Objective: To document the echocardiographic features of tetralogy of Fallot during fetal and postnatal life. Correlation of echocardiographic findings with the requirement for early intervention prior to definitive repair. Design: Retrospective observational study. Setting: A tertiary fetal cardiology unit. Patients: Fetuses with a diagnosis of tetralogy of Fallot identified from a prospective database between 1 January 1999 and 31 October 2002. Main measures of outcome: Growth of aorta and pulmonary trunk during fetal and postnatal life. Doppler assessment of the great arteries both prenatally and postnatally. Clinical outcome to definitive repair. Results: We identified 25 fetuses with tetralogy of Fallot, 23 having a pulmonary valvar diameter below the normal range at some point during gestation. The ratio of the diameter of the aortic to the pulmonary valve was abnormal in all cases. The pulmonary arterial Doppler velocity was within the normal range in six fetuses at presentation, and elevated in the remainder. In two fetuses, the right ventricular outflow tract was patent during fetal life, but had become atretic at birth. Both of these fetuses had reversal of flow in the arterial duct at presentation during fetal life. In 2 fetuses in whom we showed poor growth of the pulmonary trunk in late gestation, it was necessary to intervene early. The Doppler velocity across the pulmonary valve during fetal life did not differentiate between babies who required early intervention and those who were repaired electively. There was a marked increase in pulmonary arterial Doppler velocity following birth, which became more elevated with age. Of 18 liveborn infants, 17 have survived, with 2 having balloon dilation of the right ventricular outflow tract, and 3 insertion of a Blalock-Taussig shunt prior to definitive repair. Conclusions: In tetralogy of Fallot, features of pulmonary valvar hypoplasia and obstruction are evident during fetal life. Progression of obstruction in the right ventricular outflow tract was observed during fetal life as well as postnatally. Reversal of flow in the arterial duct, and failure of growth of the pulmonary trunk, predicted the need for early surgery to maintain pulmonary blood flow. Parents should be counselled about the possibility of emergency intervention being required after birth. Affected fetuses should be delivered at units with experience of managing the cyanosed newborn.

Our objective was to assess the efficacy of pharmacological treatment in reducing the incidence of permanent junctional reciprocating tachycardia in young children, or to bring the mean heart rate over 24 h to a normal level.

We included 21 children with a median age of 0.05 year seen with permanent junctional reciprocating tachycardia over the period 1990 through 2001. Of these children, two had abnormal left ventricular function. Follow-up visits were made at least every 6 months. We registered the presence of the tachycardia over 24 h, the mean heart rate over 24 h, and cardiac function. Treatment was started with propafenone alone, or in combination with digoxin as the first choice. Treatment was effective in 14 cases (67%), with either complete disappearance of the tachycardia after discontinuation of medication, or continuation in sinus rhythm with medication; partially effective in 4 cases (20%) when the mean heart rate over 24 h on the last Holter recording was less than 1 standard deviation above the normal for age; but was not effective in the remaining 3 cases (14%). In 3 patients treated with propafenone, or 13 given propafenone and digoxin, treatment was effective in 12 (75%), partially effective in 2 (13%), and ineffective in the other 2 (13%).

All 21 children had a normal left ventricular function at the end of follow-up. The median duration of follow-up was 2.4 years. Permanent junctional reciprocating tachycardia had disappeared spontaneously in one-third of the children, 5 being less than 1 year old. Adverse effects, seen in 5 cases, were mild or asymptomatic. No signs of proarrhythmia were registered.

Pharmacological treatment, either with propafenone alone, or in combination with digoxin, is safe and effective in young children with permanent junctional reciprocating tachycardia. The mean heart rate is normalized, and cardiac function is restored and preserved. Radiofrequency ablation may be delayed to a safer age, with the arrhythmia disappearing spontaneously in one-third.

