To investigate QT dispersion in the surface electrocardiogram of children with rheumatic carditis.

QT dispersion was quantitatively evaluated in 33 children with acute rheumatic carditis. As a control group, we studied 33 healthy children free of any disease. The children were eligible for participation if the following criteria were met: diagnosis of acute rheumatic fever based on the revised Jone’s criteria and suffering from their first attack of carditis. The echo Doppler cardiogram was performed in all children, within 48–72 hours of hospitalisation.

Patients with carditis had a greater QT dispersion than the control group. When we analyse the QT dispersion according to the severity of the carditis, we observed that the dispersion tended to be greater in those with more severe valvar lesion. The sensitivity and specificity of the measurements of the QT dispersion in predicting acute carditis were estimated by using receiver operating characteristic curves. A QT dispersion greater than 40 milliseconds had a sensitivity of 63.6% and a specificity of 93.9% in predicting acute rheumatic carditis.

The lengthening of QT dispersion may reflect on cardiac involvement in rheumatic fever and be a new important parameter in the diagnosis and therapeutic decision for rheumatic carditis.

The purpose of this study was to evaluate the utilisation of a web-based multimedia patient-accessible electronic health record, for patients with congenital cardiac disease.

This was a prospective analysis of patients undergoing congenital cardiac surgery at a single institution from 1 September, 2006 to 1 February, 2009. After meetings with hospital administration, physicians, nurses, and patients, we configured a subset of the cardiac program’s web-based clinical electronic health record for patient and family access. The Electronic Health Record continuously measured frequency and time of logins, logins during and between hospitalisations, and page views by type (imaging versus textual data).

Of the first 270 patients offered access to the system, 252 became users (93% adoption rate). System uptime was 99.9%, and no security breaches were reported. Users accessed the system more often while the patients were in hospital (67% of total logins) than after discharge (33% of total logins). The maximum number of logins by a family was 440, and the minimum was 1. The average number of logins per family was 25. Imaging data were viewed significantly more frequently than textual data (p ⩽0.001). A total of 12 patients died during the study period and 11 members of their families continued to access their Electronic Health Records after the date of death.

A web-based Patient Accessible Electronic Health Record was designed for patients with congenital cardiac disease. The adoption rate was high, and utilisation patterns suggest that the Electronic Health Record could become a useful tool for health information exchange.

Inappropriate heart rate response to exercise – chronotropic incompetence – and exercise intolerance are common in patients with a systemic right ventricle. We aimed to assess the relationship between heart rate increase, oxygen consumption, and timing of the right ventricular cardiac cycle in this cohort.

We prospectively studied nine patients with systemic right ventricles and pre-existing pacemakers using Doppler-echocardiography and treadmill exercise testing. Echocardiography was performed at increasing heart rates. Exercise tests were performed with baseline pacemaker settings and with optimised heart rate response in random order. In addition, eight age- and gender-matched controls underwent exercise testing using a similar exercise protocol.

Patients with a systemic right ventricle had significantly lower peak oxygen consumption compared to controls – 12.6 plus or minus 6.8 versus 31.4 plus or minus 6.6 metres per kilogram per minute (p = 0.0006) – at baseline and active pacemaker reprogramming failed to increase peak oxygen consumption in this cohort – 12.6 plus or minus 6.8 versus 12.4 plus or minus 4.9 millilitres per kilogram per minute (p = NS) at baseline and with reprogramming, respectively. We found not only a marked increase in total isovolumic time but also a significant reduction in total filling time and the aortic velocity time integral, p-value is less than 0.001 for all, at higher heart rates compared to baseline conditions.

This study suggests that despite chronotropic incompetence at baseline, rate-responsive pacing does not improve exercise capacity in patients with a systemic right ventricle. It further indicates that high heart rates may be detrimental in these patients by reducing diastolic filling and stroke volume. These findings may have clinical implications when considering implantation of a permanent pacemaker in this cohort.

We aimed to determine whether N-terminal pro-brain natriuretic peptide can differentiate between cardiac and pulmonary aetiologies of dyspnoea, if N-terminal pro-brain natriuretic peptide can be used for evaluating the effect of treatment in cardiac failure, and for predicting severe pulmonary diseases that are complicated by cardiac failure.

In all, 76 children with dyspnoea were enrolled; 41 of them suffered cardiac failure – 25 caused by cardiac disease, 16 caused by pulmonary disease – and 35 had dyspnoea due to pulmonary disease. The control group consisted of 32 children. We calculated Ross scores, analysed N-terminal pro-brain natriuretic peptide levels, and evaluated left ventricular systolic functions by echocardiography.

