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

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

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 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.

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.

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.

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

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.