To study the cardiac functions in Down syndrome children who did not have structural cardiac lesion by conventional and tissue Doppler echocardiography.

A total of 85 children with Down syndrome without anatomic heart disease and 50 normal control children were subjected to the assessment of right and left ventricular functions by both two-dimensional and tissue Doppler echocardiography.

Children with Down syndrome had significantly higher left ventricular ejection fraction detected by two-dimensional echocardiography and left ventricular diastolic dysfunction detected by tissue Doppler than observed in the controls. In addition, children with Down syndrome also had right ventricular systolic and diastolic dysfunctions. Children with Down syndrome had significantly higher pulmonary artery systolic pressure than the control children. There was no significant difference in the cardiac functions between children with non-disjunction Down syndrome and those with the translocation type.

Despite an apparently normal heart, children with Down syndrome may have silent disturbed cardiac functions, which may be detected by two-dimensional or tissue Doppler echocardiography. This may have an important clinical implication, especially before involving Down syndrome children in surgery or strenuous exercise.

To describe the current prevalence of paediatric hypertension and the relationships between body mass index, gender, place of residence, and blood pressure in a population of school-aged children from western Romania.

A total of 3626 children from Timisoara and the surrounding regions were examined by medical students from February, 2010 to June, 2011. Children's body mass index was interpreted on the basis of reference guidelines from Centers for Disease Control and Prevention. Blood pressure measurements were taken by the auscultation method. For defining elevated blood pressure, we used the standards published in the Fourth Report.

Overall, the prevalence of overweight was 14% and 11.8% for obesity. Boys had a higher prevalence of both overweight and obesity compared with girls. The prevalence of hypertension on our screening was 9.1% higher in boys and urban residence. The prevalence of pre-hypertension was 6.5%. With regard to body mass index, 21.1% of obese, 12.8% of overweight, and 7.1% of normal weight children presented hypertension. The strongest determinant of hypertension was body mass index percentile. Significant odds ratio was found for obesity (3.93; confidence interval: 3.11–4.95), urban residence (1.68; confidence interval: 1.35–2.1), and male gender (1.34; confidence interval: 1.12–1.6).

The results confirm a worrisome prevalence of overweight and obesity among children in Romania, accompanied by an alarming prevalence of hypertension. Overweight and obesity, male gender, and urban residence were the major contributing factors for the overall high prevalence of hypertension found. Our results point to the urgent need to adopt strategies aimed at preventing hypertension and obesity in children in Romania.

To determine the feasibility and safety of the Conquest Pro wire as an alternative to radiofrequency wire for perforation of atretic pulmonary valve and subsequent balloon dilatation and patent ductus arteriosus stenting in patients with pulmonary atresia with intact ventricular septum.

Radiofrequency valvotomy and balloon dilatation has become the standard of care for pulmonary atresia with intact ventricular septum in many institutions today.

We report eight consecutive patients in whom we used the Conquest Pro coronary guidewire, a stiff wire normally reserved for revascularisation of coronary lesions with chronic total occlusion, for perforation of atretic pulmonary valve and subsequent balloon dilatation, and stenting of the patent ductus arteriosus.

Perforation of atretic pulmonary valve was successful in seven out of eight cases. Radiofrequency valvotomy was employed after failure of perforation by the Conquest Pro wire in one case where the right ventricular outflow tract was broad based and tapered towards the pulmonary valve, and was heavily trabeculated. Failure of the Conquest Pro wire to perforate the pulmonary valve plate was mainly attributed by the failure to engage the wire at the correct position.

The Conquest Pro wire for perforation and subsequent interventions in the more straightforward cases of pulmonary atresia with intact ventricular septum is effective and safe, simplifying the entire procedure. However, the radiofrequency generator and wires remain essential tools in the paediatric interventional catheter laboratory.

Pulmonary atresia and intact ventricular septum is a complex congenital heart disease with great morphological variability. Approximately two-thirds of patients may be suitable for transcatheter pulmonary valvotomy. We reviewed our experience in the use of two different percutaneous approaches to evaluate the impact on fluoroscopy time and morbidity of a new technique to perform transcatheter radiofrequency perforation and valvotomy in newborns with pulmonary atresia and intact ventricular septum.

In all, 31 patients underwent radiofrequency perforation of the pulmonary valve. The first 14 infants were treated using a 5 French Judkins right coronary catheter, which was manoeuvred directly underneath the atretic pulmonary valve (Group A). The others were treated using a telescopic system consisting of Northstar Lumax Flex and White Lumax Guiding Catheters (Cook; Group B). In both groups, after radiofrequency perforation of the pulmonary valve, a 0.014-inch superfloppy guidewire was advanced into the descending aorta and balloon dilations were performed. Required fluoroscopy time was significantly lower in Group B (48.5 ± 28.1 versus 24.9 ± 14.4 minutes, respectively; p < 0.01). A higher incidence of unfavourable events including the need for early surgery was found in Group A.

