bluuebunny/crossref_metadata_2025 huggingface dataset:
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

Introduction

The 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 Results

Physical 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).

Conclusions

Physical 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

Our 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

Background

Complete 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 / Methods

We 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.

Results

There 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.

Conclusions

Despite 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

Increasingly, 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

Closure 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

The 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

Objectives

First, 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.

Background

The 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.

Methods

From 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.

Results

A 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.

Conclusions

Extrapolating 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

Objectives

To 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.

Background

Transesophageal 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.

Methods

We 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.

Results

Closure 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.

Conclusions

Transesophageal 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

In 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

We 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.
Treatment of atrial septal defects in symptomatic children aged less than 2 years of age using the Amplatzer septal occluder
Authors: Vogel Michael , Berger Felix , Dähnert Ingo , Ewert Peter , Lange Peter E.
Published: August 2008

Aims

To assess results of closure of atrial septal defects within the oval fossa by devices delivered by catheterisation in symptomatic infants and children under 2 years of age.

Methods and results

The Amplatzer septal occluder was used. Results, and complications of transcatheter device treatment in patients aged below 2 years were compared to previous results from our institution.

Patients

We attempted closure in 12 consecutive patients below the age of 2 years who presented with an atrial septal defect between May 1997 and 1999. Symptoms were failure to thrive in 6, frequent chest infections in 5, and the need for treatment of heart failure in the other. All were thought to have a defect suitable for interventional closure. The atrial defects were seen in isolation in 10 children, but 2 had associated pulmonary stenosis which had been treated by balloon dilation prior to placement of the Amplatzer occluder.

Results

The Amplatzer septal occluder was implanted at a mean age of 1.4 ± 0.4, with a range from 0.8 to 1.8 years. Ratios of pulmonaryto-systemic flow had been 2.1 ± 0.5, with a range from 1.6 and 3.2, and the defect was measured at 12 ± 4 mms. Fluoroscopy time was 12.8 ± 10.2 minutes, with a range from 5 to 43 minutes, and the time of the overall procedure was 162 ± 70 minutes, with a range from 85 to 360 minutes. It proved necessary to remove the device in 2 patients (16%) because of a residual shunt and movement after release. One of these developed transient neurological complications. Both subsequently underwent surgical treatment.

Conclusion

Symptomatic patients less than 2 years of age can undergo successful closure of an atrial septal defect using the Amplatzer device, but the rates of success are less, and procedure time longer, than in older children or adults.
An aortic valve with four leaflets
Authors: Sakai Yoshiyuki , Tomita Hideshi , Ukae Susumu
Published: August 2008

An aortic valve with four leaflets is a rare congenital malformation. It is usually diagnosed unexpectedly at autopsy, during replacement of the aortic valve, or by aortic angiography. Transthoracic cross-sectional echocardiography, however, can also delineate the structure of the aortic valve, and demonstrate the presence of valves with four leaflets
Cardiac tamponade in a child with ascariasis
Authors: Papadopoulos Georgios S. , Eleftherakis Nikolaos G. , Thanopoulos Basil (Vasilios) D.
Published: August 2008

Ascaris infection, when severe, may manifest with constitutional symptoms. The parasites penetrate the intestinal walls, reach the venules and lymphatics and, through the portal circulation, may affect the heart. To the best of our knowledge, involvement of the pericardium has yet to be reported. We describe here a case of severe pericardial effusion and cardiac tamponade in a child with ascariasis.
Newsletter from the Association for European Paediatric Cardiology
Authors: Silove Eric D.
Published: August 2008

The 35th annual general meeting of the Association for European Paediatric Cardiology, 14–17th June 2000, was held in the beautiful, historic city of Strasbourg. It was the largest meeting of the Association, with more than 600 registrants. They were rewarded with a scientific programme of the highest quality and a social programme to match, thanks to the outstanding organisational qualities of Dr Bernard De Geeter, whose whole family contributed at all scientific and social functions. The whole experience was most interesting, entertaining and convivial.
Phentolamine improves clinical outcome after balloon valvoplasty in neonates with severe pulmonary stenosis
Authors: Galal Omar , Kalloghlian Avedis , Pittappilly Babu M. , Dzimiri Nduna
Published: August 2008

Two neonates with severe pulmonary stenosis deteriorated immediately after successful balloon valvoplasty as a result of increased infundibular obstruction. They were treated with β-adrenoceptor blockers and intravascular expansion with limited success. Phentolamine was then given, resulting in dramatic improvement. The children could be weaned from the ventilator within hours of starting this new therapeutic modality.
Natural history of congenital pulmonary valvar stenosis: an echo and Doppler cardiographic study
Authors: Gielen Hanneke , Daniëls Otto , van Lier Henk
Published: August 2008

