bluuebunny/crossref_metadata_2025_split huggingface dataset:
A possible role for steroid therapy in preventing postoperative stenosis in totally anomalous pulmonary venous connection
Authors: Sands Andrew , Craig Brian , Casey Frank 
Published: August 2008

AbstractWe report three cases of pulmonary venous stenosis developing after repair of totally anomalous pulmonary venous connection. In each case, we used steroids as a therapeutic measure following re-operation. In two cases, we were successful in preventing significant re-stenosis. The third patient rapidly developed stenosis (mean pulmonary wedge pressure approximately 20 mmHg at cardiac catheterisation) after stopping steroids. This patient subsequently died after a third operation. The remaining patients continue to do well.
Persistence of the hepatic venous plexus with underdevelopment of the inferior caval vein: implications in the management of complex congenital heart disease
Authors: Gladman Gordon , Adatia Ian , Freedom Robert M. 
Published: August 2008

SummaryWe report two cases with persistence of the hepatic venous complex, underdevelopment of the inferior caval vein and azygos continuation to the superior caval vein. We suggest that the hepatic venous complex is a true congenital malformation rather than the result of passive enlargement of venous channels subsequent to high venous pressures. Preoperative diagnosis is important in patients with complex congenital heart disease undergoing palliative venous redirection procedures if a postoperative right-to-left shunt and cyanosis are to be avoided.
Disparity between the arrangement of the abdominal vessels and the bronchial tree and atrial appendages in a patient with mirror-imaged venous return
Authors: Soukias Nikos D. , Shinebourne Elliot A. , Carvalho Julene S. 
Published: August 2008

SummaryEither arrangement of the abdominal vessels or the morphology of the bronchial tree are usually used to predict atrial arrangement. We report a patient in whom, in the setting of normal physiology as seen with mirror-imaged venous return, and due to disparity between the arrangement of the abdominal vessels (suggestive of right isomerism) and thoracic arrangement (indicative for left isomerism), atrial arrangement could not be inferred. Direct echocardiographic imaging of both appendages revealed left isomerism.
Mitral valvar prolapse and regurgitation combined with aortic regurgitation in a child with Sanfilippo syndrome type A
Authors: Alturjuman Ahmad , Mehta Ashok V. 
Published: August 2008

SummaryCardiovascular involvement is commonly reported in various muco- polysaccharidoses. We report a first case of Sanfilippo syndrome type A in a 12-year-old white female who has developed combined progressive mitral valvar regurgitation due to prolapse and aortic regurgitation.
Complete surgical resection of intrapericardial teratoma in a neonate with compression of the central airways
Authors: Sievers Roy F. B. , Tang Augustine T. M. , Haw Marcus P. 
Published: August 2008

AbstractIntrapericardial teratoma is a rare but recognised cause of respiratory distress in neonates. Patients often present with the compressive effects of the mass within the thorax. Prompt diagnosis should be followed swiftly by surgical resection. We report an unusual case of intrapericardial teratoma in a neonate presenting with collapse of the lung which was successfully treated by surgery.
Spectrum and influence of hypoplasia of the left heart in Neonatal aortic coarctation
Authors: Tani Lloyd Y. , Minich L. Luann , Hawkins John A. , McGough Edwin C. , Pagotto Luciana T. , Orsmond Garth S. , Shaddy Robert E. 
Published: August 2008

