Background: To determine the rate of progression of dilation of the aortic root in adults with a bicuspid aortic valve. Methods: We reviewed retrospectively the transthoracic echocardiograms of 50 adults with a bicuspid aortic valve. Each patient had had at least two examinations made 12 months apart. Measurements were taken at four levels: at the basal attachment of the leaflets of the valve within the left ventricular outflow tract, at the widest point of the sinuses of Valsalva, at the sinutubular junction, and in the ascending aorta 1 cm beyond the sinutubular junction. Results: Progressive dilation occurred at all levels, ranging from 0.3 mm/yr at the basal attachment within the left ventricular outflow tract to 1.0 mm/yr, 1 cm beyond sinutubular junction. These rates of dilation were greater than the reported rate of 0.8 mm per decade in the normal population. The rate of dilation found in the ascending aorta 1 cm beyond the sinutubular junction was significantly greater than at the other sites (p = 0.005). The 21 patients with baseline measurements greater than 34 mm had a significantly higher rate of progression (p = 0.007). Sex, age, and the degree of valvar obstruction or regurgitation did not significantly influence the rate of progression of dilation. Conclusion: There is a significantly higher rate of dilation of the aortic root in adults with a bicuspid aortic valve when compared to the normal population. Periodic evaluation of the ascending aorta is essential in these patients, even after replacement of the aortic valve. Other imaging modalities should be considered if the region beyond the sinutubular junction is not well visualized by transthoracic echocardiography.

Objective: To heighten the awareness of pediatricians and pediatric cardiologists to aortic dissection, a potentially dangerous medical condition. Methods: We reviewed the charts of 13 patients, seen in four medical centers, who suffered acute or chronic aortic dissection over the period 1970 through 2000 whilst under the age of 25 years. Results: There were seven male and six female patients, with the mean age at diagnosis being 12.1 years, with a range from one day to 25 years. Congenital cardiac defects were present in five patients, and Marfan syndrome in four. In three of the patients with congenital cardiac defects, aortic dissection developed as a complication of medical procedures. In three patients, dissection followed blunt trauma to the chest. We could not identify any risk factors in one patient. The presenting symptoms included chest pain in four patients, abdominal pain and signs of ischemic bowel in two, non-palpable femoral pulses in one, and obstruction of the superior caval vein in one. Angiography and magnetic resonance imaging were the main diagnostic tools. Overall mortality was 38%. Only six patients had successful surgical outcomes. Conclusion: Due to the rarity of aortic dissection a high index of suspicion is required to reach the diagnosis in a timely manner. It should be considered in young patients complaining of chest pain in association with Marfan syndrome, anomalies of the aortic valve and arch, and chest trauma.

The morphogenesis of most types of human congenital cardiac malformations is still obscure. The reasons for this are, first, the paucity of data from human embryos and fetuses and, second, the paucity of appropriate animal models. During the past few years, we have tested several chemicals for their teratogenic potential, hoping to find, particularly in the chick, substances that could be used for the development of models for specific cardiac malformations. We have now discovered that suramin, an antitrypanosomal drug, can induce a special type of congenital cardiac defect in which the two atrial appendages are positioned to the left of the great arteries. This situation resembles the situation found in humans and classified as left juxtaposition of the atrial appendages. In the present study, we have analysed the pathomorphological features of a series of our chicken hearts to assess precisely how accurately these cardiac malformations in the chick correspond to the situation seen in the human. We found that the cases observed in the chick did, indeed, have many features in common with the human cases described in the literature. This suggests that we have developed an animal model for human left juxtaposition. Our model could be used for two kinds of embryological studies: first, documentation of the morphogenesis of left juxtaposition; and second, studies on the mechanisms driving the normal positional changes between the atriums and outflow tract of the embryonic heart during the late phase of cardiac looping. The fact that left juxtaposition of the atrial appendages can be induced by suramin might help to elucidate the molecular mechanisms underlying this type of congenital cardiac malformation. Furthermore, the fact that suramin is used for the chemotherapy of frequent tropical diseases, such as African trypanosomiasis and onchocerciasis, poses the question as to whether this drug might play a role in the aetiology of left juxtaposition in some human populations.