Doppler echocardiography allows accurate serial assessment of pulmonary valvar stenosis by measuring the velocity of the jet stream through the pulmonary valve. Between 1979 and 1997, we saw 174 patients with isolated pulmonary valvar stenosis. At admission their ages ranged from 9 days to 22.5 years. We measured the velocity over the pulmonary valve, and the thickness of the anterior wall of the right ventricle, and made a study of their electrocardiograms. We found that rapid increases and decreases occurred in almost every age-group. For patients with a trivial, mild or moderate level of stenosis, severe stenosis developed in 3, 10 and 9%, respectively. In most of the patients, 122 (90%), in whom there was more than one examination, a change in pressure gradient between −12mmHg/year and +3mmHg/year was found. Only 7 patients had an increase of more than 10mmhg per year. In contrast with our patients having aortic stenosis, these with stenosis of the pulmonary valve showed no rapid increase in early childhood. Indeed, in 58% the severity of the stenosis decreased. No correlation was found when comparing the echocardiographic measurements of the thickness of the anterior wall of the right ventricle with the voltages on the electrocardiogram. A significant relation was found however, between an increasing pressure gradient and thickened valvar leaflets (p = 0.017).
Transcatheter closure of atrial septal defects under echocardiographic guidance without X-ray: initial experiences
Authors: Ewert Peter , Daehnert Ingo , Berger Felix , Kaestner Andreas , Krings Gregor , Vogel Michael , Lange Peter E.
Published: August 2008

Background

Transcatheter closure of atrial septal defects is performed under fluoroscopy, but echocardiography has gained an important role in the procedure. With the new Amplatzer Septal Occluder a device has become available which is easy to implant with minimal fluoroscopy time. We developed an interventional procedure with this device under transesophageal echocardiography alone without fluoroscopy.

Methods and Results

Four patients (3 to 16 years of age, bodyweight 14 to 60 kg) with atrial septal defects centrally located in the oval fossa were elected for transcatheter closure. After sedation with midazolam and propofol a diagnostic and interventional catheterization was performed in all cases without fluoroscopy. Oxymetric shunt was Qp: Qs = 1.7 (1.5 to 2.1). Under transesophageal echocardiography, the defects were sized over the wire with a balloon catheter. Mean balloon stretched diameter was 10 mm (7 to 14 mm). Under transesophageal echocardiography an Amplatzer Septal Occluder was placed into the defect. In two patients this was achieved with a 5 MHz monoplane pediatric transducer, in two patients a 10mm 5 MHz multiplane probe was used. Complete closure was achieved in all patients and no complications were encountered.

Conclusion

We conclude that in selected cases with an atrial septal defect located in the oval fossa and clear-cut echocardiographic findings, an Amplatzer Septal Occluder can be safely deployed under echocardiographic guidance alone.
Elevation of atrial natriuretic peptide prohormone. Hemodynamic background of the elevation of N-terminal natriuretic peptide prohormone in children with congenital heart disease
Authors: Holmström Henrik , Hall Christian , Stokke Oddvar , Lindberg Harald , Thaulow Erik
Published: August 2008

We postulated previously that variables related to pulmonary flow are independent predictors of levels of atrial natriuretic peptide in children with congenital heart disease. The aim of this study was to test this hypothesis in relation to other hemodynamic and clinical variables.

During catheterization we measured the levels of plasma N-terminal atrial natriuretic peptide prohormone in the plasma of 68 children with congenital heart disease. All had undergone complete clinical, echocardiographic and invasive hemodynamic investigations. The influence on the prohormone was analyzed for 10 different variables in a multiple linear regression model. The variability could be explained in large parts (adjusted R2= 77.2%) by variations in atrial pressures or sizes, together with the degree of excessive pulmonary blood flow and signs of heart failure.

A value for atrial natriuretic peptide prohormone above 800 pmol/1 predicted hemodynamic imbalance (defined as elevated pressures in left or right atrium or the pulmonary arteries, and/or Qp/Qs> 1.5) with a specificity of 94%, a sensitivity of 73%, a positive likelihood ratio of 12.2, and a negative likelihood ratio of 0.29.