Objectives: Our aim was to review the clinical records from children with large pericardial effusions of inflammatory origin presenting to a tertiary referral centre over the last 21 years, with emphasis on their clinical presentation, management and outcome. Background: The common identifiable causes of pericardial effusion in children include prior cardiac surgery, bacterial pericarditis, malignancy, and connective tissue disorders. In a significant number of children, however, despite extensive investigation, it is not possible to identify a clear aetiology. A viral cause is often considered, though rarely confirmed. The clinical course of such large idiopathic pericardial effusions in children has not been extensively reported. Methods and results: We reviewed retrospectively the records of all patients seen between 1981 and 2001 with large pericardial effusions of inflammatory origin requiring drainage, excluding the effusions related to cardiac surgery or malignancy. We found 31 patients fulfilling our criterions for study. They could be divided into three groups, with 15 patients having no specific identifiable aetiology despite extensive investigation, 12 patients having evidence of bacterial pericarditis, and four with a probable immunologic disorder. Fever was present in only eight patients (53%) in the idiopathic group. All patients in the other groups had fever. Except for fever and the resultant tachycardia, it was not possible to distinguish on clinical grounds, nor on the presence or otherwise of cardiac tamponade, between those with idiopathic aetiology and those with bacterial infection. Of the patients with presumed bacterial pericarditis, five (42%) had both positive blood and pericardial fluid cultures, three (25%) had positive blood cultures, while a further three patients (25%) had only positive pericardial fluid cultures. All patients required drainage of the pericardial effusion, either under echocardiographic guidance or surgically. None of the patients died. The hospital stay was significantly shorter for those with idiopathic as opposed to bacterial pericarditis. Of those with an idiopathic aetiology, six required readmission due to recurrence of the pericardial effusion, with four patients requiring further surgical drainage. No patients required readmission with a bacterial or immunologic aetiology. No patient developed constrictive pericarditis after a median follow-up of 22 months. Conclusion: Patients with large idiopathic pericardial effusion had relatively few constitutional symptoms as compared with their gross echocardiographic findings. Those with bacterial pericarditis had more urgent need for treatment. Patients with pericardial effusion of inflammatory origin, when treated appropriately, had an excellent outcome with no mortality or development of constrictive pericarditis.

Surgical treatment of cardiac defects in infants born with extremely low weight is sometimes required during the neonatal period. Optimal timing of these operations has yet to be clarified. With this in mind, we reviewed our experience of surgical treatment for 29 infants born with extremely low weight between 1994 and 2001. The main surgical procedures were ligation of a patent arterial duct in 26, a Brock procedure in 2, and ligation of an aorto-pulmonary window in 1 infant. The age at operation ranged from 5 to 57 days, with a median of 30 days, and weighed from 506 to 902 g, with a median of 710 g. There were no deaths. For the 2 infants undergoing the Brock procedure, the reduced systemic blood flow also necessitated closure of the arterial duct. For almost all the 26 infants with a patent arterial duct, indomethacin was given as the initial therapy, but the duct had not closed completely. Increased symptomatology just before the operation due to reduced systemic blood flow, such as decreased cerebral blood flow, decreased urine output, and intestinal ischemia, mandated the earlier surgical ligation (r = −0.576, p = 0.004). The youngest infant needed an infusion of catecholamines perioperatively to maintain stable hemodynamic conditions (r = 0.554, p = 0.003). In 4 infants, including the youngest 2, steroids were administered intravenously just after the ligation. Our results suggest that reduced systemic blood flow is the main indication of surgical repair in infants born with extremely low weight. Even for one in whom the supply of pulmonary blood is dependent on the arterial duct, early reconstruction of the pulmonary arterial pathways, using the Brock procedure, followed by ligation of the duct, is required. Acute adrenal insufficiency should not be overlooked just after the surgery, particularly in the youngest patients.

