Right ventricular function is of great importance in patients with both acute and chronic ventricular overload. The early detection of right ventricular dysfunction may have an impact on therapeutic decision making, helping to prevent or further delay functional deterioration of the right ventricle.

In patients with right ventricular overload due to congenital cardiac diseases, dobutamine stress testing combined with magnetic resonance imaging, electrocardiographic changes, and monitoring of concentrations of plasma brain natriuretic peptide are very suitable parameters for the early detection of ventricular dysfunction, and should therefore be used in the follow-up of these patients.

It is apparent that no single measurement of anatomy or function can ever adequately describe the form or performance of the right ventricle. Rather, we should be looking more towards an integrated approach of different parameters for right ventricular function. The quantitative parameters described in this study can serve this purpose. The strong correlation found between these non-invasive and independent parameters encourages their clinical implementation.

Endomyocardial biopsy in children remains important in the evaluation of cardiomyopathy, myocarditis, and rejection following cardiac transplantation. We sought to evaluate the morbidity associated with endomyocardial biopsy on the basis of a large experience from a single institution.

We reviewed records of all endomyocardial biopsies performed at our institution. Complications were defined as adverse events resulting from the biopsy requiring intervention or additional observation. We also reviewed the most recent echocardiogram from all the patients for the presence and severity of tricuspid valvar regurgitation.

Between November 1986 and April 2002, we performed 1051 endomyocardial biopsies in 135 patients ranging in age from 9 days to 18 years. The internal jugular vein was the site of vascular access in 68% of the procedures. There were 10 acute procedural complications requiring intervention or additional observation. Severe tricuspid regurgitation developed in two patients who had undergone multiple biopsies after cardiac transplantation, one of whom underwent subsequent replacement of the tricuspid valve. There were no deaths or cardiac perforations. The total incidence of morbidity was 1.1%. No demographic or procedural factors were identified to be predictive of complications.

In experienced hands, therefore, endomyocardial biopsy can be safely performed in children with very low morbidity.

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.

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.

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.

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.

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.

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.

Objectives: A retrospective study was undertaken to determine the degree of pulmonary regurgitation following percutaneous balloon valvoplasty for isolated pulmonary valvar stenosis. Background: Percutaneous balloon valvoplasty is the recognised treatment of choice in pulmonary valvar stenosis with effective relief of gradient. Few studies have reviewed the degree of pulmonary regurgitation after the balloon valvoplasty. Methods: We reviewed all patients with isolated pulmonary valvar stenosis undergoing percutaneous balloon valvoplasty at a tertiary centre between December 1984 and August 1996. Those with an associated haemodynamically insignificant atrial septal defect or patent oval foramen were also included. Their medical records, echocardiograms and angiograms were studied. Colour flow Doppler was used as a semi-quantitative way of assessing the pulmonary incompetence. Results: Over the period of review, 57 procedures had been performed in 49 patients. The median age at the time of the procedure was 1.08 years, with a range from 0.01 to 13.3 years. The median period of follow-up was 5.64 years, with a range from 3.00 to 14.26 years. Immediately following the dilation of the pulmonary valve, the peak-to-peak instantaneous systolic pressure gradient was significantly reduced. Seven patients required a second dilation of the valve. Their median age at the first valvoplasty, at 0.49 year, was significantly lower than those who required only one procedure, at a median of 1.50 years. Following the valvoplasty, mild pulmonary incompetence was noted in 26 out of 42 patients the day after the procedure, but only 7% had moderate incompetence. On follow-up, there was an increase in the number of patients with moderate to severe pulmonary incompetence, from 7% to 29%. Those patients in whom the procedure was performed at a younger age had more significant pulmonary incompetence. Neither the initial gradient across the pulmonary valve, nor the size of the balloon used, were related with statistical significance to the subsequent development of pulmonary incompetence. Conclusions: The majority of the patients with congenital isolated pulmonary valvar stenosis had only a mild increase in the degree of pulmonary incompetence following a single pulmonary valvoplasty. Patients who required the procedure early in life were more likely to develop significant pulmonary incompetence. The ratio of the size of the balloon to the diameter of the valve did not significantly affect the outcome. We suggest that those patients who had more severe stenosis because of a more abnormal pulmonary valve, and hence required early intervention, were more likely to develop greater pulmonary incompetence after the valvoplasty.