N-terminal pro-brain natriuretic peptide levels were significantly higher in children with cardiac failure than in those with pulmonary disease and in controls (medians 7321, 241, 87.71 picograms per millilitre, respectively), were higher in children with cardiac failure due to pulmonary disease than in those with only pulmonary disease (medians 2728, 241 picograms per millilitre, respectively), and were higher in children who died from cardiac failure than in survivors (p < 0.05). After treatment of cardiac failure, N-terminal pro-brain natriuretic peptide levels decreased significantly (p < 0.001). The cut-off level of N-terminal pro-brain natriuretic peptide for differentiating cardiacfailure from pulmonary disease was 726.8 picograms per millilitre, sensitivity 100%, specificity 94.3%.

N-terminal pro-brain natriuretic peptide levels can differentiate dyspnoea due to cardiac failure from pulmonary diseases. It can also be used to monitor the effects of treatment of cardiac failure and to estimate the prognosis, as well as to predict pulmonary diseases that are complicated with cardiac failure.

To conduct a prospective randomised study to show the efficacy of immune suppression with prednisolone, administered at the 3-month duration of acute myocarditis.

The diagnosis of acute viral myocarditis was made based on echocardiography and serum viral antibodies. The inclusion criterion was acute myocarditis of 3 months duration. In all, 68 of 173 children were available for randomisation into a prednisolone-treated group of 44 and a control group of 24 children. The follow-up period in the prednisolone-treated group was 15.1 plus or minus 9.2 months and 13.6 plus or minus 10.6 months for the control group.

Compared with controls, 1 month after randomisation significantly more children in the prednisolone-treated group increased their ejection fraction to more than 40% (p = 0.029). Discrete analysis of change in the ejection fraction from the one at randomisation to one after 1 month of randomisation of greater than 10% and less than 10% or no change between groups showed a significantly greater number with improvement in the prednisolone-treated group (p = 0.019). At the end of the follow-up visits, a significantly larger number of children in the prednisolone-treated group had an ejection fraction of more than 60% compared with the control group (p = 0.049).

It is concluded that immune suppression with prednisolone, administered at 3 months of the onset of acute myocarditis, is effective in significantly bringing about improvement and cure in persistent left ventricular failure.

Staged left ventricular rehabilitation is a novel surgical approach in patients undergoing single ventricle palliation for borderline hypoplastic left cardiac disease, in an attempt to salvage the left ventricle. The procedure includes resection of endocardial fibroelastosis from the left ventricular free wall and apex. We hypothesised that endocardial fibroelastosis removal may significantly affect ventricular conduction and myocardial electrical characteristics.

This study included 27 patients with borderline hypoplastic left cardiac syndrome who underwent staged left ventricle rehabilitation with endocardial fibroelastosis resection following single ventricle palliation. The effect on electrical synchrony was measured by ventricular depolarisation timing (QRS duration) on electrocardiogram. Patients were evaluated for a change in QRS duration before and after fibroelastosis removal and at most recent follow-up.

The QRS change in the immediate period after endocardial fibroelastosis resection ranged from −16 to 36 milliseconds with a median of 0 (p = 0.09). However, long-term conduction delay was common in 44% (12/27) of patients having a QRS duration greater than 98th percentile for the age at the most recent electrocardiogram. Only one patient had QRS duration greater than 98th percentile before any surgical procedure. Two patients developed left bundle branch block and one developed right bundle branch block with left, but anterior-fascicular block. Overall, the QRS duration correlated with left ventricular size (R = 0.54, p = 0.006) at the most recent electrocardiogram.

Electrical dyssynchrony is a common finding in patients undergoing staged left ventricular rehabilitation after single ventricle palliation; however, it is not acutely related to surgical endocardial resection. Left ventricular size is correlated with QRS duration. Diligent follow-up is required to evaluate the effects of left ventricular growth and consideration of resynchronisation in this population.

Sildenafil is increasingly being used in the management of pulmonary arterial hypertension in the newborn. Its role in patients with congenital cardiac disease is less well defined and as yet has only been reported sporadically.

Present our experience with sildenafil treatment in patients with a failing Fontan circulation.

Retrospective review of 13 symptomatic patients after Fontan palliation who received treatment with sildenafil between January, 2006 and July, 2008.

Three patients suffered from protein-losing enteropathy, four patients presented with bronchial casts, two had severe cyanosis after fenestrated Fontan procedure, two had prolonged chylous effusions, one had a previous failure of Fontan and take-down, and one patient had arrhythmias and end-stage cardiac failure requiring conversion to an extra-cardiac Fontan. Sildenafil was used in the dosage of 1–2 milligrams per kilogram 3–4 times per day. Protein-losing enteropathy and α-1-antitrypsin levels improved in all three patients on sildenafil treatment. One of these patients had a concomitant catheter creation of a fenestration, as did two patients presenting with bronchial casts and both patients with persistent chylous effusions. All four patients with bronchial casts and two patients with cyanosis improved significantly on sildenafil treatment. Chylous effusions decreased after sildenafil and stent enlargement of a fenestration. There were no significant side effects requiring sildenafil withdrawal over a treatment period ranging from 2 months to 2 years.

