In a retrospective study, we analysed the data from 101 adults with echocardiographic follow-up after surgical repair of defects within the oval fossa at a mean age of 35 ± 17 years; 56% of the cohort being above the age of 30 years. Mean age at follow-up was 44 ± 18 years, and length of follow-up was up to 40 years (11 ± 12 years). At follow-up, atrial fibrillation or flutter was present in one quarter. Dilation of the right atrium, found in 64%, of the left atrium, found in 44%, and of the right ventricle, found in 29%, were also frequent, as well as pulmonary arterial hypertension, which was found in 30%. Diminished right ventricular ejection fraction, in contrast, was very rare, found only in 1%, and abnormal left ventricular ejection fraction was not encountered. By multivariate analysis, predictors for right or left atrial, or right ventricular, dilation were age at follow-up, degree of tricuspid regurgitation, pulmonary hypertension, and/or atrial fibrillation. In a subset of 21 patients in sinus rhythm, we correlated prospectively the diastolic and systolic function of both ventricles with levels of brain natriuretic peptide, comparing values to those of 20 age-matched controls with a mean age of 46 ± 14 years. Levels of brain natriuretic peptide were significantly higher in patients than in controls (p = 0.006), and correlated significantly with diastolic dysfunction (p = 0.007) and left atrial size (p < 0.0001). In the long-term follow-up after surgical repair of defect within the oval fossa, therefore, complete normalization of heart size and function is rare. Despite preserved systolic function, persistent diastolic dysfunction is common and is associated with elevated levels of brain natriuretic peptide, which may explain the late occurrence of atrial arrhythmias.

Objective: Cardiac catheterization is an important and frequent diagnostic intervention in children, but few systematic studies have explored the associated venous thrombotic complications. We have prospectively evaluated the prevalence of venous thrombosis, diagnosed by ultrasonography, in children catheterized at our department. Materials and methods: We examined 50 children with weight greater than seven kilograms for thrombosis of the femoral vessels. Prophylactic anticoagulation was given to half of the children who were to undergo left-sided catheterization. The enrolled children had ultrasonography of the site of puncture the day before, and the day after, the cardiac catheterization. During catheterization, blood samples were taken from 33 of the children for analysis of thrombophilic agents. Results: The children, with a median age of 34 months, had been catheterized 103 times, comprising the 50 current and 53 previous procedures. Over the course of the current procedure, interventions of various types were performed in 18 children (36%). We diagnosed thrombophilic predispositions in four patients. Despite the thrombophilic tendencies, and a high frequency of interventions, we did not encounter a single case of femoral venous thrombosis. Nor did we find any evidence of arterial thrombosis. Conclusion: Our study indicates that catheterization procedures in children are currently associated with a low prevalence of femoral venous thrombosis. Continuous assessment of vascular complications, nonetheless, is still required to prevent late effects, and to ensure the best quality of cardiac catheterisations in children.

Despite pathologic evidence of myocardial inflammation, the significance of myocarditis in children with acute rheumatic carditis remains controversial. Elevations in cardiac troponin I have been demonstrated in other forms of myocarditis. The purpose of our study was to determine if levels of cardiac troponin I are elevated, suggesting myocardial injury, in patients with acute rheumatic carditis. We identified all those patients with acute rheumatic fever, presenting between July 1998 and December 2000, who had clinical evidence of carditis, such as a new murmur of mitral or aortic regurgitation, and who had an echocardiogram, measurements of levels of cardiac troponin I, erythrocyte sedimentation rate, and/or C-reactive protein performed at the time of presentation. Their charts were reviewed for demographic and clinical data. Echocardiograms were reviewed for severity of aortic and mitral regurgitation, and measurements made of left ventricular ejection fraction, fractional shortening, and end-diastolic dimension. We found 16 patients with acute rheumatic carditis, ranging in age from 2.0 to 16.1 years, with just over one-third having symptoms of congestive heart failure. All patients had evidence of acute inflammation. There was a significant relationship between symptoms and severity of mitral regurgitation. No patient had elevated levels of cardiac troponin I level. The fact that levels of cardiac troponin I are not elevated in the serum of children with acute rheumatic carditis suggests that there is minimal myocytic necrosis in this setting. This supports the concept that acute valvar regurgitation is the major hemodynamic abnormality in these patients.