In recent years, three-dimensional imaging has provided new opportunities for visualizing congenital cardiac malformations. We present the initial clinical experience using a recently implemented system, which employs some of new interactive, real-time, techniques. We show how three-dimensional rendering based on magnetic resonance imaging can provide detailed spatial information on both intrinsic and extrinsic cardiac relations, and hence how a virtual examination can potentially provide new means to a better understanding of complex congenital cardiac malformations.

We describe a new method of three-dimensional magnetic resonance imaging of the heart that has been used to produce high quality diagnostic images in 274 patients with congenital cardiac disease, ranging in age from 1 day to 66 years. Using a steady state free precession gradient echo technique and parallel imaging, rapid acquisition of the entire cardiac volume is possible during 8 to 15 sequential breath-holds, each lasting between 8 and 15 s. We obtained high-resolution images, with a resolution of 1 mm3, at between 3 and 10 phases of the cardiac cycle.

While images of diagnostic quality were obtained in all cases, in 52 patients there was some degradation due to various factors. Children under 8 years were ventilated, and ventilation was suspended for the breath-holds. For patients breathing spontaneously a novel respiratory navigator technique was developed, using a navigator echo placed over the right hemidiaphragm. This was used successfully in 20 patients, and reduced the misalignment of images obtained during different breath-holds.

Images were analysed using multi-planar reformatting and volume rendering. Image processing took approximately five minutes for each study. End-diastolic images were processed for all patients. Systolic images were also processed in selected cases.

Further improvements in parallel imaging should reduce imaging times further, so that it is possible to obtain the full volume image in a single breath-hold. This will enable imaging of complex anatomy to be obtained using a standard imaging protocol that does not require the operator to understand the cardiac malformation, making the magnetic resonance imaging of congenital cardiac disease faster and more effective.

We describe the clinical features of idiopathic restrictive cardiomyopathy in a female infant. A marked elevation of left ventricular end-diastolic pressure, and profoundly abnormal myocardial relaxation, were detected with the use of Doppler blood flow echocardiography, coupled with the relatively new technique of Doppler tissue echocardiography. There was no clinical evidence of ongoing heart failure, but she had signs of myocardial ischaemia, and unfortunately died suddenly at the age of 13 months.

In a 4-year-old boy with severe mitral regurgitation, cross sectional echocardiography combined with Doppler interrogation confirmed the presence of isolated perforation of the aortic leaflet of the mitral valve. The perforation was closed with a patch of fresh autologous pericardium. Serial echocardiograms taken postoperatively showed no regurgitation across the mitral valve.

We describe the clinical history of a nine years old girl with Shone's syndrome. She underwent balloon angioplasty of the aortic coarctation in infancy, and later developed severe sub-aortic stenosis and moderate mitral valvar stenosis. The mitral valve was therefore replaced with a mechanical prosthesis, and the sub-aortic shelf was resected. Immediately after the operation, she developed signs of myocardial ischemia. Coronary angiography showed compression of the middle part of the circumflex artery by the mechanical prosthesis, the obstructed segment being successfully dilated using a coronary arterial stent.

We treated seromas developing in two patients in relation to left-sided modified Blalock-Taussig shunts by reattaching the original graft to the descending aorta. We postulate that seromas may develop because of the large area of contact between the graft and blood. Shortening the length of the Gore-Tex graft may therefore be a feasible treatment.

Abnormalities of chromosomes 11 and 22 are associated with congenital cardiac disease and/or various syndromes. We present a patient with partial trisomy for the long arms of chromosomes 11 and 22, the result of a maternal balanced reciprocal translocation between these two chromosomes. Our patient was dysmorphic and had coarctation of the aorta, an atrioventricular septal defect with common atrioventricular junction and exclusively atrial shunting, patency of the arterial duct, supracardiac totally anomalous pulmonary venous connection, a single kidney, and tracheobronchomalacia. This patient is unusual in having extensive left-sided cardiac involvement, a feature not usually found in this condition.

In neonates with severe aortic or pulmonary stenosis, the obstructed circulation is usually supported through patency of the arterial duct. We describe a neonate in whom an aortopulmonary window supported a critically obstructed pulmonary circulation.