Background: Disturbances of conduction are well known in the setting of acute rheumatic fever. The aim of this study is to investigate the QT dispersion as seen in the surface electrocardiogram of children with acute rheumatic fever. Methods: QT dispersion was quantitatively evaluated in 88 children with acute rheumatic fever. Patients were divided into two groups based on the absence or presence of carditis. As a control group, we studied 36 healthy children free of any disease, and matched for age with both groups. Repeat echocardiographic examinations were routinely scheduled in all patients at 3 months after the initial attack to study the evolution of valvar lesions. Results: The mean QT dispersion was significantly higher in children with rheumatic carditis. But there was no statistical difference between children without carditis and normal children. Among the children with carditis, the mean dispersion was higher in those with significant valvar regurgitation. Dispersion of greater than 55 milliseconds had a sensitivity of 85%, and specificity of 70%, in predicting rheumatic carditis, while a value of 65 milliseconds or greater had sensitivity of 81% specificity of 85% in predicting severe valvar lesions in acute rheumatic carditis. At follow-up examination, a clear reduction on the QT dispersion was the main finding, reflecting an electrophysiological improvement. Conclusions: These observations suggest that QT dispersion is increased in association with cardiac involvement in children with acute rheumatic fever.

Aims: Bosentan is efficacious in idiopathic pulmonary arterial hypertension, and the variants associated with connective tissue disease, but not currently approved for treatment of pulmonary arterial hypertension due to Eisenmenger's syndrome. We sought to evaluate its effect in adults with Eisenmenger's syndrome. Methods: We administered bosentan on the basis of compassionate use in 23 patients with Eisenmenger's syndrome, aged 37 plus or minus 14 years. Of the patients, 17 had never received specific treatment for pulmonary arterial hypertension, five were transitioned from treprostinil, and one from beraprost to bosentan. We measured functional class, saturation of oxygen, haemoglobin levels and six-minute walk distance at baseline, one, six months and at most recent follow-up. Results: Baseline functional class was IV in three, III in fifteen, and II in five patients. At follow-up, with a mean of 15 plus or minus 10 months, 13 of the 23 patients (57%) had improved by at least one functional class, from a median baseline of III to II (p equal to 0.016), mean saturation of oxygen at rest had increased from 81% to 84% (p equal to 0.001), and levels of haemoglobin had decreased from 178 plus or minus 26 grams per litre to 167 plus or minus 19 grams per litre (p equal to 0.001). Overall, the six-minute walk distance did not change from baseline of 335 metres. The distance walked by those not previously receiving specific therapy, however, improved from 318 plus or minus 129 to 345 plus or minus 123 metres (p equal to 0.03). Conclusion: Treatment of adults with Eisenmenger's syndrome using bosentan significantly improved functional class, saturation of oxygen at rest, and decreased levels of haemoglobin. Treatment with bosentan was associated with improvement in six-minute walk distance in those not previously receiving specific therapy. In patients already in receipt of specific therapy, transition to bosentan resulted in no clinical deterioration.

The patient with a functionally univentricular heart is at increased risk for ventricular dysfunction for a variety of reasons. At birth, the pulmonary and systemic circulations are in parallel, leading to pulmonary overcirculation and a volume-loaded functional ventricle. Significant atrioventricular valvar regurgitation, abnormal ventriculoarterial coupling, diastolic dysfunction, and altered ventricular geometry can also contribute to long-term ventricular dysfunction. These collected circumstances place the patient at increased risk for perioperative morbidity and mortality. We will discuss in this review the pathophysiology that leads to ventricular dysfunction at each stage of surgical palliation, as well as the strategies for perioperative management. In addition, we will highlight novel strategies for management of ventricular dysfunction.

The repair of atrioventricular septal defect with a common atrioventricular valve is reconstructive surgery at its best, and hence one of the favourite operations performed by paediatric cardiac surgeons. In the past, the post-operative course from such patients was dominated by the occurrence of pulmonary hypertension crises, which were responsible for significant morbidity and mortality. Nowadays, repair is generally undertaken early in infancy, and this approach has mitigated the problems emanating from pulmonary hypertension. Coupled with a better understanding of the anatomy, and adaptation of the surgical techniques, repair can now be achieved safely at around 2 to 4 months of life, without increasing the risk of postoperative regurgitation across the reconstructed left atrioventricular valve. In this review, we discuss the surgical techniques required for, and clinical results of, such early repair.

