Objectives: Acute rheumatic fever and its sequel, rheumatic heart disease, is a major problem in children, adolescents and young adults. Despite the widespread application of the Jones criterions, carditis is either underdiagnosed or overdiagnosed. Echocardiography is rarely used optimally for precise diagnosis. The objective of our study, therefore, was to define the potential role of echocardiography in detecting carditis in the setting of acute rheumatic fever. Materials and methods: We performed echocardiography in 452 consecutive patients with acute rheumatic fever, clinically diagnosed by the strict Jones criterions, using the patients as part of a multi-centric and double blinded prospective study. Results: Of our 452 patients, 230 were males, and 222 were females. The youngest was aged 1 year 11 months, while the oldest was a 51-year-old female. Out of the 452 cases of acute rheumatic fever, 239 patients (52.8%) had arthritis. Out of 164 cases of clinically diagnosed carditis, only 141 cases had echocardiographic evidence of carditis (85.97%). The remaining 23 patients (14%) had functional murmurs, tachycardia, or anaemia. Of the patients, 2 also had congenitally malformed hearts. Of 40 patients with rheumatic chorea, 28 (70%) had echocardiographic evidence of carditis or valvitis. Polyarthralgia was seen in 213 cases (47.12%), from which only 38 patients (17.8%) had carditis clinically, albeit that 88 patients (41.3%) showed echocardiographic evidence of subclinical carditis or valvitis. Conclusion: Echocardiography, when carried out in patients with acute rheumatic fever diagnosed strictly according to the Jones criterion, can avoid both overdiagnosis and underdiagnosis of carditis. A high incidence of carditis, or subclinical carditis, is detected by echocardiography when performed in patients with rheumatic chorea or arthralgia.

Aims: The criterions for the timing of surgical intervention in children with rheumatic mitral or aortic valvar regurgitation are not defined. I hypothesized that, in children with chronic mitral or aortic regurgitation, an index for decompensation could be created by using the ratio of the diastolic left ventricular wall thickness to the radius, and that such an index could prove useful in determining the optimal time for surgical intervention. Methods: The left ventricular echocardiograms were obtained at the tips of the leaflets of the mitral valve by M-mode echocardiography. The diastolic septal wall thickness was measured between the right and left ventricular endocardial layers, and the posterior wall thickness between the endocardium and the interphase between the epicardium and the myocardium. The left ventricular diastolic dimension was then measured, between the posterior and septal wall endocardial layers, and systolic dimension as the smallest distance detected between these layers. All diastolic measurements were made at the time of the R wave of electrocardiogram, using the leading edge technique. The ratio of wall thickness was measured using the mean of septal and posterior wall thicknesses divided by half the diastolic dimensions, the normalized thickness of the wall previously referred to as the h/r ratio and relative mural thickness. Results: The ratio of wall thickness to left ventricular radius, and its relation to systolic left ventricular pressure or systolic blood pressure, was found to be linear in 89 normal school children, and 39 children with aortic stenosis. For future predictions, I calculated the 95th percentile limits and the 95th percentile confidence bands for this relation. Using the same data, it proved possible to calculate ratios of wall thickness for various ranges of either systolic blood pressure or left ventricular peak pressure. By using the normal limits of 0.356 plus or minus 0.0316 of the ratio, appropriate for the systolic blood pressure of children with mitral regurgitation, I determined the adequacy of the ratio of wall thickness. Of the children, 51 were in ventricular failure, and these had an inadequate ratio, below two standard deviation. Of the others, 21 had an inadequate ratio to within minus one to minus two standard deviations, and 12 of these were asymptomatic, 8 were symptomatic, but only one was in ventricular failure. For 18 children with aortic regurgitation, using the same limits, one child was within 1 standard deviation and was asymptomatic, 8 fell within minus 1 to minus 2 standard deviations and 2 of these were symptomatic, 5 were in ventricular failure, and 1 was asymptomatic, while the other 9 had ratios falling less than minus 2 standard deviations, and all were in ventricular failure. Conclusion: I conclude that the index of normalized wall thickness defined as the ratio of the left ventricular wall thickness to its radius is adequate, and within normal limits, when there is compensated volume overload, but is inadequate and below normal limits when the volume overloaded left ventricle becomes decompensated. My data suggests that the persistently decreasing ratio of wall thickness below the limits of normality serves as an indicator of ventricular decompensation, and thus can be used as a new criterion for determining the optimal time for surgical intervention.