Objectives: To determine the anatomical characteristics of systemic venous collaterals formed after the Fontan operation, and the efficacy of a transcatheter strategy for management. Methods: We reviewed retrospectively the data from cardiac catherization of 50 persistently cyanotic patients after the Fontan operation. Results: A total of 54 transcatheter interventions were performed, at a mean age of 6.3 ± 3.5 years, a mean interval of 2.7 ± 2.9 years from completion of the Fontan circulation. Of 38 patients who had fenestration of the baffle at the time of surgery, 25 had patency of the fenestration, and 24 had the fenestration occluded with a device at the time of interventional treatment for associated venous collaterals. We identified a total of 68 systemic venous collateral channels, of which 36 (53%) were supracardiac, 12 (18%) cardiac, and 20 (29%) infracardiac in origin. The most common site of origin was the brachiocephalic vein (44%), followed by the left phrenic vein (25%). A longer time from surgery, at 3.3 ± 3.4 years, was associated with the identification of collaterals having a diameter larger than 4 mm (p < 0.01). The mean pulmonary arterial pressure was higher in those with larger compared to those with smaller collaterals (13.3 ± 2.8 versus 11.1 ± 2.0 mmHg, p < 0.01). Coils were used for occlusion of 61 vessels, and a Rashkind™ occluder for the remaining 7. After exclusion of the patients undergoing simultaneous closure of their fenestration, systemic saturation of oxygen increased from 89 ± 6% to 95 ± 3% (p < 0.01). Conclusion: Venous collateral channels are common in patients suffering progressive cyanosis in the setting of the Fontan circulation. The collaterals increase in size with time, and are associated with higher pulmonary arterial pressures. Transcatheter treatment is feasible, and results in resolution of cyanosis. Only continuing follow-up will show whether further collateralization occurs in time.

Background: Cardiac rhabdomyomas associated with tuberous sclerosis induce various abnormalities in the electrocardiogram. Electrocardiographic evidence of ventricular hypertrophy may appear if the tumour is electrically active. To our knowledge, electrocardiographic evidence of ventricular hypertrophy has been reported only in association with congestive heart failure. Follow-up studies of changes in electrocardiographic findings are also lacking. Methods: We studied 21 consecutive patients with cardiac rhabdomyoma associated with tuberous sclerosis, 10 males and 11 females, aged from the date of birth to 9 years at diagnosis. The mean period of follow-up was 53 months. None of the patients developed congestive heart failure. We evaluated the electrocardiographic changes during the follow-up, and their association with echocardiographic findings. Results: Of the 21 patients, 12 showed one or more abnormalities on the electrocardiogram at presentation, with five demonstrating right or left ventricular hypertrophy. In all of these five cases, the tumours were mainly located in the respective ventricular cavity. In one patient with a giant tumour expanding exteriorly, there was marked left ventricular hypertrophy on the electrocardiogram. Followup studies showed spontaneous regression of the tumours in 12 of 19 patients, with abnormalities still present in only 7 patients. A gradual disappearance of left ventricular hypertrophy as seen on the electrocardiogram was noted in the patient with marked left ventricular hypertrophy at presentation in parallel with regression of the tumour. Conclusions: The presence of cardiac rhabdomyomas in patients with tuberous sclerosis might explain the ventricular hypertrophy seen on the electrocardiogram through its electrically active tissue without ventricular pressure overload or ventricular enlargement, although pre-excitation might affect the amplitude of the QRS complex. Even in cases with large tumours, nonetheless, the electric potential might not alter the surface electrocardiogram if the direction of growth of the tumour is towards the ventricular cavity. In many cases, electrocardiographic abnormalities tend to disappear, concomitant with regression of the tumours.

