bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ebstein's malformation with covered defect of the ventricular septum
Authors: Franke Andreas , Mühler Eberhard G. , Hügel Werner 
Published: August 2008

AbstractThe case of a 27-year-old male with Ebstein's malformation combined with a ventricular septal defect is reported. On its right ventricular side the defect was covered by the dysplastic septal tricuspid leaflet, creating a small chamber in continuity with the left ventricle, but without shunt into the remaining components of the right ventricle. The ventricular septal defect and a patent oval foramen were closed operatively after a cerebral embolic event to eliminate probable sources of thromboemboli preventing the inherent risks of life-long anticoagulation for a young patient. Intraoperative morphology confirmed the preoperative findings.
Absence of the left ventricular myocardium in a newborn
Authors: Lindinger Angelika , Masur Yvonne , Limbach Hans-Gerhard 
Published: August 2008

SummaryAbsence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.
Propafenone associated granulocytopenia in a newborn
Authors: Paul Thomas , Luhmer Ingrid , Reiter Alfred 
Published: August 2008

AbstractProgressive granulocytopenia (neutrophil count <1000/ml) developed in a newborn during treatment with propafenone (200 mg/m2) for recurrent paroxysmal supraventricular tachycardia. The total white blood cell count, however, remained within normal limits. The neutrophil count increased gradually after propafenone had been stopped. The time course of treatment and the development of granulocytopenia make it very likely, but not certain, that propafenone was the responsible agent. Pediatricians, and especially pediatric cardiologists, should be aware of this rare adverse effect when they consider treatment with propafenone in children with cardiac arrhythmias. Analysis of the white cell blood count, including a differential count, is recommended in all children in order to assess the true incidence of this adverse effect.
Hypoplastic left heart syndrome: multiprofessional follow-up in the mid-term following palliative procedures
Authors: Hagemo Petter S. , Rasmussen Magnhild , Bryhn Grete , Vandvik Inger Helena 
Published: August 2008

AbstractOf the 12 children who represent the complete cohort of Norwegian patients surviving the complete palliative operative programme for hypoplastic left heart syndrome, 10 were studied by a pediatric cardiologist, a pediatric neurologist, a neuropsychologist and a child psychiatrist. We found significant morbidity. Hemodynamic function was relatively uncompromised in eight of the patients, but one had significant cyanosis due to use of an operative technique subsequently abandoned and one had obstruction of the flow of blood from the left to the right atrium. Five children had disturbances of rhythm, with one requiring insertion of a pacemaker. One child had epilepsy. Delayed motor development, most often to mild degree, was found in seven children, while six had attention problems. Mental retardation was found in two children and autistic traits in one. Despite these various problems, the parents were seemingly satisfied with their own lives, and the quality of life of their children.
A prospective, randomised trial evaluating transatrial and transventricular approaches to repair of tetralogy of Fallot
Authors: Kaushal Sunil K. , Sharma Rajesh , Iyer Krishna S. , Kothari Shyam Sunder , Venugopal Panangipalli 
Published: August 2008