In conclusion, variables related to pulmonary blood flow are influential determinants of the levels of atrial natriureic peptide in children with congenital heart disease. Atrial pressures, and symptoms of heart failure are also of major importance.
Regional differnces in birth prevalence of congenital heart disease in Malta
Authors: Grech Victor , Agius-Muscat Hugo , Savona-Ventura Charles , Pace Joe
Published: August 2008

Aim

Recent studies have shown a correlation between environmental pollutants and increased risk of selected congenital malformations. The South-East area of the Island of Malta is much more industrialised than the more rural North-West area. The aim of this study was to test the null hypothesis that there are no regional differences in Malta in the prevalence at birth of congenital cardiac malformations. Methods: Live born infants with congenital cardiac malformations born between 1990 and 1994 were allocated to 10 areas, and thence to 2 regions of 5 areas each, so as to constitute North-West and South-East Malta. Official publications gave population totals and growth rates. Those infants with congenital cardiac malformations were then compared between the different regions using χ2and the Mann-Whitney U test.

Results

The overall prevalence of congenital heart disease at birth was 8.8/1000 live births. The birth prevalence for the South-East region (10.1/1000 LB – 95% CI 8.4–12.3/1000 live births) was significantly higher than for the North-West (7.4/1000 live births – 95% CI 6.0–9.0/1000 live births) – p=0.03, Odds ratio 1.38 (95% CI 1.05–2.61). The Mann-Whitney U test showed a significant difference in the distribution amongst the 10 defined areas (p=0.016). The Central-East area had the highest prevalence of cardiac malformations in the entire Island – p=0.02, Odds ratio 1.70 (95% CI 1.10–2.61). Demography showed an efflux of individuals from the South-East of Malta.

Conclusion

The higher prevalence of congenital heart disease noted at birth in South-East Malta is unlikely to be due to genetic factors, as these would have migrated North-West along with the population movement. An environmental factor, therefore, seems more likely to be responsible for the increased predisposition to congenital heart disease in the South-East of Malta.
Detection of cardiovascular abnormalities in the nursery of a general hospital in the Amazon region: correlation with potential risk factors
Authors: Ferreira Sheila Maria Almeida Gomes , Ferreira Alberto Gomes , Maria de Lourdes Contente Gomes  , Alexandre da Costa Linhares  , Décourt Luiz Vénere
Published: August 2008

Congenital heart diseases have been studied much more extensively in children than in neonates. In this study, we report on the findings from 57 neonates seen from June of 1995 through June 1996 in the nursery of a large public hospital in Belém, Para, Brazil. All were routinely examined by a paediatrician just after birth, and, when indicated, these babies were referred to the cardiology unit of our Hospital for assessment by a paediatric cardiologist. Most of the diagnoses were made by means of Doppler and cross-sectional echocardiography with color flow mapping. Several abnormalities of the cardiovascular system were diagnosed. The most frequent was patency of the arterial duct. But, since many ducts closed spontaneously, ventricular septal defect was the most frequent lesion seen even in the nursery. Four defects (patent arterial duct, ventricular septal defect, atrial septal defect and pulmonary stenosis) together accounted for two thirds of all cardiac abnormalities. Associated non-cardiac anomalies were more frequent in those with simple lesions within the heart. All the babies with complex heart disease, and the majority of those designated as having significant lesions, died before they could be discharged. Several risk factors were investigated. Among maternal drugs, misoprostol emerged as having a possible teratogenic effect.
Early clinical screening of neonates for congenital heart defects: the cases we miss
Authors: Meberg Alf , Otterstad Jan Erik , Frøland Gisle , Hals Jardar , Sörland Svein Jan
Published: August 2008

In a population-based study of 35,218 infants born alive during the 15 years from 1982 to 1996, 353(1%) were diagnosed as having a congenital heart defect, of whom 84 (24%) were diagnosed subsequent to discharge from hospital after birth (2.4/1000). Of these, 40 (48%) had a ventricular septal defect, 14 (17%) an atrial septal defect, 9 (11%) a patent arterial duct, 8 (10%) an aortic stenosis and 13 (15%) other defects. Compared with those in whom diagnosis was made before discharge, the group of patients with defects detected late had an increased prevalence of atrial septal defects, patent arterial duct and aortic stenosis, but less decreased prevalence of ventricular septal defects (p< 0.05). Median age at detection of the defects subsequent to discharge was 6 months (range 2 weeks–11 years). Seven patients (8%) presented with clinical symptoms of cardiac decompensation. The mortality rate was significantly lower in those in whom defects were detected late (1/84; 1%) as compared with those detected immediately after birth (37/269; 14%) (p< 0.05). The total rate for early detection was the same after using one clinical examination (8.2/1000) of newborns as our basic routine instead of two (7.1/1000) (p> 0.05). A substantial proportion of congenital cardiac malformations are detected after discharge from hospital after birth. Some patients with these lesions present with cardiac decompensation and are in need of medication and surgery. One clinical examination of newborns detects congenital malformations of the heart as efficient as two.
Prenatal diagnosis of left ventricular aneurysm: a report of three cases and a review
Authors: Matias A. , Fredouille C. , Nesmann C. , Azancot A.
Published: August 2008

