The benefits of early enteral feeding in neonates are well known and yet the optimal pre-operative nutrition of prostaglandin-dependent infants with congenital cardiac disease remains ill-defined. This survey delineates international nutritional practices and trends with this population.

Paediatric practitioners responded to an Internet-based survey that explored assessment of feeding practices, criteria for feeding readiness, medication dosing, concurrent feeding with umbilical catheters, and the observed incidence of feeding intolerance. Documented nutritional strategies were not correlated with patient outcomes.

A total of 200 caregivers responded to the survey. Fewer United States caregivers (56%) reported routine pre-operative enteral feeding in prostaglandin-dependent infants when compared with caregivers outside the United States of America (93%). Of those respondents willing to feed, approximately two-thirds did not base their decision on the ductal flow direction. Numerous and heterogeneous parameters were reported to assess feeding readiness. Many caregivers report scepticism with regard to enteral feeding safety in neonates with an umbilical artery catheter, and to a lesser extent in the presence of an umbilical venous catheter. In summary, there is a prevailing lack of consensus regarding pre-operative enteral nutrition to prostaglandin-dependent neonates.

This survey demonstrates noticeable variations in pre-operative nutritional practices between providers from around the world. Arguments that support or refute this practice have little support in the medical literature. Future studies should aim to demonstrate the safety of such practice and compare the outcomes of prostaglandin-dependent neonates who were pre-operatively enterally fed with those who were not.

Interventional closure of patent arterial duct has become an accepted alternative to surgical closure. Clinical trial with “Nit-Occlud® PDA-R”.

To assess the safety and efficacy of the device, we performed a prospective clinical study between June, 2009 and December, 2010 in La Paz, Bolivia. In all, 29 – 22 female patients and 7 male patients – out of 59 patients were selected on the basis of inclusion criteria. The procedures were performed under sedation at an age and weight of 5.7 years and 22.7 kilograms, respectively, with 4–6 French arterial sheaths and 5–7 French venous sheaths. The minimal diameter of the duct was 3.5 millimetres. The procedure, fluoroscopy, and hospitalisation times were 96.4 minutes (55 to 145), 13.1 minutes (3 to 25.2), and 24 hours, respectively. The “Nit-Occlud® PDA-R” was successfully deployed in all patients. Immediate, 24-hour, 1-, 3-, and 6-month closure rates were 65.5%, 79.3%, 96.5%, and 100%, respectively. The systolic pulmonary pressure diminished from 37 millimetres of mercury (21 to 57) before the intervention to 31 millimetres of mercury (21 to 45) after the intervention. No early or late embolisation, haemolysis, left pulmonary artery, or descending aorta obstruction occurred.

We conclude that the “Nit-Occlud® PDA-R” device is safe and effective in closing patent arterial duct up to a diameter of 8 millimetres.

Complications at the vascular access sites are among the most common adverse events in congenital cardiac catheterization. The use of small-gauge catheters may reduce these events; however, other factors can contribute to the development of vascular complications.

To determine factors associated with the development of vascular access complications in children undergoing congenital cardiac catheterization.

We performed a prospective study of 403 patients who underwent diagnostic (62.5%) or interventional (37.5%) cardiac catheterization over a period of 6 months, and analysed the vascular complications during and immediately after the procedure.

The most common access-related adverse event was transient loss of pulsation (17.6%). Other less common access-related adverse events included subcutaneous haematoma (2%), bleeding (3%), vessel tear (0.2%), and vein thrombosis (0.2%). Patients who had no access-related adverse events had significantly higher age and body weight compared with those who had one or more access problems. Among 81 patients who had vascular access established in unplanned access sites, 30 patients (37%) had lost pulsations. Among the 322 patients who had vascular access established in planned access sites, however, only 41 patients had lost pulsation (13%). In addition, patients who had lost pulsations had significantly longer puncture time compared to those who had normal pulsations (p value 0.01).

Factors other than sheath size can contribute to access-related adverse events in children undergoing cardiac catheterization. Obtaining vascular access in unplanned access sites and longer puncture times increases the incidence of lost pulsations after catheterization. Younger age and smaller body weight are also associated with significant increase in access-related adverse events.

Cross-sectional two-dimensional echocardiography can establish correct diagnosis in all types of atrial communications; however, real-time three-dimensional reconstruction provides additional value to the surgeon and interventionist for better understanding of spatial intracardiac morphology.

To assess the reference values of M-mode echocardiographic measurements in healthy newborns according to body weight.

From January, 2008 to June, 2008, a total of 250 – 130 female and 120 male – term healthy newborns were included in the study. M-mode echocardiographic measurements were recorded by a paediatric cardiologist in accordance with the American Society of Echocardiography recommendations. Right ventricular anterior wall end-diastolic thickness, right ventricular end-diastolic diameter, interventricular septum end-diastolic thickness, left ventricular end-diastolic diameter, left ventricular posterior wall end-diastolic thickness, interventricular septum end-systolic thickness, left ventricular end-systolic diameter, left ventricular posterior wall end-systolic thickness, aortic root dimension, left atrium dimension, left ventricular ejection, and shortening fraction were measured.

The values of echocardiographic measurements revealed a good correlation with body weight. When body weight increased, the measured values also increased in parallel. The measured values were not influenced by gender. Ejection and shortening fraction parameters did not change with body weight and gender.

