Basic scientists, as well as cardiologists, are caught by the idea of curing ischaemic heart disease with cardiac progenitor or stem cells. This short review provides an overview of our current knowledge on the potential use of stem cells for cardiac disease. Since, in infants and children, aetiologies and pathomechanisms of critical cardiac disease are fundamentally different from those in adults, we will also address the question as to whether such young patients could be a therapeutic target at all, and in which respect it may be necessary to view treatment with stem cells from a different stance in the developing organism.

Aims: To evaluate the quality of cardiac and surgical care provided for children with congenital cardiac malformations in the eastern county of Hungary. Methods: We used the method of clinical audit based in selection of criterions, developing five such criterions concerning timely diagnosis, access to treatment, and outcome. To examine compliance with these criterions, we analysed retrospectively the routine data relating to children living in Hajdu-Bihar County. The period of observation lasted from January, 1994, until December, 2001, and was divided into two epochs in order to evaluate any changes over time. Results: In the first epoch, 28 infants, representing 0.1% of all newborns, died of congenital cardiac disease, with one of the malformations being recognised post mortem. In the second epoch, 21 infants died, representing 0.09% of newborns. In this group, each malformation was diagnosed before death. In each period, 6 infants died without having undergone cardiac surgery, and having no other non-cardiac disease. The overall postoperative mortality was 9.2% for the first period, and 4.6% for the second. The number of patients waiting for non-urgent repair had reduced significantly by the end of the second epoch. Conclusions: The results show that the timely diagnosis of congenital cardiac malformations is in line with accepted international standards. Conversely, the access to invasive treatment was limited over the period of observation, albeit that the waiting list was reduced significantly by the end of the second epoch. The postoperative mortality for those suffering congenital cardiac malformations is also comparable with international standards, except for mortality during infancy for treatment of complex anomalies. Our audit highlights the need for surgical repair of the more complex malformations during infancy.

The study was designed to investigate the value of assessing right ventricular volume by transoesophageal 3-dimensional echocardiographic techniques compared with the standard transoesophageal cross-sectional approach. Echocardiography was performed using a multiplane probe. The 3-dimensional data sets were reconstructed after electrocardiographic and respiratory gated scanning, calculating the 3-dimensional volumes by the method of multiple slices. Cross-sectional determination of volume was performed using a modified area-length method, and the biplane multiple slice method following Simpson's rule. We studied 15 patients, with ages ranging from 6 to19 years, and body surface areas from 1.1 to 1.67 square metres. It proved possible top determine volumes with both methods in all patients. As determined by 3-dimensional echo, volumes were greater, being 113.0 plus or minus 61.2 millilitres at end-systole, and 61.7 plus or minus 36 millilitres at end-diastole, than those calculated from cross-sectional images using Simpson's rule, which gave values of 92.5 plus or minus 52 millilitres, and 41.3 plus or minus 22 millilitres. Compared to the values obtained using the area-length method, at 116.9 plus or minus 61 millilitres, and 60.3 plus or minus 30 millilitres, there were only small differences at end-systole, with a bias of 1.4, and limits of agreement of 20.9 millilitres, as well as at end-diastole, when bias was minus 3.8, and limits of agreement 22.3 millilitres. Correlation was also good, with coefficients of 0.93, and 0.91, respectively. The mean difference between the volumes by 3-dimensional acquisition and the multiple slice method was larger, with higher limits of agreement, at end-diastole showing bias of 20.5, and limits of agreement of 30.1 millilitres, and for end-systole bias of 20.4, and limits of agreement of 32.2 millilitres. Our data confirm that cross-sectional echocardiographic assessment of right ventricular volumes in children with atrial septal defects is quick, and reasonably reliable in clinical practice when employing the area-length method.

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.

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.

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.

Infants who survive open-heart surgery are at risk for developmental disability, which may impact on the well-being not only of the child, but also the family. The objective of our prospective study, therefore, was to determine the long-term health-related quality of life of children with congenital cardiac malformations following open-heart surgery, and to describe the persisting level of stress in their families. To this end, 49 parents completed the Child Health Questionnaire, the Parenting Stress Index, and the Child Behaviour Checklist as part of a developmental follow-up protocol when their child was 5 years of age. Mean scores on the Child Health Questionnaire were in the normal range, with physical well-being equal to 53.5, psychosocial well-being 50.9, with only 6.4 percent and 8.5 percent of subjects, respectively, falling within the suboptimal range of less than 40. The distribution of scores on the Parenting Stress Index, however, were more variable, with over one-quarter of parents indicating a high level of stress, with almost one-fifth having low levels of stress, and just over half scoring in the normal range, with the group mean being 52.6 plus or minus 32.3. An abnormal neurologic examination before surgery was associated with lower physical health (β equal to −5.5, p equal to 0.02, r2 equal to 0.18), whereas lower arterial saturations of oxygen, less than 85 percent preoperatively, was associated with lower psychosocial health (β equal to −6.6, p equal to 0.01, and r2 equal to 0.14). The internalizing and externalizing behaviours of the child were significantly correlated with psychosocial well being, with r ranging from −0.32 to −0.52, and p less than 0.05. Parental stress also correlated with psychosocial health (r equal to −0.48 and p equal to 0.0009). Overall, the perception by the parents of the health-related quality of life of their child is favourable 5 years following open-heart surgery during infancy. Many parents, nonetheless, continue to feel either stressed or defensive about their child, particularly if their child exhibits behavioural difficulties. Our findings suggest that strategies need to be considered to enhance family well-being in the planning and delivery of health services to this population at high risk.

