemic-to-pulmonary arterial shunt had been constructed using a Gore-Tex tube graft between December 1989 and March 1996.PatientsOf 46 infants undergoing construction of a shunt, 7 (15°) died within 30 days of surgery. The shunts had to be taken down in 2 infants. Thus, 37 infants were included in the study. All but three infants received Aspirin. Aspirin was discontinued on the personal decision of individual physicians. Of 22 infants, 3 never received Aspirin, and in 19 it was stopped well before undertaking subsequent surgery. Aspirin was administered continuously to 15 infants until further surgery.ResultsThose in whom Aspirin was discontinued, or not given, and those receiving Aspirin until further surgery, were comparable concerning their age, time of follow-up, severity of the cardiac lesions, and size and type of shunt. Partial or complete occlusion of the shunt occurred in 2 of 15 (13°) infants taking Aspirin, but was seen in 12 of 22 (54°) infants in whom Aspirin was discontinued. Of these, 3 died due to acute occlusion of the shunt.ConclusionsAspirin reduced effectively the rate of occlusion of systemic-to-pulmonary arterial shunts, and should be continued as long as the shunt is in place.
Unguarded mitral orifice, mirror-imaged atrial arrangement, and discordant atrioventricular connections
Authors: Yasukochi Satoshi , Satomi Gengi , Park Insam , Ando Masahiko , Momma Kazuo 
Published: August 2008

AbstractWe report two autopsy proven cases of unguarded mitral orifice associated with mirror-imaged atrial arrangement, discordant atrioventricular connections, double outlet right ventricle, pulmonary valvar stenosis or atresia, and atrialisation of the morphologically left ventricle. The morphologically left atrioventricular junction was devoid of valvar leaflets, and there was no tension apparatus within the ventricle. To the best of our knowledge, this is the first description of this rare cardiac malformation
Comparison of acute effects on right ventricular haemodynamics of surgical versus interventional closure of atrial septal defects
Authors: Berger F. , Jin Z. , Ishihashi K. , Vogel M. , Ewert P. , Alexi-Meshkishvili V. , Weng Y. , Lange P. E. 
Published: August 2008

AbstractObjectivesWe compared the acute effects on right ventricular haemodynamics of surgical versus transcatheter closure of medium-sized atrial septal defects.MethodsWe studied 47 consecutive patients with a defect in the oval fossa and a ratio of pulmonary to systemic flows between 1.5: 1 and 2: 1. They were divided into two groups according to whether the defects were closed by surgery, performed in 23 patients, or by interventional catheterization, achieved in 24 patients. By means of transthoracic cross-sectional echocardiography, we measured right ventricular end-diastolic and endsystolic volumes and calculated ejection fractions. These calculations were performed before, and between 1 and 7 days after closure of the defect.ResultsBefore closure of the defect, all patients had an enlarged right ventricle with normal function. After closure by either method, there was no difference in the rate of normalization of end-diastolic volume, but endsystolic volume remained enlarged. Thus, the calculated ejection fraction was lower than before closure.ConclusionsThere was no difference in right ventricular volumes or function early after closure of atrial septal defects, irrespective of whether this was achieved surgically or via transcatheter closure.
The aortic valve with two leaflets — a study in 2,000 autopsies
Authors: Pauperio Helder M. , Azevedo Arthur C. , Ferreira Claudia S. 
Published: August 2008

AbstractOur study is based on the examination of 2,000 aortic valves obtained from fresh cadavers (1,499 males, 501 females) at the Institute of Forensic Medicine in Rio de Janeiro. We discovered 13 valves having two leaflets, giving a prevalence of 0.65%, much lower than generally reported in the literature. All 13 valves were from males, 10 from whites, three from mulattos, and none from blacks. Special attention was given to the raphe and the leaflets, the calcification of which can lead to stenosis. The only valves with normal texture and flexibility were two obtained from children less than one year old. All the other valves were thickened, and five of them had some degree of calcification. Six valves were judged to be functioning normally, while 7 valves were abnormal, 5 being stenotic and two showing evidence of insufficiency. The insufficiency in one was due to endocarditis, but in the other was due to redundancy of the leaflets. The only other cardiac anomaly discovered in these 13 cases was one patient with aortic coarctation. Bifoliate aortic valve, therefore, is probably the most common cardiac anomaly, although its prevalence as discovered in Brazil is lower than that reported in the literature. It affects mainly white males. After the fourth decade of life, most valves present some thickening, with stenosis being the most common complication. As is well recognised, infective endocarditis and aortic insufficiency are the other frequent complications. It is in general, nonetheless, an isolated anomaly.
Spontaneous closure and thrombosis of a ductal aneurysm in a neonate
Authors: Maisel Peccina , Brenner Joel 
Published: August 2008