AbstractThe traditional approach to repair of tetralogy of Fallot involves a right ventriculotomy for closure of ventricular septal defect. During the past two decades, reports of progressive right ventricular dilation and dysfunction, and late occurrence of ventricular arrhythmias, have led investigators to re-evaluate this approach and advocate instead the transatrial-transpulmonary approach, hoping to preserve global right ventricular function. We studied the short term effects on right ventricular function of either of the two approaches through a prospective randomised study, involving two comparable groups of patients operated in the same time frame.Between June 1993 and February 1994, 40 patients having tetralogy of Fallot with comparable preoperative characteristics, were assigned randomly to each of two groups for surgical correction.In 20 patients, correction was achieved via the transatrial-transpulmonary route. In the other 20 patients, transventricular correction was the chosen option. Six months after surgery, patients were evaluated clinically, by Doppler echocardiography, cardiac catheterisation, first pass radionuclide angiography and by 24 hours ambulatory electrocardiographic monitoring, taking note of hemodynamics, abnormalities in rhythm, and global right ventricular function.There were no early deaths or morbidity in either group. Mean immediate postoperative ratio between peak right ventricular and systemic pressures was 0.62 ± 0.22 after transatrial and 0.70 ± 0.007 after transventricular correction. All patients were in functional class I. Six months after surgery the mean ratio between peak ventricular pressures was similar in the two groups (transatrial group: 0.37 ± 0.02, transventricular group: 0.38 ( 0.01), but significantly lower than that measured in the operating room. There were no significant arrythmias in either group. Mean right ventricular ejection fraction was nearly the same in both groups (transatrial group versus transventricular group; 44.83 ± 5.65% versus 42.37 ± 8.70%). Significant global hypokinesia of the right ventricle was documented in three patients, and mild hypokinesia in another three, undergoing repair through the transventricular route while in the group undergoing transatrial repair only one patient had mild hypokinesia.We conclude that comparable hemodynamic results are obtained on short term follow-up after repair of tetralogy of Fallot by either the transatrial or transventricular route. Although more patients in the transventricular group were found to have global hypokinesia of the right ventricle, longer follow-up is necessary to establish the clinical relevence of these findings.
Mechanical properties of the pulmonary arteries after the arterial switch operation for complete transposition
Authors: Nakanishi Toshio , Momoi Nobuo , Kobayashi Hirohide , Nishikawa Toshio , Nakazawa Makoto , Imai Yasuharu , Momma Kazuo 
Published: August 2008

AbstractThis study was designed to determine, first, the stiffness of the pulmonary arteries and, second, the relationship between the stiffness of the pulmonary arteries and the success rate of balloon angioplasty in patients with complete transposition after an arterial switch operation. Indexes of pulmonary arterial wall stiffness, percent change in the radius during a cardiac cycle and the pressure elastic modulus, were calculated from the pulmonary arterial pressure and radius measured from a cineangiogram. Of the patients, 13 had no significant stenosis, while 25 had significant stenoses and, therefore, underwent balloon angioplasty. In all, we dilated 33 stenotic lesions. In the presence or absence of postoperative pulmonary stenosis, the percent change in the radius of the pulmonary trunk was significantly less than the normal value. The pressure elastic modulus of the trunk and its branches was significantly greater than normal, and increased significantly with time after the operation. In patients without pulmonary stenosis, the systolic pulmonary arterial pressure increased concomitantly with the pressure elastic modulus. Balloon dilation was successful at 17 locations and unsuccessful at 16 locations. The percent change in radius was significantly less for the failures than for the successes. Prior to 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2at most locations and balloon dilation was successful in 88%. More than 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2in 11 of 15 locations, and balloon dilation was successful in only one location. The increased stiffness of the pulmonary artery may result in increased pulmonary systolic pressure in patients without pulmonary stenosis, and result in a low success rate for balloon angioplasty in patients with pulmonary stenosis.
Interventional closure of atrial septal defects with the Amplatzer® device: first clinical experience
Authors: Bjørnstad Per G. , Masura Jozef , Thaulow Erik , Smevik Bjarne , Michelsen Svein S. , Tønnessen Tor Inge , Seem Egil , Fosse Erik 
Published: August 2008

AbstractWe report our initial experience in three children with percutaneous transvenous closure of significant atrial septal defects. A newly developed one-piece nitinol device is delivered through a long venous sheath of 7 French calibre under fluoroscopic and transoesophageal echocardiographic monitoring. Complete closure of all defects was verified by echocardiography on the morning after the procedure. No complication occurred. In the first two patients, the device slipped sideways into the defect. This was easily discovered and corrected. Our experience shows that the Amplatzer® device is reliable, easy to implant, and presents very clearly on transoesophageal echocardiography and fluoroscopy. This makes implantation a controlled procedure. Until release, the device can easily be retracted into the sheath at any time and removed. The results suggest that closure of an atrial septal defect with this device is feasible, rapid, and safe.
An institutional experience with the bidirectional cavopulmonary shunt: do we know enough about it?
Authors: Reddy V. Mohan , McElhinney Doff B. , Moore Phillip , Bristow James , Haas Gary S. , Hanley Frank L. 
Published: August 2008