Background: Cardiac remodelling is now recognised as an important aspect of cardiovascular disease progression and is, therefore, emerging as a therapeutic target in cardiac failure due to different etiologies. Little is known about the influence of different therapies for cardiac failure on the remodelling seen in infants with congenital cardiac disease. Methods: During follow-up of a prospective and randomized trial, we investigated therapeutic effects on neurohormonal activation, ventricular function, and myocardial gene expression. We compared the data from 8 infants with severe congestive heart failure due to left-to-right shunts, who received digoxin and diuretics alone, to 9 infants who received additional treatment with propranolol. Results: In these infants, β-adrenergic blockade significantly reduced highly elevated levels of renin, from 284 ± 319 μU/ml compared to 1061 ± 769 μU/ml. Systolic ventricular function was normal in both groups, but diastolic ventricular function was improved in those receiving propranolol, indicated by significantly lower left atrial pressures, lower end-diastolic pressures, and less pronounced ventricular hypertrophy, the latter estimated by lower ratios of myocardial wall to ventricular cavity areas on average of 42%. Further hemodynamic parameters showed no significant differences between the groups, except for the lower heart rate in infants treated with propranolol. In those treated with digoxin and diuretics, there was a significant downregulation of β2-receptor and angiotensin-2 receptor genes, and up-regulation of endothelin A receptor and connective tissue growth factor genes, that were partially prevented by additional treatment with propranolol. Conclusions: β-blockade is a new therapeutic approach for congestive heart failure in infants with congenital cardiac disease, producing with significant benefits on neurohormonal activation, diastolic ventricular function, and cardiac remodelling.

Background: Minimally invasive cardiac surgery is now becoming standard in the correction of simple congenital cardiac malformations. We introduced a clinical pathway for fast track recovery of school activities in children after minimally invasive cardiac surgery, and assessed the function of the pathway in children with atrial or ventricular septal defects, comparing minimally invasive surgery to repair through a conventional full sternotomy. Methods: We studied 15 children of school age who underwent repair of an atrial or ventricular septal defect through a lower midline sternotomy, and 10 children undergoing repair through a full sternotomy. The clinical pathway was for extubation to take place in the operating room, echocardiographic evaluation on the 5th postoperative day, and discharge home on the 7th postoperative day, with return to school within 2 weeks, and resumption of all gymnastic activity within 6 weeks of the minimally invasive surgery. Results: In those having a lower midline sternotomy, postoperative hospital stay was 7.4 ± 0.8 days, with return to school 8.0 ± 2.4 days after discharge. They resumed gymnastics 41 ± 11 days after the minimally invasive surgery. In those having a full sternotomy, in contrast, these parameters were 13.5 ± 2.7, 23.1 ± 8.4, and 95 ± 43 days, respectively. Of the 15 children undergoing a minimally invasive approach, 12 (80%) fulfilled the criterions of our clinical pathway. Conclusions: We conclude that minimally invasive cardiac surgery can safely be performed in children. In addition to its cosmetic role, the technique has added value in promoting early return to normal school life, including gymnastics.

The standard surgical approach for closed heart procedures in small infants and children is to use a posterolateral thoracotomy incision, which results in the division of the latissimus dorsi and serratus anterior muscles. The aim of our study was to determine the frequency and type of musculoskeletal deformities in children undergoing surgery with this approach for congenital cardiac disease.

We included 49 children, 28 boys and 21 girls, in the study. Their mean age was 10.2 ± 4.8 years, the mean age at the time of surgery was 3.8 ± 4.0 years, and they were evaluated at an average of 6 years after the thoracotomy. Of the patients, 94% had various musculoskeletal deformities. Scoliosis was observed in 15 patients (31%) but only in two patients did the curves exceed 25 degrees. Of these patients, three-fifths had aortic coarctation. Elevation of the shoulder was seen in 61%, winged scapula in 77%; while 14% had asymmetry of the thoracic wall due to the atrophy of the serratus anterior muscle. Deformity of the thoracic cage was observed in 18%; and 63% had asymmetry of the nipples.