AbstractThe natural history of a ductal aneurysm detected prenatally is presented. Neurologic concerns in the neonate and absence of cardio-respiratory compromise permitted serial echo demonstration of constriction, thickening and subsequent closure with formation of thrombus in the aneurysm.
Cyanosis due to diastolic right-to-left shunting across a ventricular septal defect in a patient with repaired tetralogy of Fallot and pulmonary atresia
Authors: Thomson J. D. R. , Forster J. , Gibbs J. L. 
Published: August 2008

AbstractCyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.
Obstruction of the right ventricular outflow tract caused by a tuberculoma in a patient with ventricular septal defect and aneurysm of the membranous septum
Authors: Santos Cleusa Lapa , Moraes Fernando , Moraes Carlos R. 
Published: August 2008

AbstractReported here is an obstruction of the right ventricular outflow tract caused by a tuberculoma in a 15-year-old boy who presented with a ventricular septal defect. The obstruction was discovered at surgery and the tuberculous aetiology was only demonstrated histologically. To the authors' knowledge, this is the first report of a tuberculoma of the heart associated with congenital heart disease.
Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery
Authors: Patel Chandrakant R. , Spector Michael L. , Zahka Kenneth G. 
Published: August 2008

AbstractDescribed is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.
Interruption of the aortic arch at the isthmus with DiGeorge syndrome and 22q11.2 deletion
Authors: Takahashi Kazuhiro , Kuwahara Takashi , Nagatsu Masayoshi 
Published: August 2008

AbstractA 6-day-old male with interruption of the aortic arch at the isthmus (type A) had the typical phenotype of DiGeorge syndrome. There was also a doubly committed juxta-arterial ventricular septal defect and an unobstructed left ventricular outflow tract. Hypoplasia of the thymus was confirmed during a modified Blalock-Park operation. He had persistent hypocalcemia, and was susceptible to infection. He was subsequently revealed by the use of fluorescence in situ hybridization analysis to have 22q11.2 deletion. Interruption of the aortic arch at the isthmus is presumed to reflect abnormal fetal hemodynamics, and is considered a distinct pathogenetic entity from interruption between the left common carotid and subclavian arteries, the latter being the variant more frequently associated with DiGeorge syndrome. In our case, the 22q11.2 deletion likely played a major role in the etiology of the interrupted aortic arch.
Rapid reversal of dilated cardiomyopathy following removal of neuroblastoma
Authors: Rosti Luca , Festa Pierluigi , Corbetta Carlo 
Published: August 2008

AbstractReported is a child with dilated cardiomyopathy, in whom medical therapy resulted in a mild improvement of cardiac function. Metabolic studies suggested the presence of a catecholamine-secreting tumour; and an adrenal neuroblastoma was identified and surgically removed. Following surgery, there was progressive and complete normalization of cardiac function. Although very rare, neurogenic tumours may be involved in the development of a dilated cardiomyopathy in the infant and child.
Cardiac anomalies associated with congenital absence of the portal vein
Authors: Massin Martial , Verloes Alain , Jamblin Paul 
Published: August 2008

AbstractThe authors discovered congenital absence of the portal vein, with visceral venous return to the right atrium, in a 5-year-old girl with aortic valvar stenosis. Interestingly, of the 19 patients, it was discovered that 11 reported with portal venous agenesis also had cardiac defects. We have, therefore, investigated the hypothesis that the congenital absence of the portal vein and the associated cardiac malformations may result from a similar embryologic insult, and that cardiac development may be affected by the systemic diversion of portal venous flow.
The syndrome of pericarditis, arthritis, and camptodactyly: an under-recognized cause of pericardial constriction in children?
Authors: Buendía Alfonso , Attié Fause , Martínez-Lavin Manuel 
Published: August 2008