AbstractBackgroundThe bidirectional cavopulmonary shunt has become a mainstay in the palliation of patients with a functionally single ventricle. Despite its usefulness, there are a number of concerns following its construction, including issues of timing, the development of pulmonary arteriovenous fistulas, and growth of the pulmonary arteries.MethodsBetween January 1990 and April 1996, we constructed a bidirectional cavopulmonary shunt in 123 patients. Median age at surgery was 9.4 months, and ranged from 24 days to 43 years. In 25 patients (20%), it was the first operative procedure. An auxiliary source of pulmonary blood flow was included in 72 patients (58%).ResultsSix patients (4.9%) died in the early postoperative period, and the overall early rate of failure (death or take-down) was 8.1%(n=10). By multivariate analysis, longer bypass time (p=0.012), age less than 1 month (p=0.03), and higher pulmonary vascular resistance (p = 0.043) were significant. Early reoperation was performed in 11 other patients to decrease (n=8) or increase (n=3) the amount of pulmonary blood flow. Early survivors were followed for a median of 15 months (range: 1 to 48 months), during which time 5 patients died and a Fontan circulation was completed in 30. Including early and late mortality, actuarial survival rates at 1 and 2 years were 91% and 88%, respectively. Among hospital survivors, the only significant predictor of poorer survival by Cox regression was age less than 2 months at the time of the initial cavopulmonary shunt (p=0.02). Atrioventricular valvar regurgitation was decreased after construction of the cavopulmonary shunt in 17 of the 28 patients (61%) in whom echocardiographic data were available to compare preoperative and postoperative status.ConclusionsThe bidirectional cavopulmonary shunt is a useful procedure in the early or intermediate term management of patients with a functionally univentricular heart. Much remains to be learned, nonetheless, about this unique physiologic system. The role of accessory pulmonary blood flow remains unclear, as does the use of the shunt as long-term palliation. Pulmonary arteriovenous fistulas are a serious concern, especially in young patients with heterotaxy syndrome (isomeric atrial appendages).
How classical are the clinical features of the “ostium secundum” atrial septal defect?
Authors: Tabery Stefania , Daniëls Otto 
Published: August 2008

AbstractSome patients with so called “secundum” atrial septal defect within the oval fossa show a large defect on the echocardiogram, although they do not have all the classical clinical features. Until now, a large atrial septal defect was thought to be characterized by a large shunt (functional defect). Experience indicates, nonetheless, that such large defects in size (anatomical defect) are not always accompanied by large shunts. In order to assess how often the classical clinical features of an atrial septal defect exist, and to investigate whether the surgical indication for closure of the defect are anatomical or functional, we carried out a retrospective study. We evaluated the records of 161 patients, with birth dates from 1973 to 1994 (age between 0 and 21 years), so as to study the classical clinical features (history, physical examination, electrocardiogram, chest X-ray, echo-Doppler studies, cardiac catheterization, surgery). Only patients with an atrial septal defect found in isolation were included.We discovered that the classical clinical features are often not present when there is a large defect, be it functional or anatomical. The anticipated features are present in roughly two-thirds of the patients. There is also a discrepancy between the anatomical and the functional sizes of the defect. This finding creates a fundamental problem for the future, namely the indications fot closure of atrial septal defects within the oval fossa. Further studies are needed to answer this important question
Cross-sectional echocardiographic determination of right ventricular volume and myocardial mass in children. Normal values and values in children with interatrial communication
Authors: Jin Zhen , Briedigkeit Walter , Gamillscheg Andreas , Berger Felix , Skinner Jonathan R , Golshahi Farideh , Uhlemann Frank , Lange Peter E. 
Published: August 2008