Thus, we found that musculoskeletal deformities are frequent after thoracotomies in children with congenital cardiac disease. Patients who have undergone such procedures for cardiac or noncardiac surgery should be followed until their skeletal maturation is complete. Techniques sparing the serratus anterior and latissimus dorsi muscles should be preferred. These adverse effects of thoracotomy may be another reason for using interventional procedures in these cases.

Congenital disorders of glycosylation are a group of inherited metabolic multisystem disorders characterized by defects in the glycosylation of proteins and lipids. In most cases, neuromuscular disease is present. The purpose of this study was to characterize the cardiological aspects in this disorder.

From the literature, we identified six children with congenital disorders of glycosylation associated with cardiac disease. We then screened for cardiovascular manifestations 20 patients diagnosed with congenital disorders of glycosylation at our own institution.

Of the 6 patients identified in the literature, 4 had hypertrophic cardiomyopathy, while in the other 2 the cardiac diagnosis was unclear. The mean age at cardiac diagnosis was 5 months, with a range from 34 weeks to 24 months. Of the patients, five had died at a mean age of 3.5 months, with a range from 1.5 to 6 months, with one documented cardiac death. Three of our 20 patients (15%) had coexistent cardiomyopathy, and in three additional patients presenting with cardiomyopathy we made the diagnosis of a congenital disorder of glycosylation. In our cohort, dilated cardiomyopathy was found in two-thirds of the patients, with hypertrophic cardiomyopathy in the other third. The mean age at cardiac diagnosis was 19 months, with a range from 0.5 to 84 months. Of these patients, two died in infancy at a mean age of 4 months, specifically at 1.5 and 7 months, due to cardiac disease, with one dying suddenly. The remaining four patients are alive with minor to severe cardiac dysfunction.

We conclude that congenital disorders of glycosylation have to be considered in the differential diagnosis of children presenting with cardiomyopathy, and that all patients with congenital disorders of glycosylation should be screened for an associated cardiomyopathy. Cardiac involvement contributes significantly to morbidity and mortality, and probably to sudden cardiac death in this disorder.

Aims: We investigated the incidence and outcome of cardiac malformations in 53 patients with Williams syndrome. Methods and results:The mean age, and period of follow-up, were 3.6 and 5.3 years, with standard deviations of 4.0 and 5.6 years, respectively. Of the patients, 45 (85%) had cardiovascular anomalies, often combined. Males presented earlier than females, at the ages of 2.1 years, with standard deviation of 2.8 years, as opposed to 4.5 years, with standard deviation of 4.2 years (p < 0.01). Supravalvar aortic stenosis occurred in 32 patients (71%), pulmonary arterial stenosis in 17 (38%), and mitral valvar prolapse in 12 (27%), 9 of these having regurgitant valves. Pulmonary valvar stenosis, ventricular septal defect, coarctation of the aorta, persistent patency of the arterial duct, hypertrophic cardiomyopathy, and subaortic stenosis all occurred less frequently. In 21 patients (47%), 24 surgical or catheter interventions had been made, most often for repair of supravalvar aortic stenosis, undertaken on 16 occasions with just one recurrence, and in 4 along with surgery to the mitral valve. Other lesions requiring intervention were pulmonary valvar stenosis, pulmonary arterial stenosis, coarctation of the aorta, and subaortic stenosis. We lost 3 patients (7%), with severe supravalvar aortic stenosis and moderate or severe mitral regurgitation, 2 early and one late after surgery. Conclusion: The most frequent cardiovascular anomalies in Williams syndrome were supravalvar aortic stenosis, pulmonary arterial stenosis, and mitral valvar prolapse, which occurred more frequently in our patients than previously observed. Patients with left ventricular pressure and volume overload were at greater risk.

