To categorise records according to primary cardiac diagnosis in the United Kingdom Central Cardiac Audit Database in order to add this information to a risk adjustment model for paediatric cardiac surgery.

Codes from the International Paediatric Congenital Cardiac Code were mapped to recognisable primary cardiac diagnosis groupings, allocated using a hierarchy and less refined diagnosis groups, based on the number of functional ventricles and presence of aortic obstruction.

A National Clinical Audit Database.

Children undergoing cardiac interventions: the proportions for each diagnosis scheme are presented for 13,551 first patient surgical episodes since 2004.

In Scheme 1, the most prevalent diagnoses nationally were ventricular septal defect (13%), patent ductus arteriosus (10.4%), and tetralogy of Fallot (9.5%). In Scheme 2, the prevalence of a biventricular heart without aortic obstruction was 64.2% and with aortic obstruction was 14.1%; the prevalence of a functionally univentricular heart without aortic obstruction was 4.3% and with aortic obstruction was 4.7%; the prevalence of unknown (ambiguous) number of ventricles was 8.4%; and the prevalence of acquired heart disease only was 2.2%. Diagnostic groups added to procedural information: of the 17% of all operations classed as “not a specific procedure”, 97.1% had a diagnosis identified in Scheme 1 and 97.2% in Scheme 2.

Diagnostic information adds to surgical procedural data when the complexity of case mix is analysed in a national database. These diagnostic categorisation schemes may be used for future investigation of the frequency of conditions and evaluation of long-term outcome over a series of procedures.

The purpose of the study was to determine the prevalence of coronary artery anomalies and to demonstrate in which cases multidetector computed tomography has an additional clinical value compared with the conventional angiography.

A total of 2375 multidetector computed tomography studies were retrospectively reviewed to determine the dominance of the coronary artery anomalies. The classification of coronary artery anomalies was made according to anatomical criteria – origin, course, intrinsic anatomy, and termination – and clinical relevance – benign versus malignant.

The coronary artery system was right dominant in 83.99%, left dominant in 8.0%, and co-dominant in 9.01% of the cases. The incidence of the origin and/or course anomalies was 1.76%, that of fistulas was 0.42%, and that of myocardial bridges was 10.82%. Multidetector computed tomography was performed after conventional angiography in 23 cases and it provided additional information regarding its origin and proximal course, as well as its relationship with the aortic root and main pulmonary trunk in 100% of the cases; eight malignant cases were found. In addition, in all of (100%) the six cases with coronary artery fistulas, conventional angiography failed to detect their terminations, which were clearly depicted by multidetector computed tomography.

Multidetector computed tomographic angiography is superior to conventional angiography in delineating the ostial origin and proximal course of anomalous coronary arteries. Furthermore, it reveals the exact relationship of anomalous coronary arteries with the aorta and the pulmonary artery. Anomalies of the intrinsic anatomy and the termination of coronary arteries are also better visualised with multidetector computed tomography.

On recognising poor growth following neonatal palliation with a systemic-to-pulmonary shunt, we sought to determine how patient- and procedure-related factors impact growth, paying attention to the role of the primary cardiologist in this process.

In a retrospective review, neonates (133 patients) receiving modified systemic-to-pulmonary artery shunts from 2002 to 2009 were studied and outpatient visits were reviewed. Patients with single- and two-ventricle circulations after shunt takedown were compared using weight-for-age z-score.

Single-ventricle patients had a higher weight-for-age z-score at neonatal surgery than two-ventricle patients (−0.4 ± 1.0 compared with −1.2 ± 0.9, with p < 0.001), but they had a greater drop in the weight-for-age z-score to the first outpatient visit (−1.1 ± 0.7 compared with −0.8 ± 0.7, with p = 0.02). After the first outpatient visit, the weight-for-age z-score was not significantly different between single-ventricle and two-ventricle patients. From multivariate analysis, a lower number of nutritional interventions by cardiologists was significantly associated with poor growth (p = 0.03). Poor growth was not associated with race, use of feeding tube, exclusive formula use, or proximity to surgical centre.