Background and purpose: There is little documented evidence of young people with congenital cardiac disease being consulted as to what help, if any, they really need in relation to their condition. Most research concentrates on the medical aspects of the condition. There are studies of psychological and social functioning, but few have directly sought the opinions of the young people. More recent research has indicated a need for health professionals to develop services to meet both psychosocial and physical needs of young people with congenital cardiac disease. The findings of the recent Kennedy Report support this need. The purpose of this study was to explore the views of young people with a range of congenital cardiac conditions, on what would help them better deal with their condition, and when and how help might be provided. Methods: This was a qualitative study using semi-structured interviews. We interviewed 16 young people in their own home. Interviews were tape-recorded and transcribed. Analysis was conducted using the “Framework” method. Results: Whilst most of the young people interviewed actively manage their condition, they think more support from others would be beneficial. Issues of activity and communication were cited most often as areas requiring more understanding from people they interact with, for example teachers and peers. The discussion focuses on how health professionals might change or develop their practice to help young people better cope with their condition. Conclusions: Most of the young people interviewed in this study had developed their own strategies for coping with their condition. Although this is a small study, the young people provided important suggestions as to how health professionals could better develop current services.

We carried out a retrospective case control analysis to evaluate the outcome, and the need for treatment, of problems with atrioventricular conduction occurring during an acute attack of rheumatic fever, assessing the occurrence of second and third atrioventricular block versus first degree block.

We reviewed and analysed the clinical, electrocardiographic and echocardiographic records of all children diagnosed in a single institute as having acute rheumatic fever during a period of seven consecutive years.

During the period from October, 1994, through October, 2001, 65 children meeting the modified Jones criterions for acute rheumatic fever were hospitalized in the Soroka University Medical Center, Israel. First-degree atrioventricular block was identified in 72.3% of the children, and resolved with no specific treatment other than non-steroidal anti-inflammatory medications. Second-degree atrioventricular block of Mobitz type I, was observed in one child (1.5%), which progressed from first-degree block, and subsequently resolved. Complete atrioventricuar block was found in 3 children (4.6%), one progressing from Mobitz type I second-degree block, and two being seen as the first presentation. Of the three children with complete atrioventricular block, one patient was not treated, the second was treated with aspirin, and the final one with combined aspirin and steroids. The disturbances of conduction resolved in all three.

We conclude that advanced atrioventricular block is rare during acute rheumatic fever. If occurring, block appears to be temporary, and resolves with conventional anti-inflammatory treatment. Specific treatment, such as insertion of a temporary pacemaker, should be considered only when syncope or clinical symptoms persist.

Background and purpose: Although much previous research has focused on the medical aspects of congenital cardiac disease, there is a growing body of research which suggests that families may need help and support with the wider issues associated with the medical condition. We have previously ascertained from young people with congenital cardiac disease their views on this subject. The purpose of this study was to obtain the views of their parents about the need for, and shape of, services for rehabilitation. Methods: This was a qualitative study using semi-structured interviews. We interviewed 17 parents in their own home. Interviews were tape-recorded and transcribed. Results: Parents would welcome more help and support from health professionals to enable them to manage more effectively the condition with their children. Particular areas of concern relate to the information they receive about the condition; communication between themselves and health professionals; establishing safe levels of activity; and managing the condition at school. Conclusions: This is a small study of the experiences and views of parents, which provides some important information on ways in which health professionals could address the current lack of services for rehabilitation.

A transient form of hypertrophic cardiomyopathy has been previously described in infants of diabetic mothers. When it occurs, it is generally benign. The purpose of our study was to establish the extent of injury to the cardiomyocytes in symptomatic and asymptomatic patients with and without hypertrophic cardiomyopathy.

Thus, we compared 35 consecutive patients to 20 healthy controls, establishing the significance, if any, of differences in cardiac troponin-I and creatine kinase, including its MB-fraction, and seeking to establish the value of these parameters in the diagnosis of cardiac injury. We also determined to levels of glucose and insulin in the serum, and took note of electrocardiographic and echocardiographic investigations. Values were determined at the 1st and 7th days after admission in the patients, while parameters were measured in the control group only on the first day.

We found that the levels of cardiac troponin-I in the serum, known to be a marker for cardiac injury, were significantly elevated in symptomatic patients with life-threatening respiratory or haemodynamic distress. We speculate that transient ventricular hypertrophy is neither the cause nor the consequence of damage to the cardiomyocytes. It would be interesting, nonetheless, to determine the relationship, if any, between cardiomyocytic damage and clinical outcome.

