bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pulmonary atresia with intact ventricular septum: Is it possible to improve survival?
Authors: Vosa Carlo , Arciprete Paolo , Caianiello Giuseppe , Palma Gaetano 
Published: August 2008

SummaryBetween February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.
Staged surgical management of pulmonary atresia with intact ventricular septum
Authors: Battistessa Sergio A. , Jackson Mark , Hamilton David I. , Patel Raman G. , McKay Roxane 
Published: August 2008

AbstractReview of an institutional experience of 81 patients admitted to the Royal Liverpool Children's Hospital between January 1958 and December 1990 with a diagnosis of pulmonary atresia and intact ventricular septum revealed that successful definitive repair was achieved in about one-third of those who underwent surgical treatment. A three-stage protocol, consisting of a systemic-pulmonary anastomosis followed by right ventricular decompression on cardiopulmonary bypass and subsequent separation of the systemic and pulmonary circulations, facilitated a biventricular correction but rarely led to a successful Fontan procedure. Both functional class and probability of survival improved after each stage of management.
Improvement in growth, and levels of insulin-like growth factor-I in the serum, after cavopulmonary connections
Authors: Stenbøg Elisabeth V. , Hjortdal Vibeke E. , Ravn Hanne B. , Skjørbøk Christian , Sørensen Keld E. , Hansen Ole Kromann 
Published: August 2008

AbstractIntroductionThe total cavopulmonary connection, and the bidirectional Glenn anastomosis, are widely used for palliation of patients with complex functionally univentricular hearts. Little attention has been paid to the potential for postoperative growth in children after these operations, which are now performed at increasingly younger age.Material and ResultsPhysical growth, and levels of insulin–like growth factor I in the serum, were measured in 20 patients, aged 11·5 ± 5·6 years, 2 (0·5–6) years after a total cavopulmonary connection in 12, or a Glenn anastomosis in 8. All patients were in functional class I or II of the categorisation of the New York Heart Association, with excellent haemodynamic and angiographic findings. None of the patients had clinical signs of protein losing enteropathy. Controls included 33 healthy children, aged 11·5 ± 2·7 years.Preoperatively, the mean Z-scores for weight and height were negative, −1·1 ± 0·8 and −0·5 ± 1·5. At follow-up, both parameters had improved significantly by 1·1 ± 0·9 and 0·8 ±1·2 percentiles, and Z-scores were comparable between the two groups (p = 0·81 for weight and p = 0·88 for height). No correlations were found between haemodynamics and the improvement in growth noted during follow-up. Increases equal to, or greater than 2 standard deviations for weight and height were seen only in children undergoing surgery before the age of 5 years. A significant correlation between age at operation and improvement in growth, however, could not be found.Levels of growth factor measured in the serum were not statistically different from levels in healthy children for either group of patients (p=0·07 for girls and p=0·37 for boys).ConclusionsPhysical growth improved significantly following the surgical procedures. The concentrations of the growth factor measured in the serum were not different from levels in healthy children, suggesting normal nutritional status in both palliative situations.
Somatic growth failure after the Fontan operation
Authors: Cohen Mitchell I. , Bush David M. , Ferry Robert J. , Spray Thomas L. , Moshang Thomas , Wernovsky Gil , Vetter Victoria L. 
Published: August 2008

AbstractOur study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings
Should we standardise the pre-operative management of babies with complete transposition?
Authors: Hornung T. S. , O'sullivan J. J. 
Published: August 2008

AbstractBackgroundComplete transposition is the most common form of neonatal cyanotic heart disease. The management of this condition has changed markedly in the last decade and there appears to be a significant variation between centres in terms of pre-operative management.Objectives / MethodsWe surveyed all paediatric cardiac surgical centres in the United Kingdom regarding pre-operative management, particularly performance and timing of balloon atrial septostomy and aortogram, imaging techniques used and discharge prior to surgery.ResultsThere is significant variation in pre-operative management: 10 centres now perform septostomy outside the catheter lab and 11 without general anaesthesia. Eight centres use echo control only and only 3 perform routine aortograms. Three centres do not perform routine septostomy. The most common age for arterial switch was at 1–2 weeks, but some routinely performed this procedure up to 1 month of age and others aim for arterial switch before one week of age. Only 3 centres routinely discharge patients between septostomy and switch.ConclusionsDespite a trend towards echo guided septostomy and earlier arterial switch there is still considerable variation in early management of patients with transposition of the great arteries. Debate within the profession leading to a more standardised pre-operative management strategy would protect both the patient and the doctor involved in the care of children with complete transposition.
The anatomy of interatrial communications – what does the interventionist need to know?
Authors: Ferreira Martins José Diogo , Anderson Robert H. 
Published: August 2008