In our experience, telescopic catheter proved to be a valid option able to decrease the fluoroscopy time of percutaneous radiofrequency perforation of pulmonary valve and consequently patients’ exposure to procedure-related risks.

Providing appropriate care for adults with congenital heart disease requires the evaluation of their current situation. There is limited research in Iran about these patients, particularly in relation to gender differences in the demographic/socio-economic and lifestyle factors, as well as disease parameters.

The sample consisted of 347 congenital heart disease patients in the age group of 18–64 years, including 181 women, assessed by an analytical cross-sectional study. The patients were recruited from the two major heart hospitals in Tehran. Data were collected using questionnaires.

The mean age of the patients was 33.24 years. Women were more often married and more often had offspring than men (p < 0.001). Educational level and annual income were similar between women and men. Unemployment was higher among women (p < 0.001), but financial strain was higher among men (p < 0.001). Smoking, alcohol, and water-pipe use was higher among men than among women (p < 0.001). Cardiac factors, for example number of cardiac defects, were similar among women and men, except that there were more hospitalisations owing to cardiac problems, for example arrhythmia, among men. Disease was diagnosed mostly at the hospital (57.4%). Most medical care was provided by cardiologists (65.1%). Only 50.1% of patients had knowledge about their type of cardiac defect.

Gender differences exist in the socio-economic and lifestyle characteristics of adults with congenital heart disease, in some cases related to the disease severity. Our findings also point to the need for interventions to increase patients’ knowledge about, and use of, healthier lifestyle behaviours, irrespective of gender. Furthermore, providing appropriate jobs, vocational training, and career counselling may help patients to be more productive.

To study coronary artery haemodynamics in adolescents with homozygous familial hypercholesterolaemia and aortic supravalvular stenosis.

Patients diagnosed with familial hypercholesterolaemia who were younger than 16 years and who had undergone transthoracic echocardiography from 2007 to 2010 were included in this study. We included patients with homozygous familial hypercholesterolaemia and aortic supravalvular stenosis and those with heterozygous familial hypercholesterolaemia. All patients underwent stress echocardiography, and left anterior descending coronary artery flow was successfully detected. Coronary flow velocity reserve was calculated as the ratio of hyperaemic mean diastolic flow velocity after injection of adenosine to basal mean diastolic flow velocity. Changes in coronary haemodynamics and the relationship between lipid concentrations were determined.

A total of 11 patients with homozygous familial hypercholesterolaemia were enrolled in this study. Lipid concentrations were measured, and the mean coronary flow velocity reserve was 1.97 plus or minus 0.51. Seven children were included in the group of patients with heterozygous familial hypercholesterolaemia. In these children, the mean coronary flow velocity reserve was 3.08 plus or minus 0.84.

The coronary flow velocity reserve of homozygous familial hypercholesterolaemic patients is lower than that of heterozygous familial hypercholesterolaemic patients, and it is associated with a high concentration of low-density lipoprotein cholesterol. Aortic stenosis and plaques compromised the ostia of the coronary artery and caused increased basal mean diastolic coronary velocity with blunted increase in peak velocity, which decreased the coronary flow velocity reserve.

Shunt thrombosis after implantation of systemic-to-pulmonary shunts in paediatric patients is common. Acetyl salicylic acid is used for anti-thrombotic treatment; however, the effect is rarely monitored, although it is known that the response varies. The aim was to determine the effects of acetyl salicylic acid medication on platelet aggregation in children with systemic-to-pulmonary shunts.

A total of 14 children – median age 12 days; ranging from 3 to 100 days – were included in a prospective observational longitudinal study. All children were treated with oral acetyl salicylic acid (3–5 milligrams per kilogram once daily) after shunt implantation. Acetyl salicylic acid-dependent platelet aggregation in whole blood was analysed with impedance aggregometry (Multiplate®) after addition of arachidonic acid. Analyses were carried out before the primary operation, before and 5 and 24 hours after the first acetyl salicylic acid dose, and after 3–6 months of treatment. The therapeutic range for acetyl salicylic acid was defined as a test result less than 60 units.

Acetyl salicylic acid reduced the arachidonic acid-induced platelet aggregation in all but one patient. Of the patients, 93% were in the therapeutic range 5 hours after acetyl salicylic acid intake, 86% were in the range after 24 hours, and 64% after 3–6 months.

