cardiac surgery is a discipline aimed at restoring the best cardiac function of diseased hearts. it is often necessary to implant devices, and surgical tools are of great help in achieving faster and safer procedures. paediatric cardiac surgery is a more recent specialization of cardiac surgery. in paediatric cardiac surgery, the problem is usually related to congenital malformations, consisting in the main of anatomical abnormalities which produce, among other problems, poor oxygenation of the blood, high cardiac afterload, cardiac failure, hemodynamic problems during growth, and so on. the paediatric cardiac surgeon uses his skill and experience to determine the optimal correction in each individual case. in many cases, however, it is not easy to predict the effects of the given surgical procedure that, for success, depends on many different parameters.

Florida is the fourth largest state in the United States of America, and the Congenital Heart Institute of Florida is the largest programme providing services for congenital cardiac surgery in Florida. Our Institute is very grateful to Bob Anderson, and the team at Cardiology in the Young, for their support of our programme, and for the opportunity to publish this Supplement. Bob Anderson was a valued faculty member, and tremendous supporter, of our third annual symposium, held at the All Children's Hospital and The University of South Florida, and devoted to echocardiographic, anatomic, surgical, and pathologic correlations of congenital cardiac disease. Our Symposium to be held in 2004, coinciding with the appearance of this supplement, will mark the third consecutive year that Bob Anderson has traveled to Saint Petersburg, Florida as a Featured Guest Speaker. In 2002, at our Second Annual Symposium, the focus of our four-day meeting was the abnormalities of the ventricular inlets and atrioventricular valves, with a full day devoted to echocardiography, followed by three single days spent discussing the tricuspid valve, the mitral valve, and the common atrioventricular valve. In 2003, at our third annual symposium, the focus of our four-day meeting was controversies concerning the hypoplastic left heart syndrome, again beginning with a full day devoted to echocardiography, followed by three single days this time discussing staged palliation by means of the Norwood sequence, replacement by cardiac transplantation, and biventricular repair. A feature of the symposium was the numerous debates. In 2003, our featured guest speakers, in addition to Bob Anderson, were Leonard L. Bailey and his wife Nancy, from Loma Linda University Medical Center, Loma Linda, California. Bob Anderson will again return in 2004, when our other featured guest speaker will also be from Great Ormond Street, namely Martin J. Elliott. The focus of our meeting in 2004 will be controversies concerning the arterial switch, the Ross procedure, and reconstructions of the right ventricular outflow tract, again beginning with a full day devoted to echocardiography, followed by three single days discussing the pulmonary valve, the aortic valve and the Ross procedure, and the arterial switch procedure, with daily debates.

Objectives: To estimate the incidence of, and profile the spread of, congenital cardiac defects, and to assess the accuracy of the echocardiographic diagnosis as performed by a neonatologist. Design: Hospital-based study. Methods: All neonates meeting our criterions, specifically those with a persistent murmur 48 hours after birth, underwent an echocardiographic examination to exclude an underlying congenital cardiac defect. All scans performed by the neonatologist were videotaped and reviewed by a paediatric cardiologist. We assessed concordance according to inter-rater agreement. Results: Out of 11,085 live births, there were 83 infants with a congenital cardiac defect, giving an incidence of 7.49 per 1000 live births, with 95% confidence interval from 5.88 to 9.09. Of the infants with a murmur persisting at or greater than 48 hours after birth, who had a median age of 4.5 days, 75% had a congenital heart defect, with ventricular septal defect being the commonest, encountered in 56.7% of cases. Concordance between the neonatologist and the cardiologist was good, with Cohen's Kappa coefficient being calculated at 0.68, and 95% confidence interval from 0.51 to 0.85. Conclusion: The incidence of congenital cardiac malformations as determined in our hospital-based study in the United Arab Emirates is similar to that described in the Gulf region and worldwide. A persistent murmur at or greater than 48 hours after birth is strongly suggestive of an underlying congenital cardiac malformation. Our experience shows that a neonatologist appropriately trained in echocardiography can perform as well as a paediatric cardiologist. Where specialist resources are limited, this allows for early diagnosis, earlier referral if necessary, and early institution of appropriate therapy.

