Pulmonary vascular disease is a risk factor for the surgical management of common arterial trunk. Surgical repair, therefore, is usually performed in early infancy, before irreversible changes can occur in the epulmonary vasculature. Because of this, there has been an increasing tendency to dispense with cardiac catheterisation as a means of assessing pulmonary vascular disease. Cardiac catheterisation, nonetheless, is still performed when there is a risk of pulmonary vascular disease, such as in older children. There are no clear guidelines, however, as to who should be catheterised. We have developed a simple screening test to help make this decision.

Objective: Primary implantation of stents is an accepted technique for treating aortic recoarctation, albeit that the effects of stenting on pressure profiles, carotid mechanical properties, intimal medial thickness, and reflection of the pulse wave have not been systematically investigated. Methods: Over the period from 1 January, 1999, to 31 December, 2002, we implanted stents to relieve aortic recoarctation in 15 patients, with a median age of 17 years, and a range from 7 to 29 years, with a median weight of 56 kilograms, ranging from 20 to 96 kilograms. Indications were a gradient of 20 millimetres of mercury or more measured in all, systemic hypertension at rest in 8, and systemic hypertension at exercise in all. Of the patients, 5 were receiving anti-hypertensive treatment. Before implantation of the stents, and after a mean follow-up of 22 months, all patients underwent an exercise test, vascular echography, and examination of the common carotid artery so as to determine its cross sectional compliance and distensibility, and the augmentation index. Results: The stents were implanted successfully in all patients. The mean gradient was reduced from 27 to 4 millimetres of mercury (p < 0.001). Systolic blood pressure at rest diminished from 140 to 131 millimetres of mercury (p = 0.04), while hypertension at rest regressed in 4 patients. Systolic blood pressure at exercise diminished from 245 to 222 millimetres of mercury (p = 0.018), and hypertension at exercise regressed in 1 patient. Anti-hypertensive treatment is still required for 4 patients. A correlation was found between systolic blood pressure at rest and initial peak-to-peak gradient (r = 0.8), and between initial gradient and percentage reduction of systolic blood pressure at rest at follow-up (r = −0.73). Compliance and distensibility of the common carotid artery were not significantly modified, albeit that the intimal medial thickness diminished from 0.64 to 0.57 millimetres (p = 0.04), and the augmentation index decreased from 5 to −1 (p = 0.012). Conclusions: Primary implantation of stents is effective in mid-term repair of aortic recoarctation. Although there is an improvement in systemic hypertension, the tensional profile and vascular sonography are not normalized. At long term follow-up, the suppression of an early reflection site of the pulse wave could decrease the wall stress of the great elastic vessels, reducing the thickness of the arterial walls.

Aims: To determine the number of infants in the Mersey and North West regions with congenital cardiac disease for whom palivizumab may be appropriate, and to examine the potential impact of introducing prophylaxis with palivizumab on these patients and their economic management. Methods: We identified those infants deemed to be at high risk, matching the population recently studied by the Cardiac Synagis Group, from the database of the cardiology department of the hospital. The number of patients under the care of the paediatric cardiologists admitted to Alder Hey Hospital with respiratory syncytial viral bronchiolitis over the last three seasons was identified from hospital coding records, and the database of the cardiology department. Results: There are 131 patients at high risk each year. Of these, over the last three “bronchiolitis seasons”, 39 infants have been admitted to the hospital with bronchiolitis due to the respiratory syncytial virus. This represents a hospitalisation rate of 10 per cent, as was seen in the study of the Cardiac Synagis Group. Using a monthly dose of 15 milligrams per kilogram for five doses, the cost per patient is 2,650 pounds sterling for the season. To treat the 131 patients seen at Alder Hey, therefore, would cost 346,800 pounds each year. Applying the reductions in hospitalisation identified in the study by the Cardiac Synagis Group to our population would produce an expected reduction in patients hospitalised from 13 to 7 per year, reducing the total length of stay in our hospital wards from 169 to 76 days, and in the paediatric intensive care unit from 93 to 21 days. This amounts to a potential saving of 190,800 pounds per year. Reducing transfers to more distant paediatric intensive care units for referrals refused because of lack of beds could save an additional 50,000 pounds. Discussion: We estimate the net cost of introducing palivizumab for this population to be 106,000 pounds per year. There would, of course, be additional costs involved in setting up this service, as well as additional savings and benefits. This cost is comparable with other new biologic therapies now routinely used in the United Kingdom, such as etanercept for juvenile arthritis. There are, currently, no other obvious therapies that have the potential to reduce admissions to hospital and intensive care during the winter months, when beds are at their most scarce.