In order prospectively to investigate the frequency and evolution of subclinical valvitis, we selected 40 consecutive patients suffering their initial attack of rheumatic fever, seen in our clinic from 1992 to 1994, and followed-up until 2001, with a mean period of follow-up of 8.1 years, and a standard deviation of 0.6 year. We also assembled a matched control group of 37 healthy children and adolescents. We discovered a murmur of mitral regurgitation in 28 (70.0%) of the patients. In 3 (7.5%) of these patients, there was also a murmur of aortic regurgitation. In the group of 28 symptomatic patients, Doppler echocardiography showed mitral regurgitation in all, and aortic regurgitation in 17. In the group of 12 patients without clinical evidence of cardiac involvement, Doppler echocardiography identified mitral regurgitation in 2, isolated in one and associated with aortic regurgitation in the other. Thus, the frequency of subclinical valvitis was 16.7%. In patients with subclinical valvitis only the aortic regurgitation regressed during the period of follow-up. In the group of 28 symptomatic patients, mitral regurgitation disappeared in 6 (21.4%), aortic regurgitation in 7 of the 17 having this feature (41.2%), while 2 patients (7.1%) developed mitral stenosis. The sensitivity and specificity of cardiac auscultation were, respectively, 93.3%, with 95% confidence intervals between 72.3% and 97.4%, and 100%, with 95% confidence intervals between 65.5% and 100%, for the diagnosis of mitral regurgitation, and 16.7%, with 95% confidence intervals between 4.4% and 42.3%, and 100%, with 95% confidence intervals between 81.5% and 100%, for that of aortic regurgitation. We conclude that the Doppler echocardiogram is an important means of diagnosing and assessing the evolution of subclinical rheumatic valvar lesions, which are not always transient. We suggest that Doppler echocardiography should be performed in all patients with acute rheumatic fever. Subclinical valvitis should be considered as mild carditis, provided that strict criterions are observed in the differential diagnosis from physiological regurgitation, and Doppler echocardiographic findings are analyzed in the context of the other manifestations of the disease.

Cardiopulmonary bypass induces a generalized inflammatory response, with fever and leukocytes, which is difficult to differentiate from an infection. Recently, procalcitonin has been proposed as an early and specific marker of bacterial infection. The influence of cardiopulmonary bypass on production of procalcitonin, therefore, must be assessed before considering this molecule as a valuable marker of infection after cardiac surgery in children. With this in mind, we measured levels of procalcitonin, interleukin 6, and C-reactive protein before and 6 h, 1, 3 and 5 days after cardiopulmonary bypass, in 25 children undergoing cardiac surgery. Cardiopulmonary-bypass induced a transient increase in procalcitonin, with a peak at 24 h, with a median of 1.13 μg/l, a 25th and 75th interquartile of 0.68–2.25, and a p value of less than 0.001. The value had returned to normal in the majority of the children by the third day after surgery. Peak values correlated with the duration of cardiopulmonary-bypass, with a r-value of 0.58 and a p value of 0.003; cross-clamp time, with a r-value of 0.62 and a p value of 0.001; days of mechanical ventilation, with a r-value of 0.62 and a p value of 0.001; and days of stay in intensive care, with a r-value of 0.68, and a p value of 0.0003. The value returned to normal after 3 days in 83% of the patients. Levels of interleukin 6 and C-reactive protein also increased significantly after surgery, and remained elevated for up to 5 days.

Thus, in contrast to other markers, levels of procalcitonin in the serum are only slightly and transiently influenced by cardiopulmonary bypass, and may prove to be useful in the early recognition of an infection subsequent to cardiopulmonary bypass.