AbstractObstruction of the left ventricular outflow tract may be associated with hypoplasia of the left heart, which importantly influences the options for treatment. Although the influence of the size of the left heart on the outcome for critical aortic stenosis has been described, less is known about the spectrum of such hypoplasia seen with neonatal aortic coarctation, and how this influences outcome. To determine, first, the spectrum and influence of hypoplasia of the left heart in neonatal coarctation, second, if the previously described critical values for adequacy of the left heart in neonates with critical aortic stenosis are applicable to neonates with coarctation, and, third, if any of the variables or associated abnormalities are risk factors for recoarctation, we studied 63 neonates who underwent repair of coarctation. From the initial echocar diogram, we measured multiple structures in the left heart, and calculated a score for adequacy as has been done for critical aortic stenosis. The sizes were compared to previously reported minimal values. We then analyzed the influence of the variables and the associated anomalies on outcome. There were no deaths. There was a broad spectrum of sizes that did not correlate with the need for re-intervention. The calculated score for adequacy would have predicted survival in only 56% of the patients, and 73% of the neonates had at least one parameter measured in the left heart below the previously reported minimal values. There is, therefore, a broad spectrum of sizes for the left heart in neonates with aortic coarctation that is not predictive of outcome. Minimal sizes, and the score for adequacy used for critical aortic stenosis, are not applicable to neonates with coarctation.
Impact of dynamic 3D transoesoesophageal echoardiography in the assessment of atrial septal defects and occlusion by the double-umbrealla device(CardioSEAL)
Authors: Maeno Yasuki V. , Benson Lee N. , Boutin Christine 
Published: August 2008

AbstractOcclusion of the atrial septal defects in the oval fossa by interventional catheterization has progressed, but still has limitations. Three-dimensional (3D) echocardiography can provide unique views unavailable by cross-sectional imaging. The objective of this study was to define the clinical application of 3D echocardiography in the assessment and monitoring of transcatheter occlusion of atrial septal defects. Three-dimensional echocardiography was attempted prior to occlusion of atrial septal defects in 41 patients (median age 8.6 years). Serial cross-sectional images were acquired by multiplane transoesophageal echocardiography and displayed by means of computer reconstruction. Dynamic 3D echocardiographic images of defects in the oval fossa were obtained in 40 of 41 patients (98%). Volume-rendering demonstrated the anterosuperior rim in 36 (90%) and the inferoposterior rim in 24 (60%), but failed to reveal small additional fenestrations in six. Sizes measured by 3D echocardiography were significantly larger than those provided by cross-sectional transoesophageal echocardiography (p =;0.007), but differed little from those obtained with balloon sizing (p =; 0.6). After occlusion, 3D echocardiography showed positions of all arms of the device in 20 of 24 cases. Location of any protruding arms, or residual defects, were also clearly revealed.Three-dimensional images obtained in 12 patients during deployment of the double-umbrella device were useful in monitoring its position (single-frame) and for explaining the mechanism of protrusion. Current 3D echocardiography provides clinically relevant information for selection of patients for closure of atrial septal defects by interventional catheterization and when monitoring during implantation. Information obtained by this technique can clarify the mechanism of deployment of the device and closure of the defect, therefore influencing outcomes.
Resonance imaging of a ruptured aneurysm of the sinus of Valsalva
Authors: Roche Kevin J. , Genieser Nancy B. , Ambrosino Michael M. 
Published: August 2008

AbstractRuptured aneurysm of an aortic sinus of Valsalva is a rare cause of left-to-right shunting. We show how resonance imaging can be used to make the diagnosis. This technique can successfully characterize the shunt as well as determine the presence of associated anomalies, such as ventricular septation and aortic regurgitation. It may be the only study required prior to therapeutic intervention.
Superior caval venous syndrome after atrial switch procedure: relief of complete venous obstruction by gradual angioplasty and placement of stents
Authors: Michel-Behnke Ina , Hagel Karl-Jürgen , Bauer Jürgen , Schranz Dietmar 
Published: August 2008