The respiratory syncytial virus is the most common cause of infection of the lower respiratory tract in infants and young children, and is the leading cause of hospitalisation and death due to viral illness during the first year of life. In otherwise healthy infants, the virus usually causes only mild respiratory illness, but premature babies and infants with chronic lung disease, those with congenitally malformed hearts, or those who are immunodeficient, are at increased risk of serious illness, hospitalisation, and death. Recent infection with the virus is also associated with increased postoperative complications after corrective surgery for congenitally malformed hearts. No effective vaccine is currently available, and treatment is limited to supportive therapy. Prevention in groups deemed to be at high-risk, therefore, is essential. In addition to measures for control of infection, prophylactic immunotherapy is indicated in selected patients. Palivizumab (Synagis®) is a monoclonal antibody indicated for the prevention of serious viral disease of the lower respiratory tract in premature infants, those with chronic lung disease, and those with haemodynamically significant congenital cardiac lesions. Palivizumab is given intramuscularly, usually as a monthly injection during the so-called “season”. In a recent international, randomised, double-blind, placebo-controlled trial in 1,287 children less than or equal to 2 years old with haemodynamically significant congenital cardiac malformations, prophylaxis achieved a relative reduction of 45 per cent in the incidence of antigen-confirmed viral-related hospitalisation, and reduced the duration of hospital stay by 56 per cent. National and international guidelines, therefore, now recommend routine prophylaxis in the first year of life in children with haemodynamically significant congenital cardiac disease.

All newborn infants have limited pulmonary reserve compared with older children. This puts them at increased risk of respiratory complications, such as those associated with infection by the respiratory syncytial virus. Young children with congenital cardiac disease are particularly likely to suffer severe disease related to infection by the virus. In these children, the extreme vulnerability of the lung to pulmonary oedema is compounded by the additional burden caused by the respiratory syncytial virus.

In addition to the well-documented acute pulmonary effects of infection with the respiratory syncytial virus, there may also be consequent long-term respiratory morbidity. Clinical studies have shown that infection by the virus in infancy is associated with a higher risk of developing subsequent bronchial obstructive disease. Much debate surrounds the mechanisms underlying this association. It is thought that a combined immunological and neurogenic response mechanism is likely. Prevention of severe respiratory disease in infants and young children with congenital heart disease due to infection by the virus may, therefore, offer both immediate and long-term benefits. Indeed, an increasing body of evidence supports this hypothesis, indicating a clinical rationale for prophylaxis against the virus in infancy, in order to reduce the chance of developing reactive airways disease and asthma in later life.

A recent, randomised, double-blind, placebo-controlled trial has demonstrated the effectiveness of palivizumab (Synagis®) for prophylaxis against infection by the respiratory syncytial virus in 1,287 young children with congenital cardiac disease. Guidelines for the use of palivizumab in these children considered to be at high risk were published by the American Academy of Pediatrics, followed by similar guidelines from the British Paediatric Cardiac Association, and recommendations from a number of other individual countries, including Canada, Germany, Spain, and France.

In May, 2004, further discussion was held between a group of 15 paediatric cardiologists at a European forum for clinical management held in Munich, Germany. The objective of this forum was to define optimal recommendations on prevention of infection by the respiratory syncytial virus in infants and young children with congenital cardiac disease, appropriate to the clinical needs and style of those practising in paediatrics in individual countries. Participants were invited because of their knowledge of the therapeutic area, and for their experience of using palivizumab for prophylaxis against the respiratory syncytial virus in children with congenitally malformed hearts. Measures to educate the carers of children with such congenital malformations on precautions against infection by the respiratory syncytial virus were discussed, along with the many different aspects of best practice for therapeutic prophylaxis with palivizumab. The most appropriate timing of prophylaxis, recommendations for which children are most likely to benefit from prophylaxis, and suggested protocols were among the issues covered. The recommendations resulting from the discussions are presented in this paper, as a step towards reaching consensus.