We report three cases of left ventricular aneurysm diagnosed prenatally and followed by fetal Doppler echocardiography. A review of the literature reveals a paucity of information about this rare cardiac malformation. Most of the described cases (6 out of 9) have remained asymptomatic during pregnancy and after birth. Our cases, in contrast, and three others in the literature, had an ominous prognosis presenting cardiac failure initially or during follow up and showing a dynamic evolution of the aneurysm. Cross-sectional echocardiography provides the diagnosis, revealing the thin-walled aneurysm, usually apical, to be connected by a broad neck to the left ventricle. Color and pulsed Doppler shows low velocity and to-and-fro flow in the aneurysm. Sequential fetal Doppler echocardiography detects the potential growth of the aneurysm relative to ventricular size, revealing any compromise of cardiac performance by a decreased mitral opening, reversed atrial shunting, a hypokinetic infero-posterior left ventricular wall, and a poor systolic thickening of the wall of the aneurysm. Compromise of cardiac function, and deleterious impact on development of the lungs during fetal life, may depend on the early onset, growth and location of the aneurysm, which may occupy most of the fetal chest. We discuss issues of prenatal diagnosis, sequential surveillance of the natural history, and factors of prognosis as well as myocardial histological data from one of our cases.
Critical obstruction of the right ventricular outflow tract by a primary hemangioendothelioma in a seven month old
Authors: Robinson Hilary A. , Keeton Barry R. , Moore Isabella E.
Published: August 2008

A 7-month-old presented with failure to thrive and a murmur. Echocardiography demonstrated a large mass in the right ventricular outflow tract, extending through the pulmonary valve. During anaesthetic induction this caused critical obstruction of the outflow tract and cardiac arrest. Pathological diagnosis showed the lesion to be a primary hemangioendothelioma. Despite surgical excision and steroid therapy, the mass continued to grow for a period of 8 weeks, but then began to regress spontaneously.
Progressive disease of the atrioventricular condution axis in an infant of an anti-Ro positive mother
Authors: Uzun Orhan , Gibbs John L.
Published: August 2008

A child of a mother with maternal anti-Ro and anti-La antibodies presented antenatally with abnormal myocardial function, and was found to have a first degree heart block at birth. The extent of the abnormality in the conduction system progressed, with appearance of left bundle branch block in addition to further prolongation of PR interval. A pacemaker was implanted prophylactically, but patient has remained well, with no further deterioration in her atrioventricular conduction.
Amiodarone-induced pulmonary toxicity in an adolescent
Authors: Kothari Shyam S. , Balijepally Sharada , Taneja Karuna
Published: August 2008

We report amiodarone-induced pulmonary toxicity in an 18-year-old who had undergone corrective surgery for tetralogy of Fallot 4 years earlier, and was treated with amiodarone because of recurrent malignant postoperative ventricular tachyarrhythmias. Toxicity was recognized on the basis of clinical features, chest X-ray, high-resolution contrast enhanced computerized tomography, and resolution of the findings subsequent to withdrawal of amiodarone and treatment with steroids. pulmonary toxicity due to amiodarone, as far as we know, has not been reported in children and young adults, and its occurence even in young adults requires wider appreciation.
Surgical ligation of a persistent arterial duct in one of conjoined thoracopagus twins prior to surgical separation
Authors: Cordoba Roque A. , Juaneda Ernesto , Alday Luis E.
Published: August 2008

One of conjoined thoracopagus twins, with separate hearts and a common pericardial sack, presented with respiratory distress because of a persistent arterial duct causing congestive heart failure in the neonatal period. Surgical ligation of the duct was performed prior to subsequent separation, with an excellent outcome.
Coarctation of the Left pulmonary artery associated with congenitally corrected transposition
Authors: Massin Martial M. , von Bernuth Götz
Published: August 2008

We describe an infant with congenitally corrected transposition, ventricular septal defect and severe pulmonary stenosis. The heart occupied a midline position. Extension of ductal tissue had resulted in occlusion of the left pulmonary artery. As far as we are aware, this is the first report of an association of coarctation of the left pulmonary artery with corrected transposition.
The basics of ventricular function
Authors: Penny Daniel J.
Published: August 2008