Introduction: We sought to establish a modern understanding of the safety and efficacy of trans-catheter pulmonary arterial angioplasty. Methods: A review of records in a sample of 104 dilations, out of a total of 711 procedures undertaken between January, 1996 and December, 2000, provided descriptive information regarding technique, adverse events, and changes achieved in luminal diameter. Because evidence during the review of angiograms suggested substantial differences according to whether the stenotic lesions were positioned proximally or distally within the pulmonary arterial tree, all analyses incorporated this classification. Results: We reviewed stenoses in 203 pulmonary arteries, 38% located proximally and 62% distally, with follow-up available concerning dilation in 92 vessels. Proximal dilations frequently involved a prior surgical site, and appeared more compliant and amenable to conventional angioplasty, as evidenced by more common elimination of the waist, but also more recoil, then requiring placement of stents. In contrast, distal lesions frequently required balloons capable of sustaining high pressures of inflation, and larger balloons relative to the size of the vessels. The proportional increase in diameter was greater for distal sites, at 90 plus or minus 77%, compared to proximal, at 64 plus or minus 70%, p equal to 0.002. Serious adverse events occurred in 3 of 104 procedures, giving a rate of serious adverse events of 2.9%. At follow-up, 9 of 92 vessels (10%), 95% confidence intervals from 5% to 18%, returned to their diameters prior to dilation, with no difference in the rate of restenosis according to the site of dilation. Conclusion: Our findings indicate the need to distinguish, and to consider, the important differences in technical issues and outcomes, when performing dilations at proximal as opposed to distal sites. Although angioplasty is effective therapy for pulmonary arterial stenosis, a subset of vessels, more often distal, remain resistant to conventional techniques.

Background: We sought to determine if the acute gains in pulmonary arterial diameter achieved by using Cutting Balloons to dilate stenoses in small arteries are maintained at follow-up. Methods: A search of our database identified all patients who underwent dilation with Cutting Balloons for pulmonary arterial stenosis between June 2001 and September 2003. We reviewed the procedural and angiographic data obtained at the initial and follow-up procedures. Results: Over the period of study, 29 patients with a median age of 3.7 years, and a range from 0.4 to 46.6, underwent treatment with Cutting Balloons at 41 procedures for one or more pulmonary arterial obstructions resistant to conventional angioplasty. At the initial procedure, we enlarged 79 vessels, with an initial minimal luminal diameter of 1.5 plus or minus 0.8 millimetres, to a diameter of 3.0 plus or minus 1.1 millimetres (p smaller than 0.001). Angiographic data indicated that 49% of the vessels showed evidence of vascular damage not requiring intervention, while stents were placed in 9 vessels. Follow-up angiography was available for 39 of the vessels, evaluated at a median of 6 months, with a range from 3 to 24 months, after the initial procedure. In these vessels, there was a mean loss of 10 plus or minus 25% in luminal diameter, p not significant. Of the 39 vessels, 8 had returned to a diameter within half of the initial diameter, giving a failure rate at follow-up of 21%, with 95% confidence intervals from 9% to 36%. All complications were identified within 24 hours of the catheterization procedure, and there were no late complications or deaths during the period of follow-up. Placement of stents produced both greater increases in diameter at the initial procedure (p smaller than 0.001), and greater losses at follow-up (p equal to 0.01). Conclusions: Despite the frequent identification of intravascular trauma, initial gains in luminal diameter after use of Cutting Balloons to dilate stenotic pulmonary arteries are maintained at follow-up.

We describe the electrophysiological studies undertaken in four patients with atrioventricular nodal reentry tachycardia in the setting of concordant atrioventricular and discordant ventriculo-arterial connections (transposition). Radiofrequency ablation was attempted in three, all with success. Clear evidence of dual antegrade pathways through the atrioventricular node was present in only one of the four, but other characteristics of discrete fast and slow pathways into the atrioventricular node were present in all. Atrioventricular nodal reentry tachycardia was inducible in all. In the three patients in whom ablation was attempted, the application of radiofrequency energy to the low medial regions of the systemic venous atrium (morphologically left) consistently caused junctional accelerated rhythm, but these lesions were not successful in eliminating the tachycardia. Successful radiofrequency ablation required a retrograde approach to the region of the slow pathway in the pulmonary venous atrium (morphologically right).