Acetyl salicylic acid reduces platelet aggregation after shunt implantation in paediatric patients, but a considerable percentage of the children are outside the therapeutic range. Monitoring of platelet aggregation has the potential to improve anti-platelet treatment after shunt implantation by identifying children with impaired acetyl salicylic acid response.

Patients with a congenitally corrected transposition of the great arteries show an increasing incidence of cardiac failure with age. In other systemic right ventricles, such as in dextro-transposition after atrial switch, excessive hypertrophy is a potential risk factor for impaired systolic function. In this trial, we sought to compare systemic function and volumes between patients with congenitally corrected transposition and those with dextro-transposition after atrial switch by using cardiac magnetic resonance imaging.

A total of 19 patients (nine male) with congenitally corrected transposition and 31 patients (21 male) with dextro-transposition after atrial switch were studied using a 1.5-Tesla scanner. Cine steady-state free-precession sequences in standard orientations were acquired for volumetric and functional imaging. Patient parameters were compared with those of a group of 25 healthy volunteers. Although patients with congenitally corrected transposition were older, they presented with higher right ventricular ejection fractions (p = 0.04) compared with patients with dextro-transposition. Patients with congenitally corrected transposition showed a weak negative correlation between age at examination and systemic ejection fraction (r = −0.18, p = 0.04) but no correlation between right ventricular myocardial mass index and ejection fraction. There was no significant difference in the right ventricular end-diastolic volumes between both patient groups.

Although patients with congenitally corrected transposition had a longer pressure load of the systemic right ventricle, ventricular function was better compared with that in patients with dextro-transposition after atrial switch. The results suggest that the systemic ventricles might have partly different physiologies. One difference could be the post-operative situation after atrial switch, which results in impaired atrial contribution to ventricular filling.

Identify trends of enrolment and key challenges when recruiting infants with complex cardiac diseases into a multi-centre, randomised, placebo-controlled drug trial and assess the impact of efforts to share successful strategies on enrolment of subjects.

Rates of screening, eligibility, consent, and randomisation were determined for three consecutive periods of time. Sites collectively addressed barriers to recruitment and shared successful strategies resulting in the Inventory of Best Recruiting Practices. Study teams detailed institutional practices of recruitment in post-trial surveys that were compared with strategies of enrolment initially proposed in the Inventory.

The number of screened patients increased by 30% between the Initial Period and the Intermediate Period (p = 0.007), whereas eligibility decreased slightly by 7%. Of those eligible for entry into the study, the rate of consent increased by 42% (p = 0.025) and randomisation increased by 71% (p = 0.10). During the Final Period, after launch of a competing trial, fewer patients were screened (−14%, p = 0.06), consented (−19%, p = 0.12), and randomised (−34%, p = 0.012). Practices of recruitment in the post-trial survey closely mirrored those in the Inventory.

Early identification and sharing of best strategies of recruitment among all recruiting sites can be effective in increasing recruitment of critically ill infants with congenital cardiac disease and possibly other populations. Strategies of recruitment should focus on those that build relationships with families and create partnerships with the medical providers who care for them. Competing studies pose challenges for enrolment in trials, but fostering trusting relationships with families can result in successful enrolment into multiple studies.

Although survival to hospital discharge among children requiring extracorporeal membrane oxygenation support for medical and surgical cardio-circulatory failure has been reported in international registries, extended survival and re-hospitalisation rates have not been well described in the literature.

This is a single-institution, retrospective review of all paediatric patients receiving extracorporeal membrane oxygenation for primary cardiac dysfunction over a 5-year period.

A total of 74 extracorporeal membrane oxygenation runs in 68 patients were identified, with a median follow-up of 5.4 years from hospital discharge. Overall, 66% of patients were decannulated alive and 25 patients (37%) survived to discharge. There were three late deaths at 5 months, 20 months, and 6.8 years from discharge. Of the hospital survivors, 88% required re-hospitalisation, with 63% of re-admissions for cardiac indications. The median number of hospitalisations per patient per year was 0.62, with the first re-admission occurring at a mean time of 9 months after discharge from the index hospitalisation. In all, 38% of patients required further cardiac surgery.

Extended survival rates for paediatric hospital survivors of cardiac extracorporeal membrane oxygenation support for medical and post-surgical indications are encouraging. However, re-hospitalisation within the first year following hospital discharge is common, and many patients require further cardiac surgery. Although re-admission hospital mortality is low, longer-term follow-up of quality-of-life indicators is required.