Objectives: The Ross procedure is increasingly utilized in the treatment of aortic valvar disease in children and adolescents. Our purpose was to compare pre- and post-operative exercise state in this population. Methods: We included patients who underwent the Ross procedure at our institution between January, 1995, and December, 2003, and in whom we had performed pre- and post-operative exercise stress tests. We used a ramp bicycle protocol to measure consumption of oxygen and production of carbon dioxide. Cardiac output was estimated from effective pulmonary blood flow by the helium acetylene re-breathing technique. Results: We studied 26 patients, having a median age at surgery of 15.7 years, with a range from 7.5 to 24.1 years. The primary indication for surgery in two-thirds was combined aortic stenosis and insufficiency. Median time from the operation to the post-operative exercise stress test was 17.4 months, with a range from 6.7 to 30.2 months. There was a trend toward lower maximal consumption of oxygen after the procedure, at 36.3 plus or minus 7.6 millilitres per kilogram per minute (83.9% predicted) as opposed to 38.6 plus or minus 8.4 millilitres per kilogram per minute (88.5% predicted, p equal to 0.06). Patients after the procedure, however, had significantly increased adiposity, so that there was no difference in maximal consumption of oxygen indexed to ideal body weight before and after the operation. In 20 of the patients, aerobic capacity improved or was stable after the operation. There was no post-operative chronotropic impairment. Conclusions: In the majority of patients following the Ross procedure, exercise performance is stable and within the normal range of a healthy age and sex matched population, despite sedentary lifestyles and increased adiposity.

Background: To determine the clinical relevance of change in the level of lactate in the serum as a meanings of establishing the efficacy of the circulation immediately after the Fontan procedure in small children. Methods: We measured the concentration of lactate in the serum consecutively in 30 patients undergoing the Fontan procedure, without fenestration, under the age of 2 years. Of these, we did not use cardiopulmonary bypass in 13. Results: Concentrations gradually increased during the first several hours after establishment of the Fontan circulation, and then eventually decreased, reaching the normal range within 48 hours, with specific values of 0.9 plus or minus 0.3 pre-operatively, 2.3 plus or minus 1.1 immediately after creation of the Fontan circulation, 4.0 plus or minus 2.4 at 6 hours, 1.6 plus or minus 0.6 at 24 hours, and finally 1.3 plus or minus 0.4 millimoles per litre at 48 hours. This trend was irrespective of use or no use of cardiopulmonary bypass. The higher initial level in the intensive care unit was related to higher systemic venous pressure, higher transpulmonary pressure gradient, higher maximal level of alanine transaminase and blood urea nitrogen, and longer duration of peritoneal drainage, the latter as a monitor of sequestration of fluid rather than peritoneal dialysis. In patients with initial levels greater than 3.0 millimoles per litre, there was extended duration of endotracheal intubation. Conclusions: Postoperative changes in the levels of lactate in the serum subsequent to creation of the Fontan procedure were peculiar, not necessarily correlated with the cardiac output, but relevant to the clinical course.

Children with a functionally single ventricle constitute just over 1% of congenital cardiac defects.1 A majority of children with the functionally univentricular circulation undergo a three-staged reconstruction to achieve completion of the Fontan circulation. The first stage is usually performed in the neonatal period, and is either banding of the pulmonary trunk, an aorto-pulmonary shunt alone, or the shunt included as part of the first stage of reconstruction. In recent years, a conduit placed from the right ventricle to the pulmonary arteries is being used as an alternate source of flow of blood to the lungs. The second stage is the bidirectional cavopulmonary anastomosis, the two surgical variations being the so-called “hemifontan”, and “bidirectional Glenn” procedures, while the third stage is the completion of the Fontan circulation, the two surgical variations being either construction of a lateral tunnel, or placement of an extra-cardiac conduit, each being possible with or without a fenestration. In many centres, patients with the functionally univentricular circulation make up one-fifth of the total surgical volume. The syndrome of low cardiac output is quite common in this population through all three stages of reconstruction, and some of these patients will eventually require cardiac transplantation. While conventional therapy, with inotropic support and afterload reduction, remains the mainstay of therapy for the failing heart in children, mechanical support is being increasingly used.3 Most of this experience is limited to extracorporeal membrane oxygenation.2–5 In this review, we discuss the current experiences with extracorporeal membrane oxygenation in patients with a functionally univentricular circulation, and describes some of their unique features. We also focus on the pulsatile ventricular assist devices capable of providing support over the longer term, and other new devices that may have a role in the future in these patients.6