AbstractIncreasingly, the interventional cardiologist is seeking to close interatrial communications by inserting devices by means of catheterisation. So as to optimise these procedures, it is adavantageous to have a firm grasp of the anatomy of the normal atrial septal structures, this then providing the basis to understand the morphology of the holes which can exist between the chambers, not all of which are true septal defects.A true septal structure can be removed without exiting from the cavities of the heart. It is the flap valve of the oval fossa, along with the anterior rim of the fossa, which fulfill this criterion. The remainder of the extensive rim of the normal fossa is no more than an infolding between the walls of the right and left atriums and their venous tributaries, and has different dimensions at various points around the ircumference. The so-called muscular atrioventricular “septum” is a sandwich incorporating a layer of epicardial fibro-adipose tissue. True defects of the atrial septum, therefore, exist because of deficiency, perforation, or absence of the flap valve. Most of these defects will prove suitable for interventional closure, but potential caveats include multiple defects, aneurysm of the flap valve, or adjacency of the fossa to the venous orifices. The other interatrial communications, namely the sinus venosus, coronary sinus, and “ostium primum” defects are outside the confines of the oval fossa. Recognition of this feature is the key to their diagnosis, and their ifferentiation from true atrial septal defects. Of these defects, only the coronary sinus defect is likely to be suitable for device closure, and then only in the very rare circumstances when it is seen in isolation
The role of echocardiography in transcatheter closure of atrial septal defects
Authors: Elzenga Nynke J. 
Published: August 2008

AbstractClosure of so-called “secundum” atrial septal defects with a device inserted on a catheter necessitates precise delineation of their morphology. Echocardiography is the diagnostic method of choice to demonstrate this morphology, and to differentiate such defects located within the oval fossa from the other variants producing an interatrial communication. Precordial echocardiography usually allows selection of cases likely to be suitable for closure in this fashion. This selection is based on the localisation and the size of the deficiency in the oval fossa, the length of the interatrial septum, and the adequacy of the infolded rims surrounding the defect. Suitability for closure is reevaluated by transesophageal echocardiography, either asa separate investigation or at the start of the interventional theterisation. This investigation requires a multiplane transesophageal echocardiographic probe, since only oblique planes will demonstrate the entrance of the systemic and pulmonary veins and their relationship to the defect. Transesophageal echocardiography serves as an important monitoring tool during the interventional procedure. As such, it is a necessary adjunct to fluoroscopy. The stretched diameter of the defect measured with a balloon is the main eterminant of the choice of the type and size of the device. This diameter can be measured fluoroscopically, as well as on echo. Colorflow mapping serves to rule out residual shunting during the occlusion of the defect with the balloon. During deployment of the device, constant echocardiographic visualisation of the device and its position relative to the atrial septum facilitates proper placement. Such constant visualisation can only be provided by repeated quick acquisitions of multiple planes. Once the device is released, the investigator should continue to record the position of the device, and assess the potential for residual shunting.Most of the devices show some subtle change in position during the first 20 minutes after implantation.
The Amplatzer septal occluder
Authors: Walsh Kevin P. , Maadi Ismail M. 
Published: August 2008