Background: We have used the Amplatzer ductal occluder for transcatheter closure of large persistently patent arterial ducts, and used our experience to assess the safety and efficacy of the device in young children and infants. Methods and patients: We used the Amplatzer ductal occluder prospectively in 43 patients with large patent arterial ducts, reviewing our experience to identify any problems or complications. Results: The procedure proved successful in 42 of the patients. We achieved complete occlusion of the duct in 33 (78.5 per cent) of the patients on the day of insertion. In 6 additional patients, complete occlusion occurred 1 week to 6 months after the procedure. Trivial leaks persisted in 2 patients, while one had a significant residual leak. Problems were encountered in 7 patients. The procedure failed in one, a device was wasted in 2, pulled through in 3, while we experienced kinking of the long Mullins sheath, being unable to retrieve the device, in one patient. Minor complications occurred in 6 patients, finding flow at a peak velocity of 2.2 metres per second in the descending aorta in 2 patients, and at 2.5 metres per second in 2 further patients, and flow at 2.5 metres per second in the pulmonary arteries of two patients. One patient experienced a major complication due to excessive bleeding. Out of the 14 patients suffering adverse events, 13 weighed less than 10 kilograms. This rate of problems and complication in these patients weighing less than 10 kilograms was significantly higher than in the patients weighing more than 10 kilograms. Conclusion: Transcatheter occlusion of moderate to large patent arterial ducts with the Amplatzer ductal occluder device is safe and effective, with a high rate of complete occlusion. Problems and minor complications may be encountered in children weighing less than 10 kilograms. If the device is to be deployed completely in the ductal ampulla, and to avoid descending aortic obstruction, the size of the retention flanges of the occluder should not exceed the largest diameter of the patent arterial duct.

Background: Congenital cardiac disease is the greatest cause of death in patients with Down's syndrome during the first two years of life, with from two-fifths to two-thirds of those with Down's syndrome also having congenital cardiac malformations. The lesions within the heart can be single or multiple. Our objective was to evaluate the frequency and type of such congenital cardiac malformations in patients born with Down's in Guatemala, and to provide baseline information for further research. Methods: We reviewed all patients with Down's syndrome who underwent a cardiologic screening examination between January, 1997, and December, 2003, in the only department dealing with Paediatric Cardiology in Guatemala. Results: Of the 349 patients reviewed, 189 (54.1 per cent) also had an associated congenital cardiac malformation. The median age at diagnosis was 6 months, with a range from 2 to 13 months. In 152 patients (80.4 per cent), the cardiac lesion was isolated, while 37 patients (19.6 per cent) had multiple defects. The most common single defect was patency of the arterial duct, found in 54 of the 189 patients (28.6 per cent), followed by ventricular septal defect in 27.5 per cent, atrial septal defect in 12.7 per cent, and atrioventricular septal defect with common atrioventricular junction in 9.5 per cent. The most frequent concomitant malformation found co-existing with other congenital cardiac lesions was patency of the arterial duct, found in 17.5 per cent. Conclusions: As far as we are aware, ours is the first epidemiologic study concerning the frequency and type of congenital cardiac disease found in Guatemalan children with Down's syndrome. The high frequency of patency of the arterial duct, and the differential distribution of the cardiac malformations associated with Down's syndrome among Guatemalan children, differ from what has been reported in the United States of America, Europe, and Asia. This difference warrants further research.

Obesity in childhood, which is associated with cardiovascular risk factors such as hypertension, is on the increase. Countermeasures are necessary. In this paper, we present the baseline and final data from the StEP TWO programme, a prospective study to prevent overweight and obesity in primary schools. Methods: We recorded and calculated, from 1689 children, anthropometric data, including analyses of bioelectric impedance, waist and hip circumferences, body mass index and its standard deviation, and the ratio of waist to hip. Blood pressure was measured after 5 minutes at rest. From the three schools involved in a programme of intervention, 121 children were invited to take part, and 40 (33.1 per cent) completed the programme. The effect was compared with 155 overweight and obese children identified at the 4 control schools. Results: 830 (49.5 per cent) boys and 848 girls (50.5 per cent) took part. Their mean age was 8.2 plus or minus 1.3 years, their height was 1.31 plus or minus 0.09 metres, they weighed 30.0 plus or minus 8.2 kilograms, and their mean index of body mass was 17.1 plus or minus 2.9 kilograms per metre squared. Of the children, 7.3 per cent were obese, 10.4 per cent were overweight, 75.7 per cent had normal weights, and 6.6 per cent were underweight. Resting hypertension was observed in 2.3 per cent of the children. Increased blood pressure was associated with a higher body weight, body mass index, standard deviation score for body mass index, and waist and hip circumferences (each p < 0.001), but not with the ratio of waist to hip. Hypertension at rest was also found in 11.0 per cent of obese children, 4.4 per cent of those who were overweight, 1.2 per cent of those with normal weight, and 1.0 per cent of underweight children (p < 0.001). After the intervention, the increase of the body mass index tended to be lower in those in whom we had intervened (p = 0.069), and in these the decrease of the standard deviation score for body mass index was significantly higher (p = 0.028). Systolic blood pressure was reduced by about 10 millimetres of mercury in those in whom we had intervened (p = 0.002), while there were no changes in the control group. Diastolic blood pressure was lowered by 3 millimetres of mercury, but this was not significant. Conclusion: Obese children had the highest values for systolic and diastolic blood pressure. Increased levels of blood pressure are associated with other parameters of obesity, such as the circumference of the waist and hip. Early preventive measurements in childhood are necessary, and appropriate intervention appears to be effective.