Objectives: The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation. Methods: We included all patients who had undergone an arterial switch operation at The Children's Hospital of Philadelphia, and had been followed for a minimum of 4 years, and had at least 2 postoperative echocardiograms. Neo-aortic valvar regurgitation was quantitatively assessed, and measurements were made of the neo-aortic root at the level of the basal attachment of the leaflets, mid-sinusal level, and the sinutubular junction. Results: We found 82 patients who satisfied the criterions for inclusion, of whom 52 patients had transposition with an intact ventricular septum, and 30 had either an associated ventricular septal defect or double outlet right ventricle. The median follow-up time was 8.8 years (4.1 to 16.4 years). The neo-aortic valve had been replaced in 1 patient. Of the patients, 3 had moderate, 66 had trivial to mild, and 12 had no neo-aortic valvar regurgitation at their most recent follow-up. The regurgitation had progressed by at least 1 grade in 38 of the 82 patients (46.4%). Neo-aortic dilation was noted at the basal attachment of the leaflets, and at mid-sinusal level, which was out of proportion to somatic growth. Conclusions: At mid-term follow-up, significant neo-aortic valve regurgitation is present in 3.7%, and trivial to mild regurgitation in 81.4% of patients. The regurgitation progressed in almost half of the patients over time. We also noted progressive dilation of the neo-aortic root out of proportion to somatic growth.

Objective: We reviewed our experience with mechanical valves inserted between 1988 and 2002 in children aged 15 years or younger. Methods: Hospital files were extracted retrospectively. Follow-up was completed by March 2005. Results: Of 41 patients, we inserted a valve in atrioventricular position in 27 children, having a median age of 3.1 years, ranging from 0.4 to 14.5 years, and in aortic position in the remaining 14, having a median age of 13.5 years, and a range from 7.0 to 14.9 years. For the valves inserted in atrioventricular position, the underlying disease was congenital in 23, rheumatic in two, post-endocarditic in one, and Marfan's syndrome in one. Mean follow-up was 7.7 years, with standard deviation of 5.3, giving a total follow-up of 209 patient years. Mortality at 30 days was 7%, and survival was 73% at up to 16 years follow-up. Events related to anticoagulation were seen in 3 patients, corresponding to 1.4% per patient year. In 6 patients (22%), heart block ensued which required implantation of a pacemaker treatment, and 5 patients (19%) had reoperations. For the implantations in aortic position, the underlying disease was congenital in 13, stenosis in 10 and insufficiency in three, and post-endocarditis in one. Mean follow-up was 6.8 years, with standard deviation of 4.6, giving a total of 95 patient years. We lost one patient within 30 days (7.7% mortality), and survival was 77% at up to 13 years follow-up. There were no incidents of thrombosis, nor events related to anticoagulation, but one patient (7%) needed insertion of a pacemaker due to a perioperative heart block, and one (7%) required new valvar replacement. Conclusions: Although preferably avoided, mechanical valves can be implanted in children with an acceptable mortality and morbidity, and good long-term results.

Ebstein's malformation is a rare congenital cardiac malformation, accounting for about 0.5% of all congenital cardiac lesions. We report our experience with the anomaly as encountered at the Sudan Heart Centre from July 2004 to April 2005. Diagnosis was based on the echocardiographic demonstration of displacement of the septal leaflet of the tricuspid valve towards the ventricular apex by greater than 8 millimetres per metre squared. Results: In a period of 10 months, we identified 12 patients with the malformation, this number constituting 2% of all patients seen with congenital cardiac disease. The age ranged from 2 weeks to 35 years. Of the patients, half were asymptomatic, while the other half presented with congestive cardiac failure, 4 of these having cyanosis in addition to heart failure. Associated diseases included chronic renal failure, impaired hearing, stunted growth, and developmental delay. Electrocardiographic abnormalities included peaked and tall P waves, seen in four-fifths, an Rsr pattern, first degree atrioventricular block, atrial fibrillation, and Wolf–Parkinson–White syndrome, including the Mahaim pattern of pre-excitation. Associated abnormalities diagnosed echocardiographically included atrial septal defects, prolapse of the leaflets of the mitral valve, left ventricular dysfunction, atrial septal aneurysm, pulmonary valvar stenosis, and pericardial effusion. Conclusions: Ebstein's malformation was seen four times more frequently at the Sudan Heart Centre when compared to the average frequency reported in the Western literature. Many of the associated diseases encountered in Sudan had not previously been reported.