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

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

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

Background: Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood. Patients and methods: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, with a mean follow-up of 10.8 years. Of the patients, 31 had died, 16 suddenly, with a median age at sudden death of 13.3 years. Results: Cox regression shows that electrocardiographic voltages, analysed as the sum of the R and S waves in all six limb leads (p equal to 0.001), and septal thickness expressed as proportion of the 95th centile for age (p equal to 0.036), were independent predictors of sudden death. When the sum of the R and S waves is over 10 millivolts, the odds ratio for sudden death was 8.4, with 95% confidence intervals from 2.2 to 33.7 (p equal to 0.0012), and finding a septal thickness over 190% of 95th centile for age gives an odds ratio of 6.2, with confidence intervals from 1.5 to 25.1 (p equal to 0.011). Noonan's syndrome, with a p value equal to 0.043, and the ratio of the left ventricular wall to its cavity in diastole, with a p value equal to 0.005, were independent predictors of death in cardiac failure, with a ratio of the mural thickness to the dimension of the cavity over 0.30 giving an odds ratio of 36.0, with confidence limits from 4.2 to 311, and a p value equal to 0.00009. At follow-up, patients deemed to be at a high risk of dying suddenly were identified by the combination of the sum of the R and S waves greater than 10 millivolts and septal thickness over 190%, with a sensitivity of 91%, specificity of 78%, positive predictive value of 50%, and a negative predictive value of 97%. Conclusions: Children at high risk of dying suddenly with hypertrophic cardiomyopathy, with a subsequent annual mortality of 6.6%, can be distinguished at the time of diagnosis from those patients having a low risk of sudden death, the latter with an annual mortality of 0.27%.

A female neonate, born at term, presented with complex cardiac anatomy dominated by right isomerism and infra-diaphragmatic totally anomalous pulmonary venous connection. Surgical repair was performed using circulatory arrest under deep hypothermia. In the postoperative period, the patient could not be weaned off mechanical ventilation, and underwent cardiac catheterization. This revealed major aortopulmonary collateral arteries, which were occluded with coils. We review the literature with specific focus on the occurrence of this unusual combination and its implication in the management during the postoperative period.

The single beat method was used to evaluate right and left ventriculo-arterial coupling in an asymptomatic patient with congenitally corrected transposition. The ratio of ventricular end-systolic to arterial elastances was normal for the left ventricle coupled to the pulmonary circulation, and depressed for the right ventricle coupled to the systemic circulation. This result suggests that chronic uncoupling of the right ventricle to the systemic circulation might play a role in the pathophysiology produced by congenitally corrected transposition.

We report the rare combination of a severely hypoplastic left ventricle and discordant ventriculoarterial connections, with associated subpulmonary obstruction of the left ventricular outflow tract. The long tortuous arterial duct originated at an acute angle from the aorta, suggesting that the subpulmonary obstruction developed early in fetal life. Residual flow via the hypoplastic left ventricle to the pulmonary circulation after insertion of an aortopulmonary shunt resulted in haemodynamic deterioration, which was instantly reversed by closing the pulmonary trunk.

Temporary wires are routinely sutured to both the atrial and ventricular epimyocardium after open heart surgery. Despite their rarity, complications related to removal of such pacemaking wires may cause life threatening situations. We describe here a patient who developed ventricular fibrillation immediately after removal of temporary epicardial pacemaking wires.

We describe our experience with prenatal diagnosis of tetralogy of Fallot with supracardiac totally anomalous pulmonary venous connection. We also suspected obstruction in the ascending vertical vein as it crossed the right bronchus and coursed superiorly to join the right superior caval vein. This finding was confirmed on postnatal echocardiography, and at autopsy.

A term infant rapidly developed profound cyanosis and metabolic acidosis shortly after an uncomplicated vaginal delivery. Echocardiography identified a flail antero-superior leaflet of the tricuspid valve, which was producing severe tricuspid insufficiency. The clinical state deteriorated despite maximal medical management, and the patient was placed on venoarterial extracorporeal membrane oxygenation. Within twenty-four hours, the metabolic acidosis corrected, inotropic support was discontinued, and the patient was weaned to minimal ventilator settings. Successful repair of the tricuspid valve was performed two days later.