Background: In recent years, the diagnosis of infective endocarditis has been enhanced by the use of echocardiography. We sought, therefore, to review its effect on the management of endocarditis in children. Methods: We reviewed all the patients presenting to our institution for evaluation for infective endocarditis from May 1994 to January 2002. The patients were stratified according to whether or not they had congenitally malformed hearts. Results: Of the 90 referred patients identified, 46 (51%) had positive ultrasonic findings. Of these, we excluded 26 patients because of the presence of indwelling lines. The remaining 20 patients with features of endocarditis had a median age of 6.5 years, and a range from 0.14 to 8.5 years. There were 4 patients with normal hearts, and 16 with congenital cardiac malformations. We identified rupture of a sinus of Valsalva in four patients, with rupture into the left ventricle in two, and into the right ventricle and right atrium in one each. The mitral valve was involved in six patients, the aortic valve in another six, including all four with rupture of the sinus of Valsalva, both mitral and aortic valves in three, the pulmonary trunk in three patients, and the tricuspid valve and a Blalock-Taussig shunt in one patient each. Organisms isolated included Streptococcus mitis in 4 patients, Streptococcus pneumoniae in 2 patients, Streptococcus sanguis in 1, Staphylococcus aureus in 3, Staphylococcus epidermidis in 1, and Enteroccocus in 2. Cultures proved negative in 7 patients. Surgical intervention was needed in 12 patients, and one died (5%). Only the left-sided chambers were involved in those with normal hearts. Both patients infected with Streptococcus pneumoniae had rupture of a sinus of Valsalva. Conclusion: Involvement of the left-sided chambers is more likely in structurally normal hearts, and in cases with rupture of a sinus of Valsalva, in which case infection with Streptococcus pneumonia should be suspected.

A common brachiocephalic trunk is an anatomic variant in which both common carotid arteries and the right subclavian artery arise from the aortic arch via a single trunk. The impact of this condition on associated congenital cardiac malformations is presently unknown. Out of a total of 1480 cardiac catheterizations performed in children over a period of 10 years, we discovered 48 patients (3.2%) to have a common brachiocephalic trunk, of whom 98% had associated congenital cardiac malformations. A spectrum of associated lesions was identified, including left-to-right shunts in 19 patients, right-sided anomalies in 18 patients, left-sided obstructive lesions in 12 patients, and coronary arterial abnormalities in 10 patients, eight of whom had other cardiac defects. Genetic syndromes were present in one-fifth of the cases. When found with left-sided malformations, the common trunk was associated with persistent hypoplasia of the aortic arch, likely related to diminished flow through the arch during development. In each of four patients in whom the brachiocephalic trunk had been used during construction of a palliative shunt, we observed inadequate growth and deformation of the pulmonary arteries. Thus, angiographic identification of a common brachiocephalic trunk may be a marker for the presence of accompanying congenital cardiac defects and coronary arterial abnormalities. Understanding the pathophysiologic effects of the common trunk is important when planning the palliative or corrective procedures, and when assessing the potential benefit of the surgical repair over the long term.

Objective: To evaluate the feasibility of the arterial switch for surgical repair of transposition, defined as the combination of concordant atrioventricular and discordant ventriculo-arterial connections, after late referral. Methods: From March 2000 to August 2001, six children underwent an arterial switch procedure following left ventricular preparation because of late referral. The mean age at referral was 8.3 months, with a range from 3 to 25 months, and mean body weight was 5.3 kg, with a range from 3.7 to 9.3 kg. The mean saturation of oxygen was 57%, with a range from 50 to 72%. Associated defects included a restrictive ventricular septal defect in three patients, aortic coarctation in one, and partially anomalous pulmonary venous connection in one. The mean interval between referral and the arterial switch procedure was 3.7 months, within a range from 1 to 7 months. A mean of 1.5 surgical procedures were undertaken to prepare the left ventricle, the most being 3 procedures, including combinations of creation of an inter-atrial communication in four patients, banding of the pulmonary trunk in five, and creation of a systemic-to-pulmonary arterial shunt in three. We evaluated left ventricle ejection and shortening fractions, left ventricular diastolic diameter and volume, right and left ventricular wall thicknesses, and the ratio of right to left ventricular values by echocardiography at referral, immediately before, and one week after the arterial switch procedure. Results: All children are alive and well, with a mean follow-up of 17 months, ranging from 9 to 26 months. Echocardiography showed a statistically significant decrease of the ratio between right and left ventricular wall thicknesses, from 1.33 ± 0.26 at referral to 0.79 ± 0.08 before the switch procedure (p < 0.005). Left ventricular function was adequate after arterial switch, with a mean ejection fraction of 79.3%, ranging from 66 to 87%, and a mean shortening fraction of 41.7%, ranging from 30 to 49%. Conclusions: Despite late referral, and initially inadequate left ventricular volume and mural thickness, children with transposition can successfully be treated with the arterial switch procedure, provided that the left ventricle is adequately prepared, using echocardiography to monitor left ventricular morphology and function.