AbstractCross-sectional echocardiography was performed on 108 healthy children (7 days – 17 years old) and 55 children (6 months - 16.5 years old) with interatrial communication. Right ventricular end-diastolic volume, end-systolic volume, stroke volume, ejection fraction, muscle volume, and the ratio of muscle to cavity were calculated on the basis of outlined cavity and myocardium of an apical fourchamber view.In the normal subjects right ventricular end-diastolic volume, end-systolic volume, stroke volume and muscle volume correlated with body surface area (end-diastolic volume: y=12.5x+7.8x2, r=0.99; end-systolic volume: y=4.8x+3.6x2, r=0.98; stroke volume: y=7.7x+4.2x2, r=0.98; muscle volume: y=14.1x+2.9x2, r=0.97), muscle/cavity ratio (0.85±0.17) and ejection fraction (58.9 ± 6.2%) were unrelated to body surface area. In the subjects with interatrial communication, the right ventricular volumes were significantly larger (p<0.001) than the normal values with a linear relationship to the ratio of pulmonary to systemic flows.Right ventricular volumes can be determined in normal children with acceptable repeatability using a standard apical four-chamber view. The growth related normal values provide a basis for future quantitative studies.
Transcatheter embolization of abnormal intrathoracic vessels using coils in the setting of children with congenital heart disease
Authors: Pfammatter Jean Pierre , Luhmer Ingrid , Kallfelz Hans Carlo 
Published: August 2008

AbstractObjectiveit was the purpose of this retrospective study to assess the efficacy and the rate of complications of transcatheter embolization of abnormal intrathoracic vessels using coils in children with complex congenital heart disease.Patients and methodsin 17 children (mean age 9.2±5.9 years) with complex congenital heart disease, occlusion by catheter intervention was attempted in 29 abnormal intrathoracic vessels: 13 aorto-pulmonary collaterals, 12 arterio-pulmonary collaterals, 2 systemic arteries supplying pulmonary sequestrations, one central venous connection and a Blalock-Taussig shunt. The mean diameter of the vessels was 4.7 ± 1.6 mm (range 2–8 mm). Steel coils with a helical diameter of three, five or eight mm were used. After selective catheterization of the vessel, they were delivered through a 5 F endhole catheter. The helical diameter of the coils was chosen in order to exceed the inner diameter of the vessel by 10–30%.Resultsas assessed by selective angiography performed 10 minutes or more after release of the coil, 27 of the 29 vessels (93%) were successfully occluded. A mean of 2.6 coils (range 1–11) were necessary for successful occlusion. Complications were encountered during 4 attempts of occlusion (14%). Of a total of 76 coils delivered, 4 coils (5%) secondarily migrated after release from the catheter, mostly to branches of the pulmonary arteries. This complication was seen predominantly in those vessels with the largest diameters. Three of the dislocated coils were left in place as they did not significantly obstruct flow of blood. One coil was retrieved by a basket catheter.Conclusiontranscatheter embolization of abnormal intrathoracic vessels using coils in children with congenital heart disease is an effective therapy. Potential complications warrant careful evaluation of the indications for these procedures.
Three-dimensional echocardiographic assessment of right ventricular volume and function in patients with pulmonary hypertension
Authors: Apfel Howard D. , Shen Zhanqing , Boxt Lawrence M. , Barst Robyn J. , Gopal Aasha S. , Allan Lindsey D. , Gersony Welton M. , King Donald L. 
Published: August 2008

AbsractThe structural complexity of the right ventricle has made quantitative evaluation difficult. Conventional cross-sectional echocardiographic methods are limited by geometric assumptions and the position of the planes used for imaging. Previous reports have demonstrated accurate three-dimensional echocardiographic quantitation of the right ventricle in-vitro and in experimental animals. We adapted a previously described method for three-dimensional reconstruction of the left ventricle to compute right ventricular volume and ejection fraction in a clinical setting.We examined 29 patients aged from 2 to 42 years with pulmonary hypertension, by three-dimensional echocardiography and resonance imaging. Correlation and agreement were calculated for volumes and ejection fractions. Three-dimensional echocardiographic reconstruction, when compared to resonance imaging, yielded r values of 0.95 and 0.93, and mean differences (bias) of 31% ± 19% and 33% ± 18%, for systolic and diastolic volumes respectively. Interobserver variability was low (12.9% and 8.0%). Ejection fraction as calculated by three-dimensional echocardiography showed close agreement with resonance images (bias=l% ±7%). Three dimensional echocardiography is now a method of measuring right ventricular ejection fraction in the clinical setting which produces results comparable to those of resonance imaging. Volume measurements correlated well for systole and diastole, but consistently underestimated values produced from resonance images.
Congenital bifid origin of right coronary artery co-existing with congenital right coronary to right ventricular fistula
Authors: Ramirez-Marroquin Samuel , Santibañez-Salgado Alfredo , Calderon-Colmenero Juan 
Published: August 2008