Objectives: To report our experience in providing cardiac technician led paediatric echocardiography services in a district general hospital in the United Kingdom. Methods: We have collected prospectively the numbers of referrals, and the proportion of abnormal echocardiograms, since inception of the service in 2000. In additional, for a period of 12 months, we have audited in detail the patterns of referral to the service, and outcomes, assessing the effect of the service on the outreach clinic run by a visiting paediatric cardiologist. Results: Use of the system resulted in detection of a wide range of abnormalities, with our audit showing that the patients received appropriate management. The total referrals to the service increased 10 fold over the 4 year period of the study. The proportion of abnormal hearts detected by echocardiography, however, dropped from 90 per cent to 16 per cent over the same period. The numbers of patients seen in the outreach cardiology clinic remained unaltered. Conclusions: Having been proved to be an effective model for the triage of children with suspected congenital cardiac disease, adoption of a cardiac technician led echocardiographic service has seen a dramatic increase in the numbers of echocardiograms requested, without decreasing the workload of the visiting paediatric cardiologist.

We describe an unusual right atrial aneurysm, occurring with progressive obstruction of the right ventricular outflow tract and increasing cyanosis, in a nine-month-old boy with chromosome 8(p23.1) deletion. Surgical resection of the diverticulum, and relief of the right ventricular obstruction, was successful, although impairment of right ventricular compliance persists after 19 months follow-up.

We describe an occurrence of long QT syndrome in association with persistent patency of the arterial duct in members of a family. Patency of the arterial duct was diagnosed in family members of 4 successive generations. The index case had long QT syndrome associated with such a patent duct. Another patient had long QT syndrome, but associated with a ventricular septal defect. We postulate that there may be a common genetic mechanism for long QT syndrome and persistent patency of the arterial duct.

Over a period of 6 days, a three-week-old male developed a long-segment coarctation, with sub-total obstruction of the descending aorta, immediately distal to the left subclavian artery. On the 24th day of life, the stenotic region was repaired by placement of a pulmonary allograft patch measuring 3 centimetres in length. Severe diffuse vascular medial thickening was discovered at the operation. Subsequent fluorescence in-situ hybridization proved positive for Williams' syndrome. To our knowledge, this is the first report of rapidly progressive infantile arteriopathy in the setting of Williams' syndrome.

Radiofrequency lesions can, theoretically, be the substrate for new persistent arrhythmias. As far as we know, this has never previously been encountered after transcatheter ablation of accessory pathways. A child with Wolff–Parkinson–White syndrome was referred for radiofrequency catheter ablation of a left-sided accessory pathway. After successful ablation of the accessory pathway using a retrograde transaortic approach, the child developed an incessant wide QRS complex tachycardia at slow rate that was resistant to pharmacologic interventions. The focus of the tachycardia was identical to the ventricular site of insertion of the eliminated accessory pathway.

We report a case where fetal echocardiography identified both complete heart block and ventricular tachycardia. The mother tested positive for anti-Ro antibodies. Prenatal detection of this unusual combination of arrhythmias prompted early postnatal evaluation, which revealed prolongation of the QT interval. Autoimmune mediated congenitally complete heart block associated with such prolongation of the QT interval has a poor prognosis. The child was successfully treated with beta blockers and implantation of a pacemaker.

Idiopathic aneurysms of the inferior caval vein are uncommon in children and adults. We describe a 14 year old boy with a saccular aneurysm of the inferior caval vein, in whom no surgical intervention was required to repair the aneurysm. The patient is being followed-up periodically for the evaluation of any increase in the diameter of the inferior caval vein.