Objectives: This study was designed to identify predictors of residual defects following deployment of the Amplatzer device to close septal defects within the oval fossa. Methods: Between February 1997 and February 2000, we used the Amplatzer device to close defects in the oval fossa in 89 patients. Of these patients, 18 (20%) had residual defects. At 6 or 12 months following placement of the device, 13 defects (14.6%) had persisted. We evaluated several variables derived from clinical features, transesophageal echocardiography and catheterization to establish predictors for residual shunting. Results: Multivariate analysis identified a shorter superior rim of less than 8 mm (Odds ratio = 10.1; 95% confidence intervals = 2.64–38.72; p = 0.001), and a smaller interatrial septum in the 30-degree transesophageal echocardiographic plane of less than 30 mm (Odds ratio = 5.5; 95% confidence intervals = 1.17–26.14; p = 0.03) as independent predictors of residual defects. When the analysis was repeated defining only those 13 patients with persisting residual defects at 6 or 12 months as failures, a short superior rim (p = 0.004) remained a predictor for residual shunting. Conclusions: Defects with a short superior rim and smaller interatrial septum in the 30-degree transesophageal echocardiographic plane independently and additively predict an increased probability of residual shunting following closure of defects in the oval fossa using the Amplatzer device.

We evaluated the concentrations of the brain and atrial natriuretic peptides in the plasma as markers of ventricular function and volume load in children with functionally univentricular hearts. We studied 7 children aged from 0.5 to 0.7 years with functionally univentricular hearts who had undergone a first palliative operation, and 10 children aged from 1.8 to 3.7 years who had undergone a bidirectional Glenn anastomosis at ages ranging from 0.4 to 1.0 year. As a control group, we studied 14 children without heart defects aged from 0.1 to 4.5 years. Levels of the brain natriuretic peptide were measured at 8.3 to 122 ng/l, with a mean of 52.8 ng/l, after the first palliative operation, compared to 0 to 16 ng/l, with a mean of 7.3 ng/l, after a bidirectional Glenn anastomosis, and 0 to 13.8 ng/l, with a mean of 5.9 ng/l, in the children serving as controls. Corresponding values for atrial natriuretic peptide were 17 to 203 ng/l, with a mean of 103 ng/l, after the first palliative operation, compared to 16 to 54 ng/l, with a mean of 29 ng/l, after the bidirectional Glenn anastomosis, and 12 to 52 ng/l, with a mean of 32 ng/l in the controls. Echocardiography showed that all the children with functionally univentricular hearts had normal ventricular function. Blood presssure, pulmonary arterial pressure, and arterial saturations of oxygen did not differ between the groups. We conclude, that in children with functionally univentricular hearts, the volume overload imposed on the heart after the first palliative operation is associated with increased production of brain and atrial natriuretic peptides, while after ventricular unloading, levels of the natriuretic peptides return to control values.