AbstractAnomalous origin of the coronary arteries is a well-known phenomenon, but is less well reported in world literature. We present our findings in a one year old boy in whom there was bifid, or “y”, origin of the right coronary artery, co-existing with a fistula from the right coronary artery which opened into the right ventricle.
Parachute tricuspid valve, supravalvar tricuspid ring, and coarctation of aorta in congenitally corrected transposition
Authors: Godart François , Piot Dominique , Rey Christian 
Published: August 2008

AbstractWe describe an unusual case of congenitally corrected transposition in which left-sided obstructions included a supravalvar tricuspid ring, a parachute deformity of the valve itself, and coarctation of the aorta. Surgical correction was performed in 3 stages. Initially we repaired the coarctation and banded the pulmonary trunk. Then we removed the band and, finally, attempted unsuccessfully to replace the tricuspid valve. When coarctation of the aorta occurs in congenitally corrected transposition, it is always wise to search carefully for abnormalities of the tricuspid valve.
Leaking aortic aneurysm after repair of coarctation of the aorta: the significance of haemoptysis and magnetic resonance imaging
Authors: Lo S. S. S. , Kilner P. J. , Somerville J. 
Published: August 2008

AbstractWe discuss a patient with recoarctation of the aorta who, 4 weeks after reoperation, presented with fresh haemoptysis and was subsequently found at delayed emergency surgery to have a leaking false aortic aneurysm. The case emphasizes the significance of haemoptysis following repair of recoarctation, the difficulty of reoperation due to abnormalities of the aortic wall, and the value of serial magnetic resonance imaging in demonstrating regions of different signal intensity from haemorrhages of varying age around a leaking false aortic aneurysm.
Conversion of the modified Fontan operation to total cavopulmonary connection: a treatment for protein-losing enteropathy
Authors: Stafanger Gerd , Pettersson Gösta , Jacobsen Joes Ramsøe 
Published: August 2008

AbstractWe describe a patient with tricuspid atresia who, after a Fontan operation three years earlier, presented with severe protein-losing enteropathy. After conversion to a cavopulmonary connection, the symptoms regressed and substitution with albumin and immunoglobulin was no longer required.
Where's the spleen? Looking for the spleen and assessing its function in the syndromes of isomerism
Authors: Phoon Colin Kit Lun 
Published: August 2008

AbstractDespite the close association of certain constellations of cardiac malformations with splenic status (right isomerism / asplenia, left isomerism / polysplenia), the association is not perfect. Given the risk of infection in the patient lacking splenic tissue, it seems prudent to assess the state of the spleen separately from the cardiovascular system, in order to decide on antimicrobial prophylaxis. A wide variety of tests is available to assess splenic function and/or anatomy. Counting pitted erythrocytes or Howell-Jolly bodies in the peripheral blood smear can be a good indicator of the lack of splenic function, although the former appears more sensitive and quantitative than the latter. Radionuclide spleen scans can provide both anatomic and functional information about the spleen, and may be a useful adjunctive test. Relatively poor spatial resolution, and interfering hepatic activity (even with spleen-specific scans), mitigate its usefulness in the young infant. The reliability of computed tomography and magnetic resonance imaging in detecting splenic tissue in the heterotaxy syndromes is unproven and perhaps only fair at best. They are probably most helpful as adjunctive tests. Ultrasonography offers excellent spatial resolution, portability, and lack of ionizing radiation, but is operator- and patient-dependent. Nonetheless, ultrasonography, coupled with an examination of the peripheral blood for Howell-Jolly bodies or pitted erythrocytes, is probably the set of tests with highest sensitivity and greatest convenience. Management strategies for the asplenic patient are also discussed.