Procalcitonin appears to be an early and sensitive marker of bacterial infection in a variety of clinical settings. The use of levels of procalcitonin to predict infection in children undergoing cardiac surgery, however, may be complicated by the systemic inflammatory response that normally accompanies cardiopulmonary bypass. The aim of our study was to estimate peri-operative concentrations of procalcitonin in non-infected children undergoing cardiac surgery. Samples of serum for assay of procalcitonin were obtained in 53 patients at baseline, 24, 48, and 72 hours following cardiac surgery. Concentrations were assessed using an immunoluminetric technique. Median concentrations were lowest at baseline at less than 0.5 nanograms per millilitre, increased at 24 hours to 1.8 nanograms per millilitre, maximized at 48 hours at 2.1 nanograms per millilitre, and decreased at 72 hours to 1.3 nanograms per millilitre, but did not return to baseline levels. Ratios of concentrations between 24, 48 and 72 hours after surgery as compared to baseline were 6.15, with 95 percent confidence intervals between 4.60 and 8.23, 6.49, with 95 percent confidence intervals from 4.55 to 9.27, and 4.26, with 95 percent confidence intervals between 2.78 and 6.51, respectively, with a p value less than 0.001. In 8 patients, who had no evidence of infection, concentrations during the period from 24 to 72 hours were well above the median for the group. We conclude that concentrations of procalcitonin in the serum increase significantly in children following cardiac surgery, with a peak at 48 hours, and do not return to baseline within 72 hours of surgery. A proportion of patients, in the absence of infection, had exaggerated elevations post-operatively.

The association of atrioventricular septal defect with common atrioventricular junction and malformations of the ventricular outflow tracts presents a significant challenge for the surgeon. In the most common of these, the association with tetralogy of Fallot, several surgical techniques have been described, and shown to deliver excellent results.1–10 On the other hand, in the setting of more extreme malformations, such as double-outlet right ventricle, discordant ventriculo-arterial connections, or common arterial trunk, albeit rare lesions, the combination presents a more formidable surgical challenge, as evidenced by the few reports of successful repair of these lesions. This challenge is both physiological, when dealing with a very sick neonate or infant, as well as anatomical in terms of the complexity of the malformation and the ability to achieve a successful biventricular repair. Our goal in this review is to discuss the surgical treatment in the setting of tetralogy of Fallot and double outlet right ventricle, with emphasis on biventricular repair.

We present a male infant, born at term, who collapsed and died at 30 minutes of age. Autopsy demonstrated concordant atrioventricular and discordant ventriculo-arterial connections with premature closure of the oval foramen, which bulged markedly into the left atrium. Premature closure of the oval foramen is rare in the setting of transposition. As far as we know, there have been only three previously reported cases.

Background: Transoesophageal echocardiography has become a powerful tool in the diagnosis and management of children with congenital cardiac malformations. Unlike adults, children will not tolerate transoesophageal echocardiography under light sedation. This study was undertaken, therefore, to evaluate the safety and efficacy of deep sedation with propofol for transoesophageal echocardiography in children examined in an outpatient setting. Methods: This is a retrospective study of patients undergoing transoesophageal echocardiography with propofol given in bolus aliquots to achieve a level of sedation adequate to insert the transoesophageal echocardiographic probe and maintain sedation throughout the procedure. Results: We included a total of 118 patients, 57% being male, with a mean age of 12.9 years. Adequate sedation was achieved using a mean propofol dose of 8.3 milligrams per kilogram, with the dose per kilogram decreasing concomitant with increasing weight of the patient. Patients less than two years of age were intubated for the procedure. There were no clinically significant changes in cardiac function or haemodynamics. Non-intubated patients received supplemental oxygen prior to, or just after, the onset of sedation, with transient hypoxaemia observed in one-fifth. Complications were rare, with minor problems occurring in 7.6%, and major ones in 4%. Conclusions: Transoesophageal echocardiography can be performed on an outpatient basis in children with a wide spectrum of congenital cardiac malformations, and propofol is an ideal sedative agent in this setting. Although not common, preparations must be made for significant haemodynamic and respiratory complications. In our study, we intubated all the children under 2 years of age.

Fenestration is still widely used in right heart bypass operations. Our study was conducted to assess its need in the most recent modification, the completion of a total cavopulmonary connection with an extracardiac tube.

The extracardiac approach was introduced at our institution in January, 1999. Since June of 2000, no patient had a fenestration. If more than 1 risk factor amongst ventricular function being more than moderately impaired, atrioventricular valvar regurgitation more than moderate, mean pulmonary arterial pressure more than 15 millimetres of mercury, mean atrial pressure higher than 12 millimetres of mercury, pulmonary arterial distortion, or other than sinus rhythm was present preoperatively, the patient was considered a “high risk” candidate. Postoperatively elevated pulmonary arterial pressure higher than 16 millimetres of mercury, prolonged effusions and requirement for drainage longer than 7 days, and death were considered endpoints in the statistical analysis.