In this study, normative values related to body weight for cardiac chambers and wall thickness were determined in healthy newborns. These reference values can be used to evaluate whether newborns have normal or abnormal echocardiographic measurement.

Atomoxetine is a central norepinephrine reuptake inhibitor used to treat attention deficit/hyperactivity disorder. The effects of atomoxetine on cardiovascular functions and QT dispersion in children with attention deficit/hyperactivity disorder have not been previously reported. The aim of this study was to analyse cardiovascular functions and QT dispersion on the surface electrocardiogram of children with attention deficit/hyperactivity disorder during atomoxetine therapy.

A total of 40 children – with a mean age of 8.6 plus or minus 2.3 years and a median age of 11 years; ranged from 8 to 14 years – with attention deficit/hyperactivity disorder – with six girls and 34 boys – were included in the study. We recorded the mean systolic and diastolic blood pressure, heart rate, corrected QT interval, QT dispersion, and left ventricular systolic functions at baseline and 5 weeks after atomoxetine therapy.

Atomoxetine decreased baseline mean systolic and diastolic blood pressure; baseline mean heart rate decreased; and baseline mean corrected QT interval and QT dispersion mildly increased. Atomoxetine decreased baseline mean ejection fraction and baseline mean shortening fraction.

The results of our study suggest that atomoxetine does not cause clinically significant alterations in QT dispersion, systolic and diastolic blood pressure, heart rate, corrected QT interval, and left ventricular systolic functions during short-term treatment in children with attention deficit/hyperactivity disorder.

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.

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.

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.

The incidence of Myocardial Infarction (MI) in patients under the age of 30 has been rarely addressed. Moreover, it is not understood why these patients develop symptomatic Coronary Artery Disease (CAD) at such an early age. Traditional risk factor assessment has not been successful in identifying these patients before they present with MI.

Retrospective, single cohort, observational study of 14,704 cardiac catheterizations performed in a community hospital between January 2006–January 2010 identified 12 cases age <30 with MI secondary to a fixed atherosclerotic lesion requiring angioplasty and stenting. The angiograms and charts were reviewed to assess the incidence and frequency of traditional risk factors such as smoking, dyslipidemia and diabetes and family history.

All the patients had single vessel disease. Many of the patients were noted to have traditional CAD risk factors. 2 patients had an intervention and then months later sustained another acute MI secondary to a new culprit lesion despite aggressive risk factor modification.

Evaluating patients for premature CAD by screening for traditional risk factors has not effectively identified at risk patients prior to presentation with MI. There is a role for studies evaluating new and novel risk factors and imaging modalities so that these patients can be identified prior to experiencing MI.

Familial Mediterranean fever may carry a potential for cardiovascular disorders because of sustained inflammation during its course; however, there has been a limited number of studies investigating the cardiac functions in children. The aim of this study was to assess both ventricular diastolic functions using conventional echocardiography and tissue Doppler imaging in children with familial Mediterranean fever.

The study population included 25 patients with familial Mediterranean fever – mean age was 11.8 plus or minus 5.30 years – and 23 healthy patients as controls – mean age was 9.88 plus or minus 3.69 years. Both ventricular functions were measured using echocardiography comprising standard M-mode and conventional Doppler and tissue Doppler imaging during an attack-free period.

The conventional echocardiographic parameters with myocardial performance index were in normal ranges and similar in patients with familial Mediterranean fever and controls, with a p-value more than 0.05. However, right ventricular diastolic dysfunction was observed in patients with familial Mediterranean fever documented by tissue Doppler imaging, with a p-value less than 0.05 for E't and A't wave ratio.

Using tissue Doppler imaging, we have demonstrated that although left ventricular functions were comparable in the patients and healthy children, right ventricular diastolic function indices were impaired in patients with familial Mediterranean fever during childhood. Impaired right ventricular diastolic function may be an early manifestation of cardiac involvement in children with familial Mediterranean fever.

Heterotaxy syndrome is caused by left–right asymmetry disturbances and is associated with abnormal lateralisation of the abdominal and thoracic organs. The heart is frequently involved and the severity of the abnormality usually determines the outcome.

We performed a direct sequence analysis of the coding sequence of genes including Zinc Finger Protein of the Cerebellum 3, Left–Right Determination Factor 2, Activin A Receptor Type IIB, and Cryptic in 47 patients with laterality defects and congenital cardiac disease.

Of the 47 patients, 31 (66%) had atrioventricular septal defects, 34 (72%) had abnormal systemic venous return, 25 (53%) had transposed or malposed great arteries, and 20 (43%) had pulmonary venous abnormalities. We identified two novel genetic changes in Zinc Finger Protein of the Cerebellum 3, and these variants were not present in 100 ethnically matched control samples. One previously reported missense mutation in Activin A Receptor Type IIB was identified in two unrelated subjects. The genetic changes identified in this study are all located in conserved regions and are predicted to affect protein function in left–right axis formation and cardiovascular development.

Mutations in Zinc Finger Protein of the Cerebellum 3 and Activin A Receptor Type IIB were identified in 4 of the 47 patients with heterotaxy syndrome for a yield of approximately 8.5%. Our results expand the mutation spectrum of monogenic heterotaxy syndrome with associated cardiac anomalies and suggest that there are other causes of heterotaxy yet to be identified.