Objective: To examine the degree to which children, considered to be at risk from infective endocarditis, had received professional education and preventive procedures in regard to dental health, and to evaluate the knowledge of their parents of the link between oral health and infective endocarditis. Materials and methods: Questionnaires were distributed to the families of 38 children under the care of paediatric cardiology. A short dental examination was carried out. Parents were asked if they knew why oral health was of particular importance in their child. Results: Of the children, 58% demonstrated evidence of previous or current dental disease, with 24% having had at least one filling, 13% with one or more teeth showing deficiency of enamel, and 39% with untreated dental caries. Only 79% of the children were registered with a dentist. According to Chi squared test, there was no difference in the dental health of registered and non registered children. Of the study group, 29% had received instruction in oral hygiene, 42% had received dietary advice, 13% had received advice regarding fluoride supplementation or had had fluoride professionally applied, and 8% had had fissure sealants. These percentages remained relatively low even if only registered children, or only registered children with previous or current dental disease, were considered. Only 64% of parents were aware of the link between the oral health of their children and infective endocarditis. Parents of children who were registered were more likely to be aware of this link than parents of children who were not registered. Conclusions: In spite of being registered with general dental practitioners, few children with congenital heart disease had received basic education in dental hygiene. Even children known to have had dental disease and, therefore, considered to be more vulnerable, were overlooked.

Background: Outcomes and costs of inpatient versus outpatient pediatric cardiac catheterization have not been extensively evaluated. Methods: For a cost-consequence analysis, we reviewed the medical records and cost data in a Swiss pediatric hospital. We compared outcomes and costs of observed inpatient management versus hypothetical planned same-day discharge for patients meeting the outpatient catheterization criterions for an American pediatric hospital. Results: Among 346 catheterization admissions occurring from January, 1998 through December, 1999, 179 met the American criterions for outpatient catheterization. Complications observed, and/or nursing interventions begun within 5 hours of catheterization, might have required overnight observation in 41 of the 179 admissions (22.9%). The remaining 138 patients were stable at five hours, and presumably could have been discharged the day of the procedure. Routine pre-discharge imaging detected significant complications following three interventional procedures. Postulated costs from the perspective of the provider, counting hospital and physician expenditure were calculated for the Swiss franc in 2000 at a rate of 1.69 francs for each American dollar, averaged 10,946 francs per inpatient, versus 9790 francs for outpatient treatment (p < 0.001 by paired t-test). Estimated revenue deficits, calculated as costs minus reimbursement, averaged 8565 francs per inpatient versus 1756 francs per patient treated as an outpatient. Conclusions: Half the patients being catheterized in the Swiss hospital met the external criterions for attempted outpatient catheterization. Most might have been safely discharged on the same day, with modest savings in costs, and reduced deficits in terms of revenue. Routine pre-discharge imaging may be more important than overnight observation. Outpatient catheterization merits prospective evaluation in Switzerland.

Background: We have used intravascular ultrasound in an attempt to clarify the extent of vascular remodelling of the aortic arch after the repair of aortic coarctation by use of a subclavian flap. Methods: We investigated 13 patients with coarctation of the aorta, ranging in age from 1.4 to 43.0 months, with a mean of 20.8 months, who underwent aortoplasty by incorporation of a subclavian flap. The mean postoperative period was 19.6 months, with a range from 0.03 to 41.2 months. The luminal morphology of the aortic arch was evaluated by intravascular ultrasound at the time of post-operative catheterization. Results: We observed 3 cases longitudinally. Over the period of observation, we found three types of morphology of the aorta at the site of incorporation of the subclavian flap, namely a snowman shape with two inflection points, a pisiform shape with one inflection point, and a round shape without any points of inflection. There was a correlation between the cross-sectional shapes at the site of the subclavian flap in the postoperative period (p < 0.01). In each case, we measured the cross-sectional area at the site of subclavian flap, at the descending aorta, and at the distal aortic arch. The cross-sectional area, and the increment of the cross-sectional area at the site of subclavian flap, was larger. Conclusion: The shape of the lumen subsequent to repair of aortic coarctation changes progressively from a snowman, to a pisiform, and finally to a round shape. Greater growth of the subclavian flap compared to the native wall of the aorta was observed for at least the first 4 years after repair. This finding may improve our understanding of the remodelling process of the arterial trunks after surgical repair.