AbstractSuperior caval venous syndrome is one of the late problems known to occur after Mustard repair of complete transposition. Reoperation may leave residual stenosis, and carries substantial risk for the patient. It is now feasible to use intravascular stents to overcome systemic venous baffle obstructions, and such an approach is probably more effective. The purpose of our study therefore, was to assess immediate and medium term results of inserting stents subsequent to gradual balloon enlargement of acquired atresia of the intraatrial baffle in patients who had undergone an atrial switch operation. We investigated five patients with complete obstruction of the superior caval venous pathway at perforation of the atretic segment was achieved using a guide wire technique. The procedure was successful in all patients. Gradual angioplasty was performed and intravascular stents were implanted. The pressure in the superior caval vein dropped to normal values, symptoms improved, and the patency of the newly created venoatrial communication was proven at mid-term follow-up. Thus critical obstructions at the superior caval venous pathway after the Mustard procedure can be reopened by interventional catheterization. Implantation of balloon-expandable intravascular stents is safe and effective in the acute relief of the obstructions, but careful long-term follow-up is mandatory.
Congenitally corrected transposition with atrioventricular septal defect
Authors: Attie Fause , Iturralde Pedro , Zabal Carlos , Rijlaarsdam Maria , Buendia Alfonso , Colin Luis , Vargas-Barron Jesus , Muñoz-Castellanos Luis 
Published: August 2008

AbstractWe describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement. All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ven-triculography.In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the pos terior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulationThe clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventric ular valves.
Echocardiographic follow-up of percutaneous closure of the arterial duct in infants
Authors: Piñeda Luis Fernández , Torrealday Hugo , Cañete Ramón Bermúdez , Azcárate María J. Maitre , Jiménez Manuel Quero 
Published: August 2008

SummarySince February 1990, we have attempted nonsurgical occlusion of persistent patency of the arterial duct using the Rashkind double-disk occluding device, of either 12 or 17 mm diameter. Results are presented from 31 patients (23 female and eight male, ages 15 months to 16 years). M-Mode, cross-sectional, Doppler and color Doppler echocardiographic studies were made prior to closure, in the following 24 hours, and six months later. Left ventricular diastolic dimension and shortening fraction, systolic intervals, and left atrium/aortic ratio were evaluated by M-Mode echo. We examined carefully the location of the device relative to the left pulmonary artery and descending aorta, also checking for disturbed flows and residual shunts. These studies showed a clear tendency for the left ventricular diameter to decrease, and revealed a significant normalization of the left atrium/aortic ratio. Successful closure of the duct was achieved in 27 cases. Follow-up studies six months later showed residual shunting in four cases. In one patient, a second device was implanted with an excellent result. At the immediate follow-up, turbulences were noted in the area of the device (nine cases) and in the left pulmonary artery (seven cases). Distortion of the anatomic orientation of the left pulmonary artery was observed in three cases in which we discovered 20 mm Hg systolic gradients. In our opinion, echocardiography is the best technique with which to follow-up these patients. The high sensitivity of color Doppler echocardiography revealed minimal distortion of the left pulmonary artery, probably generated by regional anatomic adjustment to the insertion of the device.
Echocardiographic diagnosis of inferior sinus venosus defects
Authors: Ettedgui José A. , Siewers Ralph D. , Zuberbuhler James R. , Anderson Robert H. 
Published: August 2008

AbstractSinus venosus defects are relatively uncommon forms of interatrial communication in which one of the caval veins has a biatrial connection, and partial anomalous pulmonary venous connections are the rule. The variety related to the inferior caval vein is much the rarer of the two forms, and here the defect is posterior and inferiorly placed. To determine whether an inferior sinus venosus defect could be identified preoperatively, we reviewed retrospectively the available echocardiograms and cineangiograms of the five patients with an inferior sinus venosus defect who underwent surgical repair between January 1983 and December 1991. A cross-sectional echocardiogram had been performed preoperatively in four of these patients, but the correct diagnosis had been made in only one case. Retrospective study, however, showed that the extent of the true atrial septum and the nature of the junction of the inferior caval vein with the atriums could be evaluated from the subcostal position in three of these four patients. Each of the three had an interatrial communication located posteriorly and inferiorly adjacent to the atrial connection of the inferior caval vein, along with abnormally connected right pulmonary veins. The oval fossa was intact and the coronary sinus was normal in all. Cardiac catheterization had been performed in three of the five patients. Anomalous return of right pulmonary veins was noted in each, and the posterior and inferior location of the interatrial communication was evident in two. An interatrial communication adjacent to the atrial connection of the inferior caval vein, along with anomalous connection of the right-sided pulmonary veins, are the diagnostic features of the inferior variety of sinus venosus defects. These features can be demonstrated by cross-sectional echocardiography from the subcostal position.
Primary pediatric cardiac tumors: a 17 year experience
Authors: Sallee Denver , Spector Michael L. , van Heeckeren Daniel W. , Patel Chandrakant R. 
Published: August 2008