Sildenafil can be used safely and effectively in the treatment of patients with a failing Fontan circulation.

Many studies have reported on the estimation of the development of left ventricular dimensions in children by using two-dimensional echocardiography and formulated equations to evaluate normal cardiac dimensions. We reported that height is the most simple and useful index to evaluate the normal dimensions of the left ventricle in children. Disparities have been detected between the “normal” dimensions estimated from these formulae and real dimensions in infants less than 1 year of age and neonates, by using body weight or body surface area as an index. Our objective is to assess the normal size of left ventricular end-diastolic dimension in infants less than 1 year of age and neonates, including premature neonates, by two-dimensional echocardiography by using height as an index.

The study group consisted of 243 infants, 123 males, and 120 females without congenital cardiac disease. The regression equation for the relationship between left ventricular end-diastolic dimension (Y) and height was Y (millimetre) = 0.352 × height (centimetre) + 1.86 in mature neonates and infants with height of less than 75 centimetres, whereas that in premature neonates was Y (millimetre) = 0.495 × height (centimetre) − 5.43. No significant differences were observed in the infants on the basis of sex.

The regression equations reported in the previous studies cannot be applied to data obtained from infants. In addition, three different formulae, one for each group of infants by using height as an index can be usefully applied for practical purposes.

To assess the general health and activity levels of 4- and 5-year-old children after intervention for congenital cardiac disease.

Health behaviour outcomes were assessed in 91 children who had surgery or catheter intervention for congenital cardiac disease. The children were classified into four groups according to severity. The main parameters of classification were the presence of residual symptoms, frequency of visits to general practitioner or the Accident and Emergency Department, and ability to participate in physical activity according to a calculated “activity score”.

Children had very few residual symptoms after “corrective surgery”. Those with complex congenital cardiac disease post-Fontan-type repair still had symptoms on average 18.2 days per month. Surprisingly, the complex group had fewer days “sick” from non-cardiac causes and had fewer visits to general practitioner or Accident and Emergency Departments. Regression analysis indicates that three variables had significant relevance to the general practitioner or Accident and Emergency visits: complex congenital cardiac disease, fewer visits; Townsend score – more deprivation – more visits; and maternal worry – higher maternal worry score – more visits. Regression analysis indicates that lower activity score is significantly related to complex cardiac disease and higher maternal worry score.

The majority of this group of 4- and 5-year-old children had few residual symptoms and had good exercise tolerance. Maternal worry is a significant factor in influencing both activity levels and frequency of unscheduled health service demands – general practitioner or Accident and Emergency visits.

Studies show that anti-epileptic drugs increase oxidative stress. Thus, low-density lipoprotein oxidation increases and atherogenesis is induced. Paraoxonase-associated high-density lipoprotein protects low-density lipoprotein and high-density lipoprotein oxidation. The effects of anti-epileptic drugs on paraoxonase activity has not been investigated yet. The aim of this study is to investigate the effect of anti-epileptic drugs on paraoxonase activity, lipid profiles, folat, vitamin B12, homocysteine, thyroid hormones, apolipoprotein A-1, total anti-oxidant capacity, malondialdehyd, nitric oxide, and oxidised low-density lipoprotein. The association with carotid–femoral pulse wave velocity and current biochemical parameters had been searched for assessing the effects of anti-epileptic drugs on the vascular system.

We recruited 59 epileptic patients treated with anti-epileptic drugs and 23 controls (group IV) at least 6 months ago. The epileptic group was divided into three groups by receiving anti-epileptic drugs as follows: group I: carbamazepine, group II: valproic acid, and group III: carbamazepine and valproic acid. Arterial distensibility was assessed with the Complior device.

There was no difference between the current biochemical parameters in epileptic children. Serum-free T4 was decreased, when compared with group IV. Thyroid-stimulating hormone was increased in group II, compared with group IV. The carotid–femoral pulse wave velocity was increased in group III, compared with group IV. The carotid–femoral pulse wave velocity was correlated with thyroid-stimulating hormone and valproic acid levels.

Anti-epileptic drugs may induce atherogenesis by affecting the thyroid hormones. According to the current data, the effects of thyroid hormones on vascular system may be independent of other biochemical markers. Epileptic patients using anti-epileptic drugs must be followed closely for arterial stiffness, and also for the development and progression of atherosclerosis.