Aims: To assess the influence of age at a cardiac procedure of children, who underwent elective cardiac surgery or interventional cardiac catheterisation for treatment of congenital cardiac defects between 3 months and 7 years of age, on the longitudinal development of psychological distress and styles of coping of their parents. Methods: We used the General Health Questionnaire to measure psychological distress, and the Utrecht Coping List to measure styles of coping. Parents completed questionnaires on average respectively 5 weeks prior to, and 18.7 months after, cardiac surgery or catheter intervention for their child. Results: Apart from one exception, no significant influence was found of the age at which children underwent elective cardiac surgery or catheter intervention on the pre- to postprocedural course of psychological distress and the styles of coping of their parents. Across time, parents of children undergoing surgery reported, on average, significantly higher levels of psychological distress than parents of children who underwent catheter intervention. After the procedure, parents of children who underwent either procedure reported significantly lower levels of psychological distress, and showed a weaker tendency to use several styles of coping, than did their reference groups. Conclusion: Age of the children at the time of elective cardiac surgery or catheter intervention did not influence the course of psychological distress of their parents, nor the styles of coping used by the parents. Future research should investigate in what way the age at which these cardiac procedures are performed influences the emotional and cognitive development of the children.

Background: Adverse influences arising in fetal life or immediately after birth have a permanent effect on body structure, physiology and metabolism. Evidence is now accumulating that programming of bone growth might be an important contributor to the later risk of osteoporosis. Long-term morbidity and mortality associated with tetralogy of Fallot is not completely known. The aim of the present study was to evaluate the state of the bones in adolescents after surgical repair of tetralogy of Fallot, so as to ascertain any possible repercussions of the disease on bone mineralization. Material and methods: We studied 34 adolescents with repaired tetralogy of Fallot, between the ages of 11 and 18 years, to establish their nutritional status, in terms of height, weight, and skinfolds, their body composition using an anthropometric method, their sexual maturity according to Tanner, and their food-habits as based on 24-hour recall. Bone density was evaluated by lumbar dual-energy X-ray absorptiometry. We included 34 healthy eutrophic adolescents, matched for gender and age, as controls. Results: No significant differences were observed between the patients and their controls concerning nutritional status, body composition, total energy intake and nutritional supply in macronutrients, calcium, phosphorus, magnesium and vitamin D. Bone mineral density, expressed in Z-score and g/cm2, was significantly higher in patients with tetralogy of Fallot (p < 0.01). The age at the time of the first surgical procedure, or at complete surgical repair, and the total number of surgical procedures, had no significant influence on nutritional status or bone mineralization. Gender, chronological age, sexual maturity and the index of body mass are the major determinants of bone density for both samples. Obese adolescents with repaired tetralogy of Fallot had a significantly higher bone density (p < 0.05) compared to undernourished or euthrophic patients. Conclusions: Being born with tetralogy of Fallot has no significant repercussion, by the stage of adolescence, on nutritional status, pubertal progression, and accretion of bone minerals subsequent to surgical repair. Nutritional status is the major influence on the accretion of bone mass.

Adequate nutrition is crucial to the management of children and infants with cardiac disease. Difficulties with feeding are extremely common, and maintaining an adequate caloric intake, in order to achieve sustained growth, is often not possible without nutritional support. We retrospectively reviewed our experience between 1995 and 1999 in treating 37 children with cardiac disease who underwent percutaneous endoscopic construction of a gastrostomy to augment nutritional needs. We stratified the patients into those with cyanotic heart disease, when saturations of oxygen were less than 95%; those with non-cyanotic heart disease with saturations greater than 95%, and those with minor cardiac disease associated with a systemic disorder. Each group was compared to control children matched for age, sex, and diagnosis. We evaluated the variation in standard deviation score for body weight over a median period of follow-up of 295 days. Improvements in the standard deviation score for body weight occurred in each of the groups, whereas children in the control groups demonstrated a decrease in standard deviation score for body weight. The median change of the score for body weight was significantly higher in patients managed with gastrostomy compared to controls. We conclude that supplementation using a gastrostomy tube allows the safe delivery of the caloric intake needed to support malnourished children with cardiac disease.

Discordant atrioventricular connections associated with Wolff-Parkinson-White syndrome increase the challenge of radiofrequency ablation. We report the results and techniques of radiofrequency ablation in three patients with discordant atrioventricular connections, including one patient having double outlet right ventricle with atrioventricular reentry tachycardias. There were two males and one female, aged 14 and 22 years old. We found four accessory pathways during our electrophysiological studies, with two of them manifest on the electrocardiogram, corresponding to left paraseptal and right midseptal regions. The electrophysiological study confirmed this localization, and showed two concealed accessory pathways in the right and left paraseptal regions. Radiofrequency ablation was successful in all cases without recurrence at a mean follow-up of 18.6 months. No complications were observed during the procedures. We conclude that radiofrequency ablation is feasible and effective in the ablation of accessory pathways in patients with discordant atrioventricular connections.

