Aims: To compare the rates of employment, and advice offered concerning careers, in adults with congenital heart disease and controls. To assess the impact of the severity of the congenital cardiac malformation on the chances for employment. Methods: We solicited responses from 299 adults with congenitally malformed hearts, asking them to answer questions from a questionnaire posed at interview by a trained nurse. The adults were asked to give an identical questionnaire to a friend to act as a control. We received responses from 177 of the controls. Results: The responses showed that 51 of 156 (33 per cent) adults with congenital heart disease were unemployed, and 37 of 151 (25 per cent) had been unemployed for more than a year. This is significantly more than 25 of 156 (16 per cent) matched controls unemployed, and 5 of 151 (3 per cent) controls unemployed for more than a year. Almost one-fifth of the adults with congenital heart disease (19 per cent) had received advice regarding their career which they found helpful, which is significantly fewer than the 31 per cent of controls. More (42 per cent) had been given advice against certain occupations than controls (11 per cent). Receiving career advice was significantly associated with employment in the population with congenitally malformed hearts, with almost three-quarters (73 per cent) of those given advice being employed compared to 46 per cent of those not given advice. This pattern was not seen in controls. The severity of the congenital cardiac malformation did not significantly affect the rates of unemployment. Conclusions: Whatever the severity of their disease, adults with congenitally malformed hearts are more likely to be unemployed than matched controls. They are less likely to receive useful advice regarding potential careers, and find the advice given less helpful, than controls, although receiving suitable advice is associated with being employed in the population with congenital cardiac disease.

It's always hard to follow a presentation by Carl Backer. Today, we have heard several presentations about the problem of pulmonary insufficiency. We have been shown multiple images of dilated and poorly functioning right ventricles. The obvious conclusion is that these patients should undergo replacement of the pulmonary valve. I agree with Carl that operative morbidity and mortality for replacing the pulmonary valve is minimal, but we must also consider the outcomes in the long-term. Most physicians would agree that valvar replacement is indicated in a symptomatic patient with right heart failure and exercise intolerance. In order to win this debate, therefore, it will necessary to redirect and alter the question! The question I am going to consider is: “should we replace prophylactically the pulmonary valve in asymptomatic patients with pulmonary insufficiency and right ventricular dilation?

Objective: To evaluate the role of the concentration of brain natriuretic peptide in the plasma, and its correlation with haemodynamic right ventricular parameters, in children with overload of the right ventricle due to congenital cardiac disease. Methods: We studied 31 children, with a mean age of 4.8 years, with volume or pressure overload of the right ventricle caused by congenital cardiac disease. Of the patients, 19 had undergone surgical biventricular correction of tetralogy of Fallot, 11 with pulmonary stenosis and 8 with pulmonary atresia, and 12 patients were studied prior to operations, 7 with atrial septal defects and 5 with anomalous pulmonary venous connections. We measured brain natriuretic peptide using Triage®, from Biosite, United States of America. We determined end-diastolic pressures of the right ventricle, and the peak ratio of right to left ventricular pressures, by cardiac catheterization and correlated them with concentrations of brain natriuretic peptide in the plasma. Results: The mean concentrations of brain natriuretic peptide were 87.7, with a range from 5 to 316, picograms per millilitre. Mean end-diastolic pressure in the right ventricle was 5.6, with a range from 2 to 10, millimetres of mercury, and the mean ratio of right to left ventricular pressure was 0.56, with a range from 0.24 to 1.03. There was a positive correlation between the concentrations of brain natriuretic peptide and the ratio of right to left ventricular pressure (r equal to 0.7844, p less than 0.0001) in all patients. These positive correlations remained when the children with tetralogy of Fallot, and those with atrial septal defects or anomalous pulmonary venous connection, were analysed as separate groups. We also found a weak correlation was shown between end-diastolic right ventricular pressure and concentrations of brain natriuretic peptide in the plasma (r equal to 0.5947, p equal to 0.0004). Conclusion: There is a significant correlation between right ventricular haemodynamic parameters and concentrations of brain natriuretic peptide in the plasma of children with right ventricular overload due to different types of congenital cardiac disease. The monitoring of brain natriuretic peptide may provide a non-invasive and safe quantitative follow up of the right ventricular pressure and volume overload in these patients.

Patients with acute rheumatic fever sometimes present with atypical signs and symptoms. In these circumstances, the Jones criterions may not be sufficient to make a clinical diagnosis. We describe here two patients with unusual presentations, highlighting that, both in regions where the disease is endemic, or where it is seen only sporadically, physicians should be more alert and careful in making the diagnosis.