AbstractThe Amplatzer Septal Occluder is made from a Nitinol wire mesh shaped into 2 disks with a connecting waist, which serves to center the device in the defect while occluding it. The Amplatzer device is also available in a configuration with no central waist for use in patients with patent oval foramen, or multiperforated aneurysm of the interatrial septum. For the purposes of this review, we analysed our experience using the Amplatzer device in 150 patients with interatrial communications. Of these, 104 had a defect within the oval fossa, 33 a patent oval foramen, and 13 had undergone fenestration of a Fontan procedure. Of those with defects within the oval fossa, a device was implanted in 100 patients, and 2 of these patients subsequently required surgical intervention, 1 because of migration and the other because of malformation of the device. Of the remaining 98 patients, complete occlusion has been achieved in 90% at 1 year. Any residual leaks are either trivial or small. In those with a patent oval foramen, the septal occluder was used to close 20, whilst the device designed specifically for this purpose was used in 13. On follow-up contrast echocardiography, only 2 patients have a small residual right-to-left shunt. Complete occlusion was achieved for all the Fontan fenestrations, although 1 patient later underwent surgery for baffle dehiscence. Other significant complications occurred in 2 patients who developed deep vein thrombosis, and 3 patients who suffered transient supraventicular arrhythmias. Although the Amplatzer device has been in clinical use for only 3 years, its unique design, and ease of use, has resulted in its widespread adoption by many entres. The results to date are very encouraging, but it must be remembered that there is, as yet, no long-term followup data available for this life-long implant.
Evaluation of the morphology of the oval fossa for placement of devices
Authors: Ussia Gian Paolo , Momenah Tarek S. , Ursell Phillip , Brook Mike M. , Moore Philip , Luca Francesco De , Silverman Norman H. 
Published: August 2008

AbstractObjectivesFirst, to examine the morphology of heart specimens with defects of the oval fossa so as to define the factors that facilitate appropriate selection of the size of devices used for inteventional closure. Second, to examine the relationship between morphology and transthoracic and transesophageal echocardiography.BackgroundThe success of transcatheter closure is influenced by the variable morphology of deficiencies with the oval fossa, and of the relationship of the fossa itself to adjacent structures. More appropriate selection could reduce the incidence of failures.MethodsFrom over 100 specimens in the cardiac registry at the University of California, San Francisco, we judged 16 hearts with atrial septal defects within the oval fossa, either in isolation or associated with other cardiac malformation, to be suitable for this study. We measured the dimensions of the defect and the surrounding rims of the fossa. All values were normalized to the diameter of the aortic root.ResultsA fenestrated defect was present in 9 specimens (56%). The shape defect itself was oval in all specimens, with a ratio of major to minor axes of 1.70 ± 0.63– The major axis took one of three main directions with respect to the vertical plane: in 11 specimens (69%) it was at horizontal; in 3 (19%) it was at oblique at an angle of 45 degrees; and in 2 (12%) it was vertical. Discordance was noted in some hearts between the major axis of the defect and that of the oval fossa. Structures closest to the rim of the fossa were the aortic mound, the coronary sinus, and the hinge point of the aortic leaflet of the mitral valve.ConclusionsExtrapolating from these specimens permitted identification of the major and minor axes of the atrial septal defect by transthoracic and transesophageal echocardiography. Our study has identified landmarks and dimensions that may be employed to improve effectiveness of selection of patients for transcatheter closure of defects within the oval fossa.
Transesophageal echocardiographic predictors for successful transcatheter closure of defects within the oval fossa using the CardioSEAL® septal occlusion device
Authors: Momenah Tarek S. , McElhinney Doff B. , Brook Michael M. , Moore Phillip , silverman Norman H. 
Published: August 2008

AbstractObjectivesTo define the utility of transesophageal echocardiography in predicting the likelihood of a successful procedure and residual shunting in patients undergoing transcatheter closure of defects within the oval fossa using the CardioSEAL® device.BackgroundTransesophageal echocardiography is used to monitor transcatheter closure of interatrial defects within the the oval fossa, but predictors of successful closure and residual shunting have yet to be determined.MethodsWe reviewed transesophageal echocardiograms obtained from 26 consecutive patients undergoing attempted transcatheter closure of interatrial defects within the oval fossa between January, 1997 and May, 1998. Assessment of the atrial septum, the septal defect, and the rims of the oval fossa bordering the defect was performed in 3 planes: longitudinal, 4- chamber, and basal short-axis.ResultsClosure proved successful in 24 patients (92%). The defect was significantly larger, and the anterosuperior rim of the defect smaller, in the 2 patients in whom occlusion was not successful. Residual shunting 24 hrs after closure was detected in 14 patients. Significant predictors of leakage included smaller posterior and superior rims, a larger shunt prior to closure, and herniation of a one left atrial arm of the device into the right atrium. In all cases, the sites of leakage were the superior rim of the defect at the superior cavo-atrial junction, and the anterosuperior rim behind the aortic root. Herniation of a left atrial arm into the right atrium was seen in 7 patients (29%). In all, it was the anterosuperior arm which herniated Doppler color flow was suboptimal in detecting residual leaks, and was enhanced substantially with the use of contrast echocardiography.ConclusionsTransesophageal echocardiography allows excellent assessment of the oval fossa and deficiencies of its floor in all of their dimensions. It is an important tool for guiding the deployment of the occlusion device in patients undergoing attempted transcatheter closure of defects within the fossa. Contrast echocardiography should be used for optimal detection of residual shunting.
A European multicentric experience using the CardioSEAL® and Starflex double umbrella devices to close interatrial communications holes within the oval fossa
Authors: Carminati M. , Giusti S. , Hausdorf G. , Qureshi S. , Tynan M. , Witsenburg M. , Hess J. , Piechaud JF , Bonhoeffer P. , Donti A. , Ovaert C. , Sievert H. , Elzenga N. , Talsma M. , van Oort A. , Ernst J. , Gewillig M. , DeGeeter B. 
Published: August 2008