Our study group included 84 patients who underwent surgery up to August, 2004. A previous bidirectional cavopulmonary anastomosis had been accomplished in 73 patients at a mean age of 27.01 plus or minus 32.60 months, with a median of 11.5 months, without creating an additional source of flow of blood to the lungs.

At the time of the total cavopulmonary connection, the mean age was 66.4 plus or minus 60.1 months, with a median of 37.1 months, and a range from 17.3 to 251.2 months, with 50 patients being younger than 48 months.

We deemed 16 patients to be at “high risk”. These patients were older at the time of bidirectional cavopulmonary anstomosis (p smaller than 0.016), at the time of completion (p smaller than 0.019), and also differed in size at time of completion (p smaller than 0.020). They required a longer time on cardiopulmonary bypass (p smaller than 0.015), and reached higher early postoperative pulmonary arterial pressures after completion (p smaller than 0.025). There were no differences between groups of patients having up to 1 or more risk factors in regard to need for intubation (p smaller than 0.511), pulmonary arterial pressures after extubation (p smaller than 0.817), and duration of chest drainage (p smaller than 0.650). Three patients died, one in the group deemed at high risk. There was no death in the last 38 patients.

We conclude that a total cavopulmonary connection with an extracardiac tube can be performed without fenestration, even if the patients are deemed to be at increased risk. Early staging of patients with functionally univentricular physiology might be one of the keys for these findings.

Aims: Our purpose was to assess the value of magnetic resonance angiography as a non-invasive alternative to catheterization in the evaluation of patients with tetralogy of Fallot, including those with pulmonary atresia. Methods and results: We evaluated prospectively, using magnetic resonance angiography, 30 patients, aged from 1 to 18 years, 15 with tetralogy of Fallot and pulmonary stenosis, and 15 with pulmonary atresia. The studies obtained using magnetic resonance provided adequate visualization of the aorta, and provided excellent imaging of the pulmonary trunk and its right and left branches. Compared with catheterization, magnetic resonance had 100 percent sensitivity, specificity and accuracy for defining the presence or absence of the pulmonary arteries. Magnetic resonance also had 93.9 percent sensitivity, 98.2 percent specificity, and 96.7 percent accuracy for detection of stenosis or hypoplasia of the pulmonary arteries. We detected 25 major aortopulmonary collateral arteries with magnetic resonance, but only 22 with conventional angiography. There was complete agreement between the two methods in detecting patency of the arterial duct in 6 patients, and of Blalock-Taussig shunts in 12 patients. Conclusion: Magnetic resonance angiography is a useful tool in the evaluation of patients with tetralogy of Fallot. It can be considered a non-invasive alternative to cardiac catheterization in the evaluation of the pulmonary vascular anatomy.

The most appropriate way of describing the congenital cardiac malformations unified because the atrial chambers are joined across the atrioventricular junctions to morphologically inappropriate ventricles has long been contentious. In the past, the lesions have been described in such arcane terms as mixed levocardia,1 while “ventricular inversion” still retains it currency in some circles. As we will show in this review, the abnormal arrangements at the atrioventricular junctions can be found with various patterns, but most frequently the patients also have the arterial trunks arising from morphologically inappropriate ventricles. This combination is best described as congenitally corrected transposition, and will form the focus of our review. It is salutary to note that, when von Rokitansky gave the first description of this combination,2 one of his illustrations was ideally suited to aid the understanding of modern-day echocardiographers (Fig. 1). We hope to emulate von Rokitansky in our own review.

For children with hypoplastic left heart syndrome, contemporary management over three stages includes a window of high risk for sudden death between the initial stage of palliation, the Norwood operation itself, and the second stage, creation of the bidirectional superior cavopulmonary connection. The risk is highest at a time when patients have been discharged from the hospital to grow and prepare for the second stage,1–4 and has persisted despite the remarkable improvements in immediate postoperative and hospital survival after the initial surgery.5,6 Potential contributing factors to the increased vulnerability to sudden death between the stages include the limited circulatory reserve inherent in the parallel circulations supported by a functionally univentricular heart, the reliance on a prosthetic shunt which is susceptible to thrombosis, and congenital or acquired anatomical cardiovascular abnormalities such as aortic atresia, residual obstruction in the aortic arch, tricuspid valvar insufficiency, or right ventricular dysfunction.7–12