The aim of our study was to establish the prevalence and the prognostic value of haematological abnormalities in children with cardiac failure.

A series of 218 consecutive children with a first diagnosis of idiopathic dilated cardiomyopathy were retrospectively examined. Haematological evaluation was performed at first diagnosis. Death or cardiac transplantation was the main outcome measure.

The median age was 0.6 years, ranging from 1 day to 15.8 years and median follow-up was 2.65 years, ranging from 0 to 17.2 years. After a median interval of 0.2 years, ranging from 0 to 8.7 years, 56 patients died and 25 were transplanted. Event-free survival at 1 and 5 years was 68% (95% confidence interval, 63–75%) and 62% (95% confidence interval, 56–69%). Blood levels of haemoglobin less than 10 grams per decilitre, urea over 8 millimoles per litre, and C-reactive protein over 10 milligrams per litre were found in 24%, 20%, and 24% of patients, respectively. The log-rank test showed that haemoglobin (p = 0.000) and C-reactive protein (p = 0.021) were predictors of death or transplantation. In the multivariate Cox model, haemoglobin (hazard ratio = 0.735; confidence interval = 0.636–0.849; p = 0.000) and urea (hazard ratio = 1.083; confidence interval = 1:002–1:171; p = 0.045) were predictive of poor outcome. Cubic spline functions showed that the positive role of haemoglobin on survival was linear for values less than 12 grams per decilitre and null for values more than 12 grams per decilitre. Adaptive index models for risk stratification and Classification and Regression Tree analysis allowed to identify the cut-off values for haemoglobin (less than 10.2 grams per decilitre) and urea (more than 8.8 millimoles per litre), as well as to derive a predictor model.

In children with idiopathic dilated cardiomyopathy, anaemia is the strongest independent prognostic factor of early death or transplantation.

Excessive body fat, mainly abdominal fat, is associated with higher cardiovascular risk. However, a fat localisation measurement that would be more indicative of risk in adolescents has not yet been established.

This study was conducted in order to evaluate the correlation between body fat location measurements and cardiovascular disease risk factors in female adolescents.

A total of 113 girls – 38 eutrophic according to their body mass index but with a high percentage of body fat, 40 eutrophic with adequate body fat, and 35 with excessive weight – were evaluated using 15 anthropometrical measurements and 10 cardiovascular risk factors.

The central skinfold was the best measurement for predicting variables such as glycaemia and high-density lipoprotein; waist circumference for insulin and homeostasis model assessment; coronal diameter for total cholesterol and low-density lipoprotein; sagittal abdominal diameter for triglycerides and leptin; hip circumference for blood pressure; and the central/peripheral skinfold ratio for homocysteine. The correlation between the measurements and the number of risk factors showed that waist circumference and the waist/stature ratio produced the best results.

The results suggest that the body fat distribution in adolescents is relevant in the development of cardiovascular risk factors. Simple measurements such as waist circumference and the waist/stature ratio were the best predictors of a risk of disease and they should therefore be associated with the body mass index in clinical practice in order to identify those adolescents at higher risk.

Achievement of adequate oral nutrition is a challenging task after early neonatal cardiac surgery. This study aims to describe predictors of oral feeding outcomes for neonates after early surgical interventions.

A retrospective review of neonates admitted with congenital cardiac disease over a period of 1 year. We analysed predictors of the need for a feeding tube at discharge and the amount taken at each feeding. Multilevel modelling was used to look at individual change over time predicting oral amount at each feeding.

We identified 56 neonates. Diagnoses were heterogeneous; 23% of the infants had associated genetic syndromes and 45% required pre-operative mechanical ventilation. The median time from birth to surgery was 8.4 days, with 29 infants fed orally before surgery. The mean time from surgery to first oral feeding attempt was 12 hours. Time from surgery to oral feeding, the amount taken with first feeding, and cross-clamp times were significant predictors of oral feeding success, whereas the presence of a comorbidity – genetic abnormality – and longer ventilator dependency predicted failure. Almost half of the neonates required a feeding tube upon discharge, and no infant discharged was solely breastfed. Discharge with a feeding tube was associated with greater weight gain at that time.

Neonates with congenital cardiac disease face significant barriers to successfully achieving oral feeding on hospital discharge. Enteral feeding guidelines focus on physiological stabilisation and do not always address the developmental milestones necessary to support oral feeding. Future prospective studies are necessary to identify multimodal strategies to optimise early feeding.

To assess the quality of imaging modalities of a new micro multiplane transoesophageal echocardiogram probe.

This is a prospective study of micro transoesophageal echocardiogram S8-3t probe used at a single institution between 15 December, 2009 and 15 March, 2010. The images were compared with standard paediatric or adult probes where possible. Assessors prospectively rated imaging quality – two dimensional, colour flow imaging, pulse wave, and continuous wave Doppler – with a subjective 4-point scale (1 = poor to 4 = excellent).