The purpose of the study was to develop a questionnaire measuring health-related R1 quality of life for children and adolescents with congenital heart disease, the ConQol, that would have both clinical and research applications. We describe here the process of construction of a questionnaire, the piloting and the development of a weighted scoring system, and data on the psychometric performance of the measure in a sample of 640 children and young people recruited via 6 regional centres for paediatric cardiology from across the United Kingdom. The ConQol has two versions, one designed for children aged from 8 to 11 years, and the other for young people aged from 12 to 16 years. Initial findings suggest that it is a valid and reliable instrument, is acceptable to respondents, and is simple to administer in both a research and clinical context.

Background: Our purpose was to determine the outcome of pregnancy and delivery in patients with coronary arterial lesions caused by Kawasaki disease. Methods and Results: We surveyed by mail the Japanese national experience of pregnancy and delivery in patients known to have Kawasaki disease. The first questionnaire was returned by 154 of 207 (74%) institutions, and 16 of the 154 had knowledge of deliveries in their patients. Based on a second questionnaire, and previous Japanese case reports, we identified 46 deliveries in 30 patients from 16 institutions. The age at delivery ranged from 18 to 35 years, with a median of 27 years. Of the patients, 4 had undergone coronary arterial bypass grafting. Low-dose aspirin was given in 16 patients. The deliveries, 27 in all, had been vaginal in 20 patients, albeit that 7 required assistance by forceps or vacuum extraction under epidural anesthesia. Caesarean section had been performed in 11 patients, 3 for obstetric indications, and 1 for chest discomfort in the third trimester. Although there were no cardiac events, obstetric complications occurred in 2. Conclusion: The results of pregnancy and delivery were favourable. The mode of delivery should be primarily determined by obstetrical considerations, rather than the coronary arterial lesions caused by Kawasaki disease. Excessive anticoagulant therapy may not be needed for this population.

Common arterial trunk is frequently associated with anomalies of coronary arterial origin and distribution. We describe two infants in whom coronary intervention was performed during the repair of common arterial trunk, with reversal of ischaemic left ventricular dysfunction. We discuss the surgical implications of recognition of the coronary arterial anomalies during the repair of common arterial trunk.

A 16-year-old girl with no previous cardiac problems presented to the casualty with septic shock. Investigations revealed endocarditis involving the mitral valve. At the time of surgery, she was noted to have a large left atrial appendage herniating into the left pleural cavity due to partial absence of the pericardium. Complete or partial absence of the pericardium is an uncommon congenital anomaly. Though various complications and presentations are reported, it has not been identified as a risk factor for endocarditis. We discuss the possible mechanism, and need for prophylaxis against endocarditis, in patients with absence of the pericardium.

Anomalous drainage of the right superior caval vein into the morphologically left atrium as an isolated cardiac malformation is a rare anomaly. Most patients present with cyanosis. Thus far, about 20 cases have been reported in the literature. We report a case of cyanosis due to this malformation in a male neonate, which was complicated by the meconium aspiration syndrome. The malformation was diagnosed by echocardiography and cardiac catheterization. Surgery resulted in complete recovery.

Three patients with heart failure after chronic right ventricular apical pacing were treated with resynchronization. Biventricular pacing was used for two patients, and the other was treated with left univentricular pacing. In all patients, we observed a dramatic improvement of left ventricular dimension, function, and clinical state. We conclude that biventricular or left ventricular pacing is superior to right ventricular apical pacing in children who are pacemaker-dependent.

Acute ingestion of large quantities of alcohol is known to be able to trigger acute myocardial infarction. A 19-year-old boy presented at the emergency department of our hospital with complaints of severe retrosternal chest pain. One night before this event, he had drunk large amounts of alcohol. The level of alcohol in his blood was measured at 0.59 grams per litre. A 12-lead electrocardiogram showed elevations of the ST segment, averaging from 2 to 10 millimetres, in leads V1-6, DI and aVL. Since consumption of alcohol is very common in the community, the triggering effect of binge-drinking and consumption of large amounts of alcohol on acute myocardial infarction should be considered as a crucial subject for public health so as to raise the consciousness of the population, especially young persons.