AbstractThe syndrome encompassing the combination of pericarditis, arthritis, and camptodactyly is a rarely described cause of pericardial constriction in children. It is likely that this association is being under-recognized. We report a new case in which the skeletal abnormalities were subtle. The syndrome should be included in the differential diagnosis of any child with persistent non-inflammatory pericardial effusion. A careful search at the bedside for the associated skeletal abnormalities should lead to the correct diagnosis. Pericardiectomy is the treatment of choice.
Transthoracic 3-dimensional echocardiography in the assessment of subaortic stenosis due to a restrictive ventricular septal defect in double inlet left ventricle with discordant ventriculoarterial connections
Authors: Vogel Michael , Ho S. Yen , Anderson Robert H. , Redingtont Andrew N. 
Published: August 2008

AbstractTo evaluate the accuracy and clinical utility of three-dimensional echocardiography in the assessment of the size and shape of the ventricular septal defect in double inlet left ventricle.MethodsWe validated the technique in an autopsy study, and then performed a clinical investigation. Six autopsied hearts were immersed in a waterbath and examined with 3-dimensional echocardiography. We identified the cross-section within the dataset which optimally displayed the ventricular septal defect “en face”, and compared its smallest and largest diameters, as well as its area. The ventricular septal defect was then filled with a silicone sealant and a section prepared for direct measurement. In patients, we measured the diameters and area of the ventricular septal defect in endsystole nad computed the aortic valvar area in endsystole from the cross-section showing the aortic valve “en face”. Ten patients with double inlet left ventricle, aged between 2 and 15 years, were studied using rotational or parallel scanning. All patients had undergone banding of the pulmonary trunk at a mean age of 7 (3–36) days, usually at the time of repair of the coarctation. Two patients had undergone surgical enlargement of the ventricular septal defect prior to echocardiographic examination.ResultsThe correlation between the areas of the ventricular septal defect in the specimens measured directly and by 3-dimensional echocardiography was r=0.98, with limits of agreement between −0.1– 0.08 cm2. In the patients, the area of the defect was measured as 3.9±2 cm2, whereas the aortic valvar area was 2.6±0.9 cm2. The ratio between the areas was 1.5 (0.5–2.3). Three patients with areas of the ventricular septal defect smaller than those of the aortic valve had resting Doppler gradients between double inlet left ventricle and the aorta of 16, 20 and 30 mm Hgs, respectively.Conclusions3-dimensional echocardiography provides accurate assessment of the area of the ventricular septal defect in double inlet left ventricle, and is helpful in identifying patients with subaortic stenosis caused by restrictive defects.
Normal values of signal-averaged electrocardiographic parameters and QT dispersion in infants and children
Authors: Vialle Emmanuelle , Albalkhi Rim , Zimmerman Marc , Friedli Béat 
Published: August 2008

AbstractVentricular late potentials, and dispersion of the QT interval, are markers for risk of ventricular arrhythmias. Normal values for these parameters are well established in adults, but may not apply for children. This study has investigated the age dependency of signal averaged electrocardiographic parameters and QT dispersion in 111 normal children aged from 5 days to 16 years. The results indicate that parameters change with age: duration of filtered QRS and low amplitude (< 40 μV) terminal signal increase with age, especially in the younger patients. Filtered QRS is 88.9 ± 7.87 ms in infants, and increases to 108.7 ± 8.51 in teenagers (p<0.001). Low amplitude terminal signals are 17.0 ± 3.44 ms in infants, and 24.5 ± 5.64 ms in teenagers (p< 0.001). Root mean square of the last 40 ms decreases with age, but remains stable after the age of 10 years (122.4 ± 33.30 μV in infants, 60.9 ± 31.27 in teenagers,p<0.001). QT dispersion, on the other hand, does not change significantly with age. The mean value for the whole group is 36 ± 13.7 ms. A weak but significant correlation exists between QT dispersion and filtered QRS. Thus, age must be taken into consideration when interpreting signal-averaged electrocardiograms, but not when measuring QT dispersion.