A total of 24 studies were performed on 23 patients, with a median weight = 11.7 kilograms (2.6–72 kilograms) and a median age of 3 years (0.16–60 years). Of the 23 patients, one neonate (2.8 kilograms) had transient bradycardia on probe insertion. Imaging in patients less than 10 kilograms was of full diagnostic value and new information was obtained in eight out of ten patients. Pulse wave and continuous wave Doppler was consistently good across all weight groups. There were high frame rates and good imaging quality to a depth of 4–6 centimetres in all studies. A comparison with a larger alternative probe was available for 12 studies (weight 11.9–72 kilograms). The median micro transoesophageal two-dimensional image quality score was 3 (2–4) and 4 (3–4) with the comparative probe. For the 10- to 30-kilogram group, image quality with the micro transoesophageal echocardiogram probe was judged as inferior to larger standard probes. Adult sized patients had good imaging of near the field, allowing guidance for percutaneous device closure of the atrial septum.

The micro multiplane transoesophageal echocardiogram probe provides imaging of diagnostic quality in neonates. In larger patients, it offers good imaging of near field structures. In the intermediate-sized child (10–30 kilograms), standard paediatric probes provide better imaging.

Radiofrequency catheter ablation is the mainstay of therapy for supraventricular tachyarrhythmias. Conventional radiofrequency catheter ablation requires the use of fluoroscopy, thus exposing patients to ionising radiation. The feasibility and safety of non-fluoroscopic radiofrequency catheter ablation has been recently reported in a wide range of supraventricular tachyarrhythmias using the EnSite NavX™ mapping system. The NO-PARTY is a multi-centre, randomised controlled trial designed to test the hypothesis that catheter ablation of supraventricular tachyarrhythmias guided by the EnSite NavX™ mapping system results in a clinically significant reduction in exposure to ionising radiation compared with conventional catheter ablation.

The study will randomise 210 patients undergoing catheter ablation of supraventricular tachyarrhythmias to either a conventional ablation technique or one guided by the EnSite NavX™ mapping system. The primary end-point is the reduction of the radiation dose to the patient. Secondary end-points include procedural success, reduction of the radiation dose to the operator, and a cost-effectiveness analysis. In a subgroup of patients, we will also evaluate the radiobiological effectiveness of dose reduction by assessing acute chromosomal DNA damage in peripheral blood lymphocytes.

NO-PARTY will determine whether radiofrequency catheter ablation of supraventricular tachyarrhythmias guided by the EnSite NavX™ mapping system is a suitable and cost-effective approach to achieve a clinically significant reduction in ionising radiation exposure for both patient and operator.

The objective of this study was to detect structural and functional changes in the left and right ventricles in obese Egyptian adolescents.

Anthropometric and echocardiographic parameters, including tissue Doppler imaging, were obtained from 70 obese adolescents with average body mass index of 34 plus or minus 3.8 and compared with 50 age- and sex-matched controls, with a body mass index of 21.6 plus or minus 1.9. Cardiac dimensions, stroke volume, left ventricular and right ventricular systolic and diastolic functions were evaluated. The obese group had a higher end-diastolic septal and posterior wall thickness and left ventricular mass index than the non-obese group. Body mass index, mid-arm and hip circumference values showed significant correlations with these echocardiographic variables. Systolic and diastolic functions of the left ventricle were normal in both groups, although stroke volume was high in the obese group. The right ventricle tissue Doppler parameters were similar in both groups. However, the S wave of the septal/lateral tricuspid valve annulus was reduced in the obese group, but not to the level reflecting systolic dysfunction. This was inversely correlated with hip, waist, and mid-arm circumference. Stepwise multiple regression analysis showed that the mid-arm and hip circumference followed by the body mass index are significant predictors of these early cardiac abnormalities.

Left ventricular hypertrophy is present in obese children, although both systolic and diastolic functions are normal. Tissue Doppler imaging revealed a minor, but still significant, reduction in the right ventricular systolic function. Mid-arm and hip circumference are predictors of left ventricular hypertrophy.

Hyperglycaemia has been identified as a risk factor for adverse outcomes in critically ill patients, including those who have undergone cardiopulmonary bypass. Tight glucose control with insulin therapy has been shown to improve outcomes, but is not common practice for children following cardiopulmonary bypass. We examined the relationship between blood glucose level and systemic and cerebral oxygen transport in a uniform group of neonates after the Norwood procedure.

Systemic oxygen consumption was measured using respiratory mass spectrometry in 17 neonates for 72 hours postoperatively. Cardiac output, systemic and total pulmonary vascular resistances – including the Blalock–Taussig shunt, systemic oxygen delivery and oxygen extraction ratio, as well as arterial lactate and glucose, were measured at 2- to 4-hour intervals. Cerebral oxygen saturation was measured by near-infrared spectroscopy.