Sudden death and ventricular tachycardia are known to occur late after correction of tetralogy of Fallot. Abnormal dispersion of the QT interval, ventricular late potentials, and prolongation of the QRS complex, alone or in combination, are useful markers of the risk for such complications. Our present prospective study investigates the time course of appearance of two markers, dispersion of QT and JT, and ventricular late potentials, before and after corrective surgery. Dispersion of QT and JT, and signal averaged electrocardiographic parameters, were determined sequentially in 20 patients before, a mean of 9 ± 3 days after, and again 35 ± 11 days post-operatively. Dispersion of QT was already abnormal before surgery in two-fifths of the patients, but increased markedly in the later post-operative period. Ventricular late potentials were absent before surgery and in the immediate post-operative period, but were found in one-fifth of patients 1 month later. We conclude that abnormal dispersion of QT is, to some extent, a fact of the natural history of tetralogy of Fallot, but is significantly amplified by surgery. Ventricular late potentials, on the other hand, are absent before surgery, but appear with some delay after the operation, probably as a result of scarring rather than the surgical incision itself. Long-term follow-up is needed to assess the significance of these findings.

An aortopulmonary window is a communication between the ascending aorta and pulmonary trunk in the presence of two separate arterial valves, and is often complicated by other associated defects. We sought to determine management and related outcomes in patients with this malformation.

We identified those patients presenting between 1969 and 1999 from the databases held in our Departments of Cardiology, Pathology and Cardiovascular Surgery. We obtained data relating to issues concerning demography, clinical findings, imaging, management and outcome.

The median age at presentation for the 42 patients identified, of whom 23 were female, was 62 days, with a range from birth to 6 years. Associated cardiac defects were present in 34 patients, including interruption of the aortic arch in 6 patients. The correct diagnosis was initially missed in 13 patients. Of the patients, six died without surgical repair, and 1 patient was lost-to-follow-up. Repair was performed in 35 patients, subsequent to repair of other defects in 4, in association with repair of other defects in 17, of whom 3 died, and as an isolated procedure in 14 patients, one of the latter being treated by transcatheter closure. Overall, there were 9 deaths, all in patients with complex associated defects, except 1 patient with a missed aortopulmonary window after repair of aortic coarctation. Kaplan-Meier estimates of survival were 81% at 3 months until 11.5 years, and 69% up to 21 years. Only the presence of interrupted aortic arch was independently associated with increased time-related mortality, the hazard ratio being 5.87 (p = 0.009).

The outcome for an isolated lesion is excellent. Mortality occurs mainly before repair, mostly with complex associated lesions, particularly interruption of the aortic arch.

Of all the complex malformations that affect the heart, those that have produced the greatest difficulty, over the years, in terms of logical description and classification are the ones with the circulations supported by a functionally univentricular mass. The problems in description stem from the fact that, although the ventricular mass functions as a singular entity, the majority of lesions falling within this category, from the stance of morphology, possess two ventricular chambers. For many years, and indeed, even now in many centres, the hearts are described as being “univentricular”, or alternatively as “single ventricles”. There is still no consensus as to which particular lesions should be included within this “univentricular” category. The paradigm of the group is usually taken as double inlet left ventricle.1 In this lesion, the dominant left ventricle is accompanied by a rudimentary second chamber, albeit that arguments continue as to whether the second chamber is an incomplete right ventricle,2 or simply an infundibulum.3 Further arguments raged as to whether tricuspid atresia should be included within the univentricular category.4,5 The debate is rationalised when we recognise that all the potentially univentricular hearts are treated nowadays by constructing the Fontan circulation,6 or one of its variants,7 as indeed are some unequivocally biventricular hearts.8 Clarification and simplification of the anatomical arrangement in this functionally univentricular category, as we will show in this review, is provided by rigorous application of the crucial philosophic principle of analysis called the “Morphological Method”,9 coupled with separate analysis of the ventricular mass and the atrioventricular junctions.10,11