Background: We reviewed our 12-year experience with staged reconstruction for hypoplasia of the left heart, examining the results of each surgical step and the impact of the year of the Norwood operation on survival. We compared survival of patients with hypoplasia of the left heart subsequent to completion of the Fontan circulation to survival of patients with a dominant left ventricle undergoing a Fontan procedure. Patients: Between 1989 and 2001, we performed a first stage procedure in 89 patients. Their median age was 9 days, with a range from 2 to 140 days, and the median weight was 3.4 kg, with a range from 2.4 to 5.4 kg. Results: Survival at 1, 4, and 10 years was 55%, 49%, and 49%, respectively. We experienced 23 early deaths (26%), and 12 deaths between the stages of the Norwood cascade. Of our patients, 42 underwent the second stage, and 30 the third stage. Prior to the first stage, symptoms of necrotising enterocolitis, and of obstructed pulmonary venous return, influenced survival significantly. The latter was eliminated as risk factor when surgery was performed within the first week of life. During the later part of our experience, survival at the first stage operation improved significantly, with survival at 3 years increasing from 42% to 75% for the patients at standard-risk (p = 0.017), and from 17% to 42% for those deemed to be at high-risk (p = 0.1). No deaths occurred in 23 patients older than 3 years of age, all of whom had proceeded through the third stage. After completion of the Fontan circulation, the survival of the patients with hypoplasia of the left heart at 4 years was comparable to the survival of patients undergoing the Fontan procedure with a dominant left ventricle (88% versus 90%, p = 0.8). Conclusions: Early and intermediate survival has improved significantly over the period of 12 years. Late death has been uncommon, and none of our patients are listed for cardiac transplantation.

Background: In adults with heart failure, neurohormonal overstimulation is related to the progression of the disease, and influences prognosis. β-blockers, which modulate neurohormonal activation, now play an essential role in the pharmacological management of heart failure in adults, but their use in children is very limited. Patients and Methods: To investigate the effects of carvedilol administration on neurohormonal activation and left ventricular function, carvedilol was added to standard treatment for heart failure in 9 patients with dilated cardiomyopathy due to heart muscle disease. Standard treatment has been in place for at least 1 month. The protocol consisted in a baseline evaluation to assess neurohormonal activation, and echocardiographic evaluation of left ventricular function. This was followed by a final evaluation at 12 months from carvedilol loading. Carvedilol was started at 0.05 mg/kg/day, and increased every two weeks until the target dose of 0.8 mg/kg/day was reached. Results: Carvedilol administration was associated with a significant reduction in plasma norepinephrine (p = 0.00001), dopamine (p = 0.0001), aldosterone (p = 0.00001) and activation of the renin-angiotensin system (p = 0.0006). Similar reductions in vanilmandelic and homovanillic acid were noted. After 12 months, a positive remodeling took place, with significant reductions in end-diastolic (p = 0.004) and end-systolic diameters (p = 0.009), and an increase in left ventricular ejection fraction (p = 0.001). No adverse effects needing reduction or interruption in the dosage were noted in the run-in phase, nor in the period of maintenance. Conclusion: Carvedilol is a safe complement to standard therapy for heart failure in children, allowing a significant reduction of neurohormonal activation with evident benefits on both ventricular function and the clinical condition.