AbstractWe reviewed 22 cases of primary pediatric cardiac tumors followed at our institution from January 1981 through November 1997, analyzing them by subtype, age and manner of presentation, location, associated findings, interventions, and clinical course. Rhabdomyomas were the most common (11), followed by intrapericardial teratomas (2), myxomas (1), fibromas (1), hemangiomas (1), mesotheliomas (1), and rhabdomyosarcomas (1), with 4 undetermined tumors. The majority (77%) of tumors were diagnosed before the age of one year, including six prenatally. The most common presentations were murmurs (5), arrhythmias (5), and abnormal screening fetal ultrasound examinations (4). Tumors were located most frequently in the right ventricle (13) and left ventricle (7), with multiple tumors being present in 10 cases. Eight patients (36%) had associated arrhythmias or conduction abnormalities, and of the 11 patients with rhabdomyomas, tuberous sclerosis was diagnosed in 8. Eight patients underwent cardiac catheterization, including two for electrophysiologic study with radiofrequency ablation, and seven patients had complete or partial tumor resection. The follow-up period ranged from 2 months to 15 years, and there were 3 tumor-related deaths. Therefore, despite the benign histology of most primary pediatric cardiac tumors, there may be significant associated morbidity and occasional mortality. As echocardiographic techniques such as fetal ultrasonography have continued to improve, however, these cardiac tumors have increasingly been detected early before significant symptoms develop.
Thrombotic occlusion of the main stem of the left coronary artery in a neonate
Authors: Abdurrahman Luby , Schwartz Steven M. , Beekman Robert H. 
Published: August 2008

AbstractThrombotic coronary arterial occlusion, and myocardial infarction, are rare in the newborn. We report such a happening presenting shortly after birth with cardiogenic shock, no left ventricular output and a systemic circulation dependent on flow from a patent arterial duct.
Simultaneous surgical treatment of atrial septal defect and atrial flutter using a simple modification of the atrial incision
Authors: Henglein Dagmar , Cauchemez Bruno , Bloch Gérard 
Published: August 2008

AbstractThe reentrant circuit of common atrial flutter is known to located in the right atrium between two anatomical barriers. Recent electrophysiologic studies have defined the tricuspid annulus as the anterior barrier, and the terminal crest and its continuation as the eustachian ridge as the posterior barrier. Construction of a bidirectional block to conduction between these two barriers by means of lesions created with radiofrequency current have been shown to be effective in ablating the flutter. We now find that surgical creation of such a block to conduction between the barriers by a simple modification of the atrial incision line is equally effective. In a 6-year-old boy, who was admitted to our hospital for closure of an atrial septal defect and treatment of sustained atrial flutter, the atriotomy was performed perpendicular to the terminal groove and extended towards the tricuspid annulus, placing some crythermal lesions between the end of the incision and the annuals. The special defect was closed using a Dacron patch. The child was free of arrhythmia both during the postoperative stay and over the initial three months of follow-up. We conclude that this simple modification of the atrial incision line provides cure of atrial flutter in children who require atriotomy for repair of congential cardiac anomalies. It may also be beneficial in preventing ‘incisional’ reentrant tachycardia.
Self-efficacy and physical activity in adolescents with trivial, mild, or moderate congenital cardiac malformations
Authors: Bar-Mor Galit , Bar-Tal Yoram , Krulik Tamar , Zeevi Benjamin 
Published: August 2008