a hydraulic circulatory duplicator is a hydraulic circuit, closed or open that physically reproduces one human district, along with the effects of any connected apparatus, on local fluid dynamics. such hydraulic circulatory duplicators are usually made up of rigid or deformable pipes, pressure loss generators, such as valves or sponge elements, compliant elements such as a pneumatic room, elastic elements, pumping elements, which may be centrifugal, axial, or roller, and tanks. all the elements are connected by joints. the systems are completed by transducers of pressure or flow, and systems for visualising flow or measuring other parameters of interest.

Atrial arrhythmias are associated with enlarged atrial chambers and an increased duration of the P wave. Repair of atrial defects within the oval fossa is expected to normalize atrial size. Few studies, however, have evaluated electrical and morphological atrial features after repair. Our study was performed to determine if atrial abnormalities exist after surgical closure of such atrial septal defects, and whether early closure improves outcome. We recruited patients who had undergone surgical closure of a defect within the oval fossa, so-called “secundum” atrial septal defects. Electrocardiograms, signal averaged electrocardiograms, and echocardiograms were performed. Two-tailed test and Pearson correlation was utilized for statistical analysis. The population consisted of 20 patients and 27 controls, with the mean age of the patient being 11.25 ± 5.10 years, their age at surgery 6.55 ± 5.10 years, and the time since surgery 4.70 ± 2.61 years. The size of the right (23.88 ± 6.35 ml/m2 versus 18.84 ± 4.43 ml/m2) and left (21.91 ± 12.47 ml/m2 versus 17.72 ± 4.83 ml/m2) atrium were significantly larger in the patients. The duration of the P wave (108 ± 16 ms versus 96 ± 8 ms) and the duration of the PR interval (155 ± 18 ms versus 138 ± 23 ms) were longer. No correlation existed between age or interval since surgery with atrial sizes or measurements of the signal averaged electrocardiogram. We conclude that, despite surgical repair, abnormalities exist in patients with an atrial septal defect. Early surgery does not appear to prevent the atrial abnormalities.

Apart from closure of atrial septal defects, there is little information concerning the use of the CardioSEAL family of occluders in congenitally malformed hearts. We review here our initial experience using the CardioSEAL and STARFlex occluders in 12 patients aged 17.3 ± 11.2 years, with a range from 4 to 34 years. Of the patients, 5 had fenestrated extracardiac Fontan procedures, 5 had persistent patency of the arterial duct, 1 had a leak across a Mustard baffle, and the final patient had a huge pulmonary arteriovenous malformation. We implanted successfully 9 CardioSEAL, and 3 STARFlex occluders, with sizes from 17 to 40 mm. In one patient, the occluder embolized to the right pulmonary artery, from where it was retrieved through the catheter. In two patients, there was a trivial residual leak immediately after implantation, but no patient had a residual leak after 6 months of follow-up. We noted improved ventricular dimensions, without any fractures of the arms of the occluders, perforations, or disturbances of flow after 2.4 ± 0.9 years of follow-up. We have demonstrated, therefore, the versatility of the CardioSEAL and STARFlex occluders, which have been used safely and effectively to close a variety of intra and extracardiac communications other than atrial septal defects.

patients with congenitally malformed hearts characterised by a functionally single ventricle are currently treated using several procedures that bypass the right heart, such as the fontan and hemi-fontan operations, the bidirectional cavopulmonary anastomosis, and the total cavopulmonary connection. all these options are based on the procedures introduced for palliative correction of tricuspid atresia by fontan and baudet in 1971. introduced with the purpose of reducing the pre-operative volume overload, the surgical task mainly consists of separating the pulmonary from the systemic circulation. irrespective of the specific operation performed, we can call the resulting circulation the fontan circulation.