Aims: To determine the accuracy of data relating to pulmonary vascular resistance data in patients with a bidirectional Glenn anastomosis as calculated using predicted versus measured uptake of oxygen. Methods: We studied retrospectively the data from 33 patients with a bidirectional Glenn anastomosis who underwent cardiac catheterisation prior to surgery to complete the Fontan circulation. Their weight ranged from 5.4 to 51.7 kg, and they were aged up to 12 years. Uptake of oxygen was measured using the Deltatrac II metabolic monitor. From the calculated indexed pulmonary vascular resistance, cases were stratified according to the risk of failure of the subsequent Fontan circulation. The six patients with a resistance of greater than 4 Um2 were deemed at high risk, the six with a resistance from 3 to 4 Um2 at moderate risk, and the 21 patients with a resistance less than 3 Um2 at low risk. Uptake of oxygen was also estimated from the predictive formulas of Lindahl, Lundell et al. and LaFarge and Miettinen. The indexed resistance was similarly calculated using these formulas and a comparable stratification of risk made from this data. Results: The predicted values for uptake of oxygen were consistently higher than those measured, leading to an underestimation of indexed resistance, with mean difference between −0.62 and −1.57 Um2. This difference resulted in misclassification of between five and nine of the 12 patients considered at moderate or high risk as being at low-risk. No other haemodynamic data could reliably separate the subjects deemed at low-risk from those considered to be at high-risk. A transpulmonary gradient of greater than 7 mm of mercury was found to be 100 percent specific for elevated indexed resistance, but only 60 percent sensitive. Conclusions: In patients with bidirectional Glenn anastomoses, all formulas based on predictive uptake of oxygen lead to underestimation of the true indexed pulmonary vascular resistance, to an extent that could significantly influence clinical decision-making. The transpulmonary gradient is not a reliable surrogate for indexed pulmonary vascular resistance.

Aim: To report results of transcatheter occlusion of moderate to large patent arterial ducts, having a minimum diameter above 2.5 mm, with the Amplatzer duct occluder, and to compare these with results achieved using Rashkind or Sideris devices and Cook detachable coils. Design and setting: Retrospective study conducted on intention-to-treat data from a tertiary referral centre. Patients: Since 1989, 116 consecutive patients, 77 females and 39 males, underwent percutaneous closure with several devices. We used the Rashkind double umbrella in 23 patients, the Sideris buttoned device in 39 patients, coils in 17 patients, and the Amplatzer duct occluder in 37 patients. The median age of the patients was 37 months, and the median weight 13 kg. The mean minimum diameter of the duct was 3.8 ± 1.22 mm, with a median of 3.5 mm, and range from 2.5 to 10 mm. Results: Implantation succeeded in all but 9 of the children (92%). The time of fluoroscopy was shorter, and full occlusion was better as judged on angiography, in patients closed using the Amplatzer device, despite closure of larger ducts, than in patients closed using other devices (p < 0.0001, p = 0.0003, and p = 0.0015 for the Rashkind, Sideris, and coils, respectively). Complications included embolisation in 2 patients, and haemolysis in 3 patients. In 12 patients, a second device was inserted because of residual shunting noted during follow-up. Complete occlusion was achieved earlier after implantation (p = 0.0002), and the rate of complete occlusion was better in patients receiving an Amplatzer device (97%, p = 0.024) than in patients undergoing closure with other devices. Conclusion: Transcatheter closure of moderate to large patent arterial ducts using the Amplatzer duct occluder is an effective and safe procedure, providing better results than those achieved using other occluders.

We implanted either large or medium Palmaz stents, or a Palmaz Corinthian stent, in various stenotic vessels, such as the pulmonary arteries, pulmonary veins, aorta, or superior caval vein. Using angiograms, we measured the diameter of the stenotic vessel before or after the implantation, the minimal diameter of the lumen, the minimal diameter of the largest fully expanded balloon used to expand the stent, and the diameter immediately after withdrawal of the balloon.

The minimal diameter of the fully expanded balloon, and the minimal diameter of the lumen subsequent to expansion, were 8.2 ± 2.4, and 7.7 ± 2.3 mm, giving an absolute recoil of 0.5 ± 0.4 mm, and a proportional recoil of 7 ± 4%. There was no significant difference in either the absolute or proportional recoil for any of the stents, or for any of the different stenotic vessels. The proportional recoil correlated linearly with the minimal diameter of the lumen prior to the procedure, and with the ratio of the stenosis to the balloon, while the diameter of the stenotic vessels, the minimal diameter of the largest fully expanded balloon, the proportional stenosis prior to the procedure, and the ratio of the balloon to the diameter of the stenotic vessel, had no significant correlation with proportional recoil. The proportional recoil exceeded more than one-tenth when the minimal diameter of the lumen prior to the dilation was less than 3 mm, or the ratio of the balloon to the stenosis was greater than 3.0.