AbstractOur purpose was to examine the cognitive processes that influence involvement in physical activity among 100 adolescents, 55 boys and 45 girls, ranging in age from 12 to 18 years, with trivial, mild, or moderate forms of congenital cardiac disease. We hypothesized, first, that the severity of the congenital cardiac malformation itself has an indirect effect on self-efficacy regarding physical activity, and that the relationship between the two is mediated by the recommendations of the cardiologist and the attitude of the mother. Second, we argued that self-efficacy serves as a mediating variable between the recommendations of the cardiologist and the attitude of the mother, on the one hand, and involvement in physical activity, on the other. The results confirmed both hypotheses. In a population of adolescents with trivial to moderate congenital cardiac malformations, beliefs in self-efficacy, rather than severity of the disease, were the most influential factors in determining whether or not adolescents will engage in sports or other physical activities. We also demonstrated the importance of the role played by the recommendations of the cardiologist in determining both the attitudes of the mother and the belief in self-efficacy of the adolescents.
Cardiac surgery in a girl with trisomy 13
Authors: Strømme Petter , Thaulow Erik , Geiran Odd 
Published: August 2008

AbstractWe describe a profoundly retarded infant girl with multiple anomalies caused by trisomy 13. Due to heart failure, which was resistant to medical treatment, we closed successfully a ventricular septal defect at three months of age. She died at 10 months of age. Despite the short survival, we believe that the patient benefitted significantly from the surgical repair of her cardiac defect.
Patency and development of stenosis in modified Blalock-Taussig shunts constructed using expanded polytetrafluoroethylene
Authors: Godart François , Qureshi Shakeel A. , Simha Ary , Fagg Nuala , Deverall Philip B. , Anderson David , Baker Edward , Tynan Michael 
Published: August 2008

AbstractConstruction of a modified Blalock-Taussig shunt using polytetrafluroethylene is frequently used for palliation in infants with complex cyanotic heart disease. This study was performed to assess angiographically the late patency rate of such prosthetic shunts. A modified Blalock-Taussig shunt had been constructed in 82 children and, of these, 63 (77%) patients had post-operative angiography and were included in this study. There were 29 males and 34 females, ranging in age from 1 day to 164 months. A second shunt was needed in 7 patients. Thus, 70 shunts were studied. Of the shunts, 16 were 4 mm in diameter, 28 shunts 5 mm in diameter, and 26 shunts 6 mm in diameter. Postoperative angiography was performed on 1 day to 132 months (median 39 months) after construction of the shunts. Acute post operative occlusion occurred in 2(3%) patients, who had 5 and 6 mm shunts, respectively. At the latest follow-up (median 39 ± 26 months), 6(9%) late occlusions were noticed. Overall patency was 89% for all the shunts, with 94% of the 4 mm shunts, 85% of the 5 mm and 88% of the 6 mm shunts being patent. Regression analysis showed a progressive reduction in diameter of the graft with time. This narrowing of the graft may have been related to the formation of a ‘neointima’ due to fibrous hyperplasia.
Surgical treatment in infancy of ductal aneurysm associated with a vascular ring
Authors: Hartyánszky István L. , Szolnoki Judit , Mózes Gèza 
Published: August 2008

AbstractA 5-week-old male, born with an aneurysm of the arterial duct forming part of a vascular ring, underwent successful surgery without cardiopulmonary bypass. From our search of the literature, it seems, that this combination of rare congenital malformations has not previously been reported.
Atrioventricular septal defect with tetralogy of Fallot
Authors: Dalvi Bharat , Gupta Krishnagopal , Sharma Satyavan 
Published: August 2008

SummaryWe present a case of atrioventricular septal defect associated with tetralogy of Fallot which was diagnosed by cross-sectional echocardiography and angiography. The diagnosis was confirmed at necropsy. This case is unusual for the absence of Down's syndrome. We discuss the role of clinical, echocardiographic, hemodynamic and angiographic studies.