Objective: To determine the quality of life in individuals with corrected tetralogy of Fallot. Methods and subjects: Questionnaires concerning quality of life were sent to all 87 surviving patients aged between 16 and 40 years who had undergone intracardiac repair of tetralogy of Fallot and follow-up in the Wessex Cardiothoracic Unit, and to 87 age and sex matched controls, with medically treated haemodynamically insignificant ventricular septal defects. Results: The only significant difference found between the cases and controls was in requirements for schooling, where those with tetralogy of Fallot were more likely to require additional educational help at school (p = 0.044). For all other aspects of quality of life examined by the questionnaire, including social and genetic history, exercise ability, and health related quality of life, no significant differences were found. Different operative techniques, such as transjunctional patching, right ventriculotomy, and previous palliative shunting, did not affect the quality of life of our population with Tetralogy of Fallot, on average twenty years after their surgery, although the range of operative techniques was limited. Neither age at surgery, nor time since surgery, was correlated with measurements of quality of life. Conclusions: Those who have undergone surgical correction of tetralogy of Fallot have a normal quality of life, with few differences compared to controls.

Background: We investigated the aerobic capacity of 168 adult patients who had undergone successful surgical repair of tetralogy of Fallot at the University of Toronto Congenital Cardiac Centre for Adults. Methods: We compared values of peak uptake of oxygen, peak heart rate, forced vital capacity, and forced expiratory volume in 1 second to predicted values for their age groups. Results: The patients who had undergone surgical repair of tetralogy of Fallot demonstrated an overall diminished peak uptake of oxygen, at 51%, and peak heart rate, at 79%, compared to predicted values. No difference in peak aerobic capacity was found according to the initial surgical strategy of palliation or repair. Conclusions: Adult patients who have undergone surgical repair of tetralogy of Fallot have lower peak uptake of oxygen, and peak heart rate, compared to predicted values. The reduction in the peak heart rate may affect their exercise capacity. The peak uptake of oxygen also decreased with increasing age at the time of testing, and the age at surgical repair.

We describe a case of fetal pulmonary hypertension and tricuspid regurgitation due to non pharmacologically induced ductal constriction observed at 36 weeks' gestational age. The hypertension resolved spontaneously soon after birth, with no functional consequences. Right bundle branch block is the only permanent anomaly, still being seen on the electrocardiogram at the age of 34 months.

Cardiac fibromas are rare benign tumours of connective tissue that occur most frequently in children within the left ventricle. Spontaneous regression has not been observed, and surgical intervention is usually required. We have successfully treated a 1-year old girl with a giant fibroma of the right ventricle using the principles of the Batista procedure. The diagnosis was primarily made using transthoracic echocardiography.

Innocent murmurs in childhood are common, and often they do not reflect cardiac disease. We have performed a prospective review, by means of a questionnaire, to clarify the parental perception of the innocent murmur identified in their child. Whenever possible, depending on age, we also sought the input of the child.

Of a cohort of 63 infants and children with innocent murmurs, a response was obtained from 40 parents and 8 children to an initial questionnaire, with 26 parents replying to a second questionnaire a year later. No parent reported any symptoms in their children, or had imposed any restrictions. Of the parents, however three sets considered regular review was necessary. We conclude that the diagnosis of an innocent murmur by a paediatric cardiologist, aided by investigations including cross-sectional echocardiography, and an opportunity for explanation and reassurance, led to the child being treated normally.

We successfully performed percutaneous transluminal angioplasty to treat severe renovascular hypertension with left ventricular failure in a 5-month-old infant. Using the transcarotid approach, we dilated the stenotic left renal artery without any difficulties, using progressively larger balloons designed for dilation of coronary arteries.

The prevention of unexpected cardiac events in the young remains elusive. Among them, some are lethal. Early identification of life-threatening arrhythmogenic disorders which often manifest in infancy, childhood or even later, may allow initiation of effective preventive therapies. A large prospective study has indicated that some infants with prolongation of the QT interval in the first week of life died suddenly, and would have previously been labelled as victims of the sudden infant death syndrome. Furthermore, in infants with this diagnosis, post-mortem molecular screening has revealed the presence of the long QT syndrome. As an evolution of this background, some European countries have begun to consider the possibility of introducing in their National Health Services the performance of an electrocardiogram during the first month of life in all newborns, as part of a programme for cardiovascular screening. Most adult cardiologists, however, have no or minimal experience with electrocardiograms recorded in infants. Accordingly, the European Society of Cardiology has instituted a Task Force with the objective of creating guidelines for the interpretation of the neonatal electrocardiogram, focusing on the most clinically relevant abnormalities and on the ensuing options for management and referral. The main objective of the present document is to provide adult cardiologists with a practical approach to neonatal electrocardiography, and paediatricians and neonatologists with a tool that should facilitate medical interaction on cardiologic issues. There are important differences between neonatal and adult electrocardiograms. When a cardiologist examines the electrocardiogram of an apparently normal and healthy infant, the focus has to be on distinguishing between patterns that should cause no alarm, and those that require action or additional investigations. To provide clues for this distinction has been the main objective of the members of this Task Force. Whenever possible or appropriate, we have also suggested steps in management.