There has been increasing interest in the study of ventricular function in the patient with congenital heart disease. Numerous indexes have been derived for the assessment of ventricular function, suggesting that none is ideal. While the derivation of some measures of ventricular function have relied on advanced mathematical principles, it is still possible for the non-mathematician to obtain important insights into ventricular function from an assessment of the events which underpin the cardiac cycle. In this review, I use the mechanics of the cardiac cycle to introduce basic concepts of ventricular function for the non-expert. In this way, I analyse ventricular systolic and diastolic performance and describe the contribution of regional variability of function to overall performance. This approach also highlights the role of the ventricle in overall cardiovascular and metabolic homeostasis.
Richard Desmond Rowe MD, MB, ChB February 10, 1923 to January 18, 1988
Authors: Freedom Robert M.
Published: August 2008

Dick Rowe (Fig.) would be embarrased by this attention, as he was such a very modest man. Yet it is appropriate that the late Richard Desmond Rowe be included inCardiology in the Young'sPaediatric Cardiology Hall of Fame. As I consider the life and contributions of Dick Rowe, I am at first troubled, indeed saddened, by the realization that more than a decade has passed since his untimely and premature death just weeks before his planned retirement. I have his picture on the mantle of my office. Not a day goes by that I don't look at his countenance, however fleeting, reflecting on my good fortune to have worked with and considered Dick Rowe as mentor, colleague, teacher and friend. This sense of good fortune is shared by virtually all those touched by his warmth and generosity.
Twisted atrioventricular connections in double inlet right ventricle: evaluation by magnetic resonance imaging
Authors: Kim Tae Hoon , Yoo Shi-Joon , Ho Siew Yen , Anderson Robert H.
Published: August 2008

Twisted atrioventricular connections occur almost exclusively in the hearts with biventricular atrioventricular connections. Only one example of double inlet left ventricle has been illustrated in which the axes of the two atrioventricular valves crossed each other. We describe herein three patients, and one autopsied specimen, with double inlet right ventricle in which magnetic resonance imaging clearly demonstrated twisted atrioventricular connections.
Impact on parents of feeding young children with congenital or acquired cardiac disease
Authors: Imms Christine
Published: August 2008

Feeding an infant is an interactive process that facilitates social, emotional and culturally based skills. Children with congenital or acquired cardiac disease frequently require supportive regimes with regard to feeding so as to maintain weight, resulting in altered experiences for both the child and family. This study evaluated the practical, emotional and social ramifications for parents, of having a child with cardiac disease who also experienced difficulties with oral feeding. The study sampled three groups of parents who had children less than 3 years of age: those with cardiac disease who had difficulty in feeding, those with cardiac disease and no such difficulty, and those with no medical diagnosis. Parents completed a questionnaire about feeding, a time diary of activities involved in feeding, and Tuckman's Mood Thermometers, which measure anger and ‘poorness-of-mood’ associated with feeding the identified child. Parents of children with cardiac disease and a feeding difficulty reported a significantly more negative mood-state, and significantly longer time associated with feeding, than parents of children in the other two groups. Emerging themes from qualitative analysis of the data suggested that having a child with congenital cardiac disease producing difficulty in feeding had a strong negative impact on the whole family.
Severe tortuosity and stenosis of the systemic, pulmonary and coronary vessels in 12 patients with similar phenotypic features: a new syndrome?
Authors: Fadley Fadel Al , Manea Waleed Al , Nykanen David G. , Fadley Abdulah Al , Bulbul Ziad , Halees Zohair Al
Published: August 2008

We describe what is, to the best of our knowledge, a previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels. We evaluated 12 patients with similar phenotypes, from eight different families. Detailed echocardiographic and angiographic evaluations were performed in all, and biopsies of the skin in seven. All patients have elongated facies, prominent ears, micrognathia and laxity of their joints. Angiographic pictures showed a varying degree of lengthening and tortuosity of systemic, pulmonary, and coronary arteries. Pulsatile carotid arteries formed cervical masses in 2 patients, and three had severe renal arterial stenoses. All showed varying degrees of branch and peripheral pulmonary arterial stenosis, necessitating placement of stents in six. Biopsy of the skin proved normal in all seven patients studied, thus excluding cutis laxa, Ehlers-Danlos and Marfan syndromes. The constellation of abnormalities suggests a genetic syndrome of connective tissue etiology. Further genetic studies, and gene mapping, are underway.
Long-term results after valvotomy for congenital aortic valvar stenosis in children
Authors: Lambert Virginie , Obreja Daniela , Losay Jean , Touchot-Koné Anita , Piot Jean-Dominique , Serraf Alain , Lacour-Gayet François , Planche Claude
Published: August 2008

As interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10–16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a rangefrom 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15–49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.