An absolute recoil of around 1 mm is common when a large or medium Palmaz, or a Palmaz Corinthian stent, is implanted in great vessels. Balloons with a diameter of approximately one-tenth greater than that of the adjacent vessel may be needed if the minimal diameter of the lumen is small prior to the procedure.

New data are emerging on the use of palivizumab as prophylaxis against infection with the respiratory syncytial virus in infants with congenital cardiac disease. Following a 4-year multicentre randomised trial, it was shown that prophylactic injections with palivizumab were effective and safe for such children. Prophylaxis consists of 5, monthly, intramuscular injections of palivizumab, at a dose of 15 mg/kg, given during the season for infection with the respiratory syncytial virus. Timing is at the discretion of the physician, depending on the onset of the season locally. It is suggested that, in the United Kingdom, this should be commenced in mid-September. To help clinicians to identify appropriate candidates for palivizumab, a working group of the British Paediatric Cardiac Association has developed recommendations.

Infants, namely those under 1 year old, with congenital cardiac disease likely to benefit from prophylaxis include those with haemodynamically significant lesions, particularly increased pulmonary blood flow with or without cyanosis; pulmonary venous congestion, pulmonary hypertension or long-term pulmonary complications, residual haemodynamic abnormalities following medical or surgical intervention (patients who have undergone cardiopulmonary bypass should receive an injection as soon as they are medically stable), cardiomyopathy requiring treatment, and congenital cardiac disease likely to need hospital admission for medical or surgical intervention during the season of infection with the virus. Prophylaxis with palivizumab may also be indicated, at the discretion of the physician, in some children with complex cardiac disease over the age of 1 year. Children less likely to benefit from prophylaxis are those with haemodynamically insignificant disease, or those with lesions adequately corrected by medical or surgical intervention.

The appropriate timing of intervention in patients with chronic aortic incompetence allows recovery of ventricular function. We sought to determine the optimal timing of the Ross procedure for chronic aortic incompetence in young patients. We retrospectively analysed case notes, and measured pre- and postoperative echocardiographic indexes of left ventricular function, in patients who had undergone the Ross procedure for chronic aortic incompetence. Methods and results: We found 21 patients with preoperative and postoperative data suitable for analysis. Their age at operation ranged from 5.6 to 26 years, with a median of 13.8 years, and the duration of follow-up was from 0.5 to 6.8 years, with a median of 2.4 years. The preoperative left ventricular end-diastolic dimension was converted to a z-score, and this was used as a threshold to divide the population. Using the threshold of a preoperative left ventricular z-score of more than 3 to divide the population did not show any difference in postoperative parameters of left ventricular function. Significant differences were found postoperatively, however, in both the left ventricular z-score and the ratio of left ventricular end-diastolic radius to posterior wall thickness in diastole, with a cutoff preoperative threshold z-score greater than 4. Conclusion: The increase in the ratio of left ventricular end-diastolic radius to the thickness of the posterior wall in diastole would suggest that there is disruption of left ventricular short axis architecture and myocardial contractile function when intervention is postponed. The significantly larger left ventricular dimension at end-diastole, despite the reduction in volume loading post surgery, may also demonstrate irreversible structural changes. Our data would suggest that recovery of left ventricular function is less likely when the left ventricular z-score has reached the value of 4, and that, ideally, intervention should be performed when the z-score approaches or exceeds 3.

To date, no reference values have been provided for right and left atrial filling in normal children. The aim of our study, therefore, was to characterize measurements of superior caval, hepatic, and pulmonary venous flow using Doppler echocardiography in a large group of normal children to reflect the effects of age, body mass index, sex, heart rate and respiration.

Doppler echocardiographic examinations of the superior caval, hepatic and pulmonary veins were performed during inspiration and expiration in 72 healthy children with a mean age of 6.73 ± 5.10 years. The subjects were segregated into four age groups, namely infants <2 years, preschool children between the ages of 2 and 7 years, children of school age between 7 and 11 years, and adolescents older than 11 years.