Magnetic resonance imaging is a powerful diagnostic tool, able to give us accurate anatomical and functional insights concerning the structure of the heart, and capable of overcoming some of the drawbacks and limitations of echocardiography and catheterization. The aim of our current review is to highlight the advantages and applications of the technique in the evaluation of patients with functionally univentricular hearts before and after conversion to the Fontan circulation. Most of these patients will have undergone surgery several times in their life, with a certain body size, and with less than optimal echocardiographic windows because of the surgical scars. Moreover, vascular access is sometimes unavailable due to previous catheterizations. Furthermore, resonance imaging is non-invasive, and can be performed without sedation in patients older than 8 years. Since many of the current techniques, some of them still experimental, will be used extensively in future clinical practice, physicians should be aware of the full spectrum of capabilities of resonance imaging. We will highlight all these applications in the sections which follow.

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.

Congenital cardiac disease is recognized as a major predisposing substrate for infective endocarditis. We present three cases of unanticipated intraoperative discovery of vegetations in patients with congenital cardiac malformations without preoperative clinical, laboratory or echocardiographic evidence of infective endocarditis, and their successful treatment. The relevant literature is reviewed, with emphasis of the need to maintain a high index of suspicion for the presence of endocarditis during repair of congenital cardiac lesions.

We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions.

The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. Patients and methods: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. Results: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prologed intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessuful balloon dilation, underwent surgical reoperation. Conclusions: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.

Objectives: To identify predictive factors of complications occurring during paediatric interventional catheterisation. Background: Interventional paediatric catheterisation is still burdened by a substantial risk. Risk factors, however, have rarely been investigated. Methods: We analysed prospectively 1,022 interventional procedures performed over a period of 8 years, excluding 260 procedures for atrial septostomy. We considered several patient-related variables, specifically age, weight, and gender, type of procedure, times required for fluoroscopy and the overall procedure, technical challenge, and the severity of the clinical condition. We also analysed variables linked to the environment, specifically the date of the examination, whether the operator remained in training, the novelty of the material, any breakdown in the installation, and errors made by the operator. We classified complications as those without clinical consequence, those which proved lethal, those requiring cardiopulmonary resuscitation, elective or emergency surgery, hospitalisation in the intensive care unit, and those leading to recatheterisation. Results: Our average incidence of complications was 4.1 per cent, which did not change significantly during the period of study. Of the patients, 4 died, 7 needed urgent surgery, 5 elective surgery, 3 hospitalisation in intensive care unit, and 8 recatheterisation. Independent risk factors for complications were technical challenge, critical clinical condition, operator in training, operator error, and breakdown of the installation. Young age was not associated with a higher risk of complications. Patients in whom no cause for complication could be found, either related to their own features or the environment, had a risk of complication of 1.4 per cent (95 per cent confidence intervals from 0.7 to 2.5 per cent). Conclusions: Our data show that variables relating either to the patient or the environment of catheterisation are associated with an increased risk of procedural complications. Knowledge of the risk factors can improve the odds of paediatric interventional catheterisation.

Objective: Infective endocarditis is uncommon condition, with a high degree of morbidity and mortality. It is less common in children, albeit tending to be associated with congenital cardiac malformations. We describe our experience of the need for surgical treatment in children with infective endocarditis. Patients and methods: We analyzed retrospectively the records of 9 children aged below 16 years seen between May 2003 and March 2005 with infective endocarditis, reviewing the demographic details, clinical presentation, microbiological and echocardiographic data, operative findings, and outcome. Results: Apart from pre-existing renal insufficiency in 1 patient, congenital cardiac malformations were the predisposing factors. Blood cultures were positive in 3, but remained negative in the other 6 patients. The indications for surgical treatment included uncontrolled sepsis, congestive heart failure, recurrent endocarditis, patch or graft dehiscence, and pseudoaneursymal formation. Death due to uncontrolled sepsis resulting in multiorgan failure occurred in 1 patient, who had tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. Another patient died late postoperatively due to cardiac failure after relapse of the endocarditis in the setting of negative blood cultures. Conclusion: Despite advances in antimicrobial therapy, diagnosis, and measures of treatment for infective endocarditis, complications continue to be responsible for substantial morbidity and mortality. Since blood cultures are frequently negative, clinical and echocardiographic findings should be the major determinants of strategies used for treatment. We believe that our small series of patients seen over the past two years in which surgical treatment was performed will be helpful in guiding the clinical perspectives for children with infective endocarditis.