AbstractIn this review, we describe the experience from 13 European centres using the CardioSEAL and Starflex double umbrella devices to close interatrial communications within the oval fossa (so-called ‘stcundum’ defects). Between October 1996 and April 1999, the procedure was attempted in 334 patients with a mean age of 12 years and a mean weight of 44kg. The mean measured stretched diameter of the defect was 15 mm. In the overall group, the defect was solitary in 245 patients (73%), multiple in 21 (6%), associated with an aneurysm of the flap valve in 15 (5%), was represented by patency of the oval foramen in 44 (13%), and was a fenestration in a Fontan repair in 9 (3%). In all patients, the devices were inserted under general anesthesia, using fluoroscopic and transesophageal echocardiographic control. Implantation was achieved in 325 (97,3%). The device embolized within either a few minutes or a few hours in 13 patients (4%). Of these, uncomplicated surgical repair was undertaken in 10, while the device was retrieved in 3 using catheters and a second device was successfully implanted. Residual shunting was detected immediately after the procedure in 41% of the patients, with the incidence decreasing to 31% at discharge, 24% at 1 month, 21% at 6 months, and 20.5% at one year. During the period of follow-up, elective surgical repair became necessary in two patients, due to malposition of the device in one, and late embolization in the other. Fractures of arms were seen in 6.1%, most commonly with the largest devices. All those with fractured arms of the device were asymptomatic, and no clinical complications related to the fractures were observed. There were no arrythmias, endocarditis, valvar distortion, thromboembolic events, or other complications. After one year of follow-up, clinical success, defined as complete closure of the defect or presence of only a trivial leak, had been obtained in 92.5% of the patients. We conclude, therefore, that these devices produce excellent results when used to close defects of small to moderate size. Results are less than optimal, or else complications ensure, when attempts are made to close very large defects.
Experience in one centre using the buttoned device for occlusion of atrial septal defect: comparison with the Amplatzer septal occluder
Authors: Godart François , Rey Christian , Francart Charles , Cajot Marie André , Brevière Georges Marie , Vaksmann Guy , Devos Patrick , Coullet Jean Michel , Vincentelli André 
Published: August 2008

AbstractWe report our experience using the buttoned device to close defects within the oval fossa and probe-patent oval foramens, comparing the findings with those obtained with the Amplatzer septal occluder. from 1992 to 1997, we used the buttoned device to close defects in 73 consecutive patients, 64 with defects in the oval fossa and nine with patent foramens. We compared this experience with a further series of 62 patients seen from 1997 to 1999 in whom the Amplatzer septal occluder was used. Successful implantation was achieved in three-quarters of those with septal defects in whom the buttoned device was used, in all of those in whom the buttoned device was used for patent foramens, and in nine-tenths of those in whom closure was attempted using the Amplatzer occluder. Immediate surgery was needed in 3 patients in whom a buttoned device was used, one because of embolization and two with residual shunts and a straddling device. Similar immediate surgery was needed to retrieve one embolized Amplatzer occluder. During follow-up, surgery was needed in a further 7 patients, all having had insertion of a buttoned device, because of atrial perforation in one and a significant residual shunt in the remainder. At late follow-up, the rate of complete occlusion was 69% in the patients in whom the buttoned device was used to close a septal defect, 100% when the buttoned device was used for patent foramens, and 95% in those treated with the Amplatzer occluder. Our experience shows that the Amplatzer occluder produced a significantly higher rate of occlusion for larger defects, and with a shorter fluoroscopy time than the buttoned device. The Amplatzer septal occluder, therefore, is our preferred device for closure of defects within the oval fossa.