Blood glucose levels ranged from 2.8 to 24.6 millimoles per litre. Elevated glucose level showed a significant negative correlation with cardiac output (p = 0.02) and cerebral oxygen saturation (p = 0.03), and a positive correlation with oxygen extraction ratio (p = 0.03). It tended to correlate positively with systemic vascular resistance (p = 0.09) and negatively with oxygen delivery (p = 0.09), but did not correlate with oxygen consumption (p = 0.13).

Hyperglycaemia is negatively associated with systemic haemodynamics, oxygen transport, and cerebral oxygenation status in neonates after the Norwood procedure. Further study is warranted to examine tight glucose control with insulin therapy on postoperative systemic and cerebral oxygen transport and functional outcomes in neonates after cardiopulmonary bypass.

To assess the cardiac catheterisation findings of all children in whom cardiac magnetic resonance imaging found great artery stenosis.

We conducted a retrospective analysis of all 45 consecutive children with congenital cardiac disease who were undergoing cardiac catheterisation for intervention on cardiac magnetic resonance-defined great vessel stenosis, between January, 2006 and August, 2008.

Following cardiac magnetic resonance, 60 significant great vessel stenoses were identified and referred to cardiac catheterisation for intervention. All patients were catheterised within a median and interquartile range of 84 and 4–149 days, respectively, of cardiac magnetic resonance. At cardiac catheterisation, the children were aged 11.5 years – with an interquartile range of 3.8–16.9 years – and weighed 34 kilograms – with an interquartile range of 15–56 kilograms. Comparing cardiac magnetic resonance and cardiac catheterisation findings, 53 (88%) findings were concordant and seven were discordant. In six of seven (86%) discordant observations, cardiac magnetic resonance defined moderate–severe great vessel stenosis – involving three branch pulmonary arteries and three aortas. This was not confirmed by cardiac catheterisation, which revealed mild stenoses and haemodynamic gradients insufficient for intervention. In one patient, a mild, proximal right pulmonary artery narrowing was found at cardiac catheterisation, which was not mentioned in the cardiac magnetic resonance report. There was no difference between discordant and concordant groups on the basis of patient age, weight, interval between cardiac magnetic resonance and cardiac catheterisation, or type of lesion.

Invasive assessment confirmed cardiac magnetic resonance-diagnosed great vessel stenosis in the majority of this cohort. The predominant discordant finding was lower catherisation gradient than predicted by morphologic and functional cardiac magnetic resonance assessment. Flow volume diversion – for example, unilateral pulmonary artery stenosis – and anaesthetic effects may account for some differences. Prospective refinement of cardiac magnetic resonance and interventional data may further improve the validity of non-invasive imaging thresholds for intervention.

Our animal model suggests that quantification of ductal flow from colour Doppler pixels is possible. We aimed to clarify whether this method can be used to determine a clinically significant ductal shunt in children.

We retrospectively quantified ductal flow from saved images from 20 children who had been admitted for device occlusion of patent ductus arteriosus. Colour Doppler images over the main stem of the pulmonary artery were obtained in longitudinal cross-sections. The colour pixel percentages during diastole, representing ductal flow, were correlated with the documented shunt, measured invasively according to Fick's principle.

The ratio of pulmonary to systemic flow correlated best with the sum of the percentages of green colour pixels (r = 0.73, r2 = 0.54, p < 0.001). When the shunt was 1.5:1 or more, 12 out of 13 infants had 50% or more of the region of interest covered with green pixels – sensitivity 92%, specificity 71%. The correlation between ductal diameter and pulmonary-to-systemic flow ratio was less significant (r = 0.6, r2 = 0.37, p < 0.03).

We conclude that clinically significant shunts with pulmonary-to-systemic flow ratio over 1.5 can be diagnosed with this method where neither the size of the patient nor echocardiographic settings seem to be critical. The method could be used to provide an objective indication for ductal closure, but further prospective studies in children are needed to verify the power of the method.

To identify risk factors for loss to cardiology follow-up among children and young adults with congenital heart disease.

We used a matched case-control design. Cases were born before January, 2001 with moderate or complex congenital heart disease and were previously followed up in the paediatric or adult cardiology clinic, but not seen for 3 years or longer. Controls had been seen within 3 years. Controls were matched 3:1 to cases by year of birth and congenital heart disease lesion. Medical records were reviewed for potential risk factors for loss to follow-up. A subset of cases and controls participated in recorded telephone interviews.