The identification of the important relationship between shape and function of ventricular chambers represents a milestone of modern cardiology. Application of the law of Laplace for an ideal sphere furnishes intuitive insights on the progression of heart failure. A dilated heart, by virtue of its large size, must generate greater stress in the myocardial wall to achieve sufficient pressure so as to eject the required amount of blood. The mural hypertrophy represents a compensatory mechanism, guaranteeing a lower stress. When the ratio between the radius of the chamber and the thickness of its wall increases abnormally, the heart fails.

In the past, most arguments concerning transposition devolved on how best to define it. When Mathew Baillie described the first case, at the end of the eighteenth century, he had no problems. He simply called it a singular malformation!1 Subsequent to Baillie's description, arguments raged amongst morphologists and pathologists as to whether the entity was best described on the basis of how the aorta was related to the pulmonary trunk, or according to the origin of the arterial trunks from inappropriate ventricles. There is little doubt now that all clinicians diagnose transposition on the basis of discordant origin of the arterial trunks from the ventricular mass, although there is still debate as to whether this discordant origin is best described in terms of connections2 or alignments.3 There remain some, nonetheless, who still describe entities such as “double outlet with transposition”, using this phrase to describe the arrangement in which both arterial trunks arise from the right ventricle, but with the aorta positioned anteriorly. For those adopting the concepts of connections or alignments, this combination is clearly impossible.4 Yet for those who choose to define “transposition” on the basis of the anterior location of the aorta,5 this would remain an entirely logical description. These problems of nomenclature, and many more aspects of the morphology of discordant origin of the arterial trunks from the ventricular mass, are discussed at length in the first review of this part of the supplement,6 co-authored by myself, who believes firmly in the concept of connections, and Paul Weinberg, who is equally convinced that the cardiac segments are best described in terms of alignments rather than connections. Our joint review hopefully shows that differences in nomenclature nowadays are relatively minor, and even where they exist, they do not stand in the way of reaching consensus on how best to describe the segmental combination in which the atrial chambers are joined to morphologically appropriate ventricle, but the ventricles support morphologically inappropriate arterial trunks. It is this particular morphological arrangement that, for the purposes of this section of our supplement, is described as “transposition”.

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.

I-cell disease is a rare, hereditary, and fatal disease, which is often complicated with disease of the cardiac valves. We have experienced a case of severe mitral valvar insufficiency in a 9-month-old male infant with I-cell disease. Mitral valvoplasty was difficult, because the leaflets were remarkably hypertrophic, and the tendinous cords were elongated and adherent. Despite these difficulties, surgery proved effective in the treatment of the congestive heart failure, allowing for further treatment of the I-cell disease.

Hypoplastic left heart syndrome is a rare but serious form of congenital cardiac disease, characterized by underdevelopment of the components of the left heart, rendering the left ventricle non-functional. Its aetiology is largely unknown, but there is certainly a genetic component. Prenatal diagnosis nowadays uncovers about half of cases. Postnatal options for treatment include comfort care, 3-stage palliative surgery, or cardiac transplantation. In this review, we discuss the morphology, possible pathogenetic mechanisms, clinical management, and perspectives of prenatal intervention based on work in animal models.

Background: Health-related quality of life in adults with congenital cardiac disease appears to be impaired, but the determinants of this alteration remain largely unknown. The aim of our study was to examine the impact of arrhythmias on quality of life in patients late after the atrial redirection operation for transposition, or after complete correction of tetralogy of Fallot. Patients and methods: We enrolled 32 patients with transposition, and 35 patients with tetralogy of Fallot, in a prospective study. Quality of life was assessed using RAND-36 questionnaires. Quality of life data were compared with those of an age-matched control population. Patients underwent 24-hour and 7-day electrocardiographic recordings, echocardiography, and exercise testing. Results: Patients without arrhythmias had quality of life comparable to that of the general population. All 8 domains in the questionnaire showed scores 22 to 51 percent lower in 12 patients with atrial tachyarrhythmias, and/or sinus nodal dysfunction, requiring insertion of a pacemaker in comparison with 55 patients without arrhythmias. In patients with transposition, low scores were not only associated with arrhythmias, but also correlated with impaired exercise capacity. Conclusions: Atrial tachyarrhythmias, and sinus nodal dysfunction, requiring insertion of a pacemaker, were associated with impaired quality of life in adults late after atrial surgical correction of transposition or correction of tetralogy of Fallot.