Age has significant effect on the systolic and reverse atrial flows within the superior caval vein (p < 0.05). No change in the Doppler velocities was observed related to body mass index or sex. All peak systolic velocities decreased significantly during expiration (p < 0.05). This decrease was most prominent in the hepatic vein (26%), but less remarkable in the superior caval vein (5.7%) and the pulmonary veins (3.9%). During expiration, the peak diastolic flow in the superior caval and the hepatic veins decreased, while the reverse atrial flow in the hepatic vein increased (p < 0.05). Pulmonary venous velocities were similar in all age groups (p > 0.05). Except for the systolic pulmonary venous velocities, these parameters were not influenced by respiration (p > 0.05). The diastolic time, the interval between reverse atrial flow and ventricular systole reflected by the R wave on the electrocardiogram, and the interval between ventricular systole and diastolic flow, were negatively correlated with heart rate (p < 0.05; r = −0.35, −0.85, and −0.8 respectively), and positively correlated with age (p < 0.05; r = 0.3, 0.8, and 0.7 respectively). They were not influenced by respiration.

Our study provides data of the patterns and the normal ranges of velocities of superior caval, hepatic, and pulmonary venous flow in a series of normal children. The results can now be used for comparison with the patterns found in the setting of disease.

We designed a multi-hospital prospective study of children less than 12 years to determine the comparative clinical profile, severity of carditis, and outcome on follow up of patients suffering an initial and recurrent episodes of acute rheumatic fever. The study extended over a period of 3 years, with diagnosis based on the Jones criteria. We included 161 children in the study, 57 having only one episode and 104 with recurrent episodes. Those seen in the first episode were differentiated from those with recurrent episodes on the basis of the history. The severity of carditis was graded by clinical and echocardiographic means. In those suffering their first episode, carditis was significantly less frequent (61.4%) compared to those having recurrent episodes (96.2%). Arthritis was more marked in the first episode (61.4%) compared to recurrent episodes (36.5%). Chorea was also significantly higher in the first episode (15.8%) compared to recurrent episodes (3.8%). Sub-cutaneous nodules were more-or-less the same in those suffering the first (7%) as opposed to recurrent episodes (5.8%), but Erythema marginatum was more marked during the first episode (3.5%), being rare in recurrent episodes at 0.9%. Fever was recorded in approximately the same numbers in first (45.6%) and recurrent episodes (48.1%). Arthralgia, in contrast, was less frequent in first (21.1%) compared to recurrent episodes (32.7%). A history of sore throat was significantly increased amongst those suffering the first episode (54.4%) compared to recurrent episodes (21.2%). When we compared the severity of carditis in the first versus recurrent episodes, at the start of study mild carditis was found in 29.8% versus 10.6%, moderate carditis in 26.3% versus 53.8%, and severe carditis in 5.3% versus 31.8% of cases, respectively. At the end of study, 30.3% of patients suffering their first episode were completely cured of carditis, and all others showed significant improvement compared to those with recurrent episodes, where only 6.8% were cured, little improvement or deterioration being noted in the remainder of the patients. We conclude that the clinical profile of acute rheumatic fever, especially that of carditis, is milder in those suffering their first attack compared to those with recurrent episodes.

Objective: Modern surgical techniques now offer the potential for reconstructing rather than replacing the mitral valve in those patients who require an operation during childhood. The purpose of this study was to assess the outcome in our centre following mitral valvar surgery in children. Methods: Between 1992 and 2002, 51 consecutive children, with a mean age of 12.5 ± 3.5 years, underwent surgical treatment of mitral valvar stenosis and/or insufficiency in the Cardiovascular Surgical Department of Istanbul University. Of the children, 20 underwent repair, whilst we replaced the valve in 31 patients. In one, we inserted a pulmonary valvar autograft in mitral position. Results: There were neither early nor late deaths in those who underwent mitral valvar repair. Similarly, there were no early deaths in those in whom the valve was replaced, but two patients died late, while six outgrew their prosthetic valves and required insertion of a further valve within 10 years of their first operations. Conclusion: Our experience shows that procedures designed to repair the mitral valve are effective and reliable when performed in children, with low mortality, and a lower incidence of reoperation when compared with those having replacement of the mitral valve.