A total of 74 cases (66% male) were compared with 222 controls (61% male). A history of missed cardiology appointments was predictive of loss to follow-up for 3 years or longer (odds ratio 13.0, 95% confidence interval 3.3–51.7). Variables protective from loss to follow-up were higher family income (odds ratio 0.87 per $10,000 increase, 0.77–0.98), cardiac catheterisation within 5 years (odds ratio 0.2, 95% confidence interval 0.1–0.6), and chart documentation of the need for cardiology follow-up (odds ratio 0.4, 95% confidence interval 0.2–0.8). Cases lacked awareness of the importance of follow-up and identified primary care physicians as their primary source of information about the heart, rather than cardiologists. Unlike cases, controls had methods to remember appointments.

A history of one or more missed cardiology appointments predicted loss to follow-up for 3 or more years, as did lack of awareness of the need for follow-up. Higher family income, recent catheterisations, and medical record documentation of the need for follow-up were protective.

This study compares the prevalence and perinatal mortality of congenital heart defects on La Réunion with European (EUROCAT) standards.

Data were extracted from a EUROCAT-affiliated congenital malformations registry, covering 88,025 births during the period 2002–2007, on the whole island territory. A total of 512 congenital heart defects were registered, including 424 live births, 18 foetal deaths from 16 weeks of gestation, and 70 terminations of pregnancy. The total prevalence of congenital heart defects was 5.8 per 1000 births and live birth prevalence was 4.8 per 1000. The total prevalence of non-chromosomal congenital heart defects was 5.1 per 1000 births, of which 3% were perinatal deaths, 33.3% prenatally diagnosed, and 11.6% termination of pregnancy. Severe non-chromosomal congenital heart defects – excluding ventricular septal defects, atrial septal defects, and pulmonary valve stenosis – occurred in 2.1 per 1000 births, of which 10.3% were perinatal deaths, 59.1% prenatally diagnosed, and 24.3% termination of pregnancy. Of the severe congenital heart defects, the rates of single ventricle (0.20‰), Ebstein anomaly (0.11‰), common arterial trunk (0.25‰), and atrioventricular septal defect (0.62‰) exceeded averages found in Europe, although coarctation of the aorta was infrequent. Conversely, rates of ventricular septal defects, atrial septal defects, and pulmonary valve stenosis were inferior to European standards. Slightly less than half of the congenital heart defects of chromosomal origin were associated with Down syndrome.

In La Réunion, the total prevalence of congenital heart defects is far inferior to that found in Europe. The difference can be attributable to lower prevalences of mild congenital heart defects.

Little is known about the quality of life, health, family, education, and employment status among adult men with repaired tetralogy of Fallot.

A total of 68 men who underwent repair of tetralogy of Fallot between 1971 and 1991 were studied. Fifty-three patients answered the SF-36 health survey and additional questions regarding offspring, education, and employment status. The men with repaired tetralogy of Fallot were compared with 32 healthy men and 40 women who also underwent repair of tetralogy of Fallot in the same period.

The patients scored lower than healthy men in the SF-36 categories physical functioning, general health, and physical component summary. There were no statistically significant differences in the scores from male and female patients except a lower score in bodily pain among women. Educational level for men operated for tetralogy of Fallot was similar to the general male population, whereas fewer were employed and more were retired, undergoing rehabilitation or receiving social benefits. The reproduction rate was lower compared with the general population (0.65 versus 1.02 children per man) but relatively higher than the rate among women with tetralogy of Fallot (0.88 versus 1.84 children per woman). The risk of having a child with congenital heart disease was 8.3%.

Men operated for tetralogy of Fallot have good quality of life and educational status. They start a family, although their reproduction rate is two-thirds that of the general population. The risk of having a child with congenital heart disease is higher compared with the background population. The overall quality of life is similar for men and women operated for tetralogy of Fallot.

In our recent study, we first reported that mutation in vascular endothelial growth factor-A is associated with bicuspid aortic valve stenosis. However, to date no groups have explored the role of vascular endothelial growth factor-A variations in the aetiology of congenital tricuspid aortic valve stenosis.

We sequenced all eight coding exons and exon–intron boundaries of the vascular endothelial growth factor-A gene in deoxyribonucleic acid samples of a cohort of 32 sporadic patients with tricuspid aortic valve stenosis, 300 normal controls, and 103 disease controls – conotruncal defects – in order to identify sequence variants.

We identified a c.973C > T heterozygous nonsense variation in exon 6 of the vascular endothelial growth factor-A gene in a patient with an isolated tricuspid aortic valve stenosis. The c.973C > T variation, which was absent in all controls, changes a highly conserved arginine at amino acid position 325 to a stop codon (p.Arg325X) and is predicted to produce a truncated protein of 324 amino acid residues. The proband's parents had a normal cardiac phenotype; however, his father was a carrier of the p.Arg325X variation, which indicates that the p.Arg325X variation is inherited and incompletely penetrant.

We report for the first time that the p.Arg325X nonsense variation in the vascular endothelial growth factor-A gene may be associated with congenital tricuspid aortic valve stenosis.