The significant drop in the weight-for-age z-score from neonatal surgery to first outpatient visit suggests that these patients may receive inadequate nutrition. The poorest performers received the least number of outpatient changes to their diet. This finding underscores the critical role of the primary cardiologist in optimising weight gain through adjustments in nutrition.

To investigate the impact of flat-detector computed tomography on the clinical assessment of patients with cavopulmonary connections, and to evaluate the obtained diagnostic accuracy and supplementary information, as well as the value of overlaid three-dimensional reconstructions on fluoroscopic images during catheter-based interventions.

We analysed 31 consecutive patients retrospectively in whom flat-detector computed tomography was used to visualise the cavopulmonary connection. We investigated patients with cavopulmonary connections either early post-operatively (first group), before converting to a total cavopulmonary connection (second group), and patients with failing total cavopulmonary connection (third group). Flat-detector computed tomography based on a single rotational angiography was used to create a three-dimensional vascular model. The clinical value of flat-detector computed tomography was evaluated using standard categories of diagnostic utility. Used contrast volume and radiation exposure were quantified.

Within 18 months, flat-detector computed tomography was performed in 31 cases with cavopulmonary connections. The median age was 1.9 years (range 0.3–43 years). In the first group, we found anomalies in 4 out of 8 cases, which led to therapeutic or prophylactic procedures; in the second and third groups, we performed interventions in 14 out of 23 cases. The overall clinical value was always rated superior to conventional biplane angiography. The median dose area product was 91.8 microgray square metres (range 33.0–679.3 microgray square metres). The required contrast medium was 2.08 millilitres per kilogram (range 0.66–4.7 millilitres per kilogram).

Flat-detector computed tomography improves the diagnostic accuracy in cavopulmonary connections and provides additional diagnostic information, which may lead to therapeutic or prophylactic procedures. Overlaid three-dimensional images on fluoroscopy facilitate and provide security for interventions.

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.

To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly.

Data on long-term outcome of children with Ebstein's anomaly are scarce.

Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention.

A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1–16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%.

In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

We sought to describe the evaluation, treatment, and follow-up of adolescents who presented to a single institution with chest pain and an elevated troponin I value in the absence of typical symptoms of pericarditis or myocarditis.

We performed a retrospective review of patients in the age group of 10–18 years of age with no history of significant heart disease admitted to our institution from 2000 to 2010 after presenting with chest pain and an elevated troponin I value.

A total of 16 patients were identified with a median age of 16.5 years (range 11.2–17.8 years). Of these 13 (81%) were male and 10 (63%) showed evidence of localised ST elevations on electrocardiogram. The median peak troponin I level was 17.8 nanograms per millilitre (range 0.89–227, normal less than 0.4). There were eight patients (50%) with a diagnosis of coronary vasospasm, three patients (20%) with atypical myopericarditis, one patient with coronary anomaly, one patient with hypercoagulable disorder, and one patient with prolonged supraventricular tachycardia. In two patients, no definitive diagnosis was made. There was one patient who needed catheter-based intervention, which involved stenting of a coronary artery after a procedure-related complication.

In our cohort of adolescents without history of significant cardiac disease, chest pain and elevated troponin I levels were attributed to a variety of causes. Although coronary vasospasm and atypical myopericarditis were seen most commonly, coronary anomaly was identified in one case. Magnetic resonance imaging proved a useful diagnostic tool to assess coronary artery anatomy and myocardial changes suggestive of myocarditis. On the basis of these results and a review of the literature, a general evaluation algorithm is presented.

The objectives of this study were to compare behaviour problems and competencies, at home and school, in 7-year-old children with congenital heart disease with a sibling control group, to examine the prospective determinants of outcome from infancy, and to explore whether any gains were maintained in our sub-group of children who had participated in a previous trial of psychological interventions in infancy.

A total of 40 children who had undergone surgery to correct or palliate a significant congenital heart defect in infancy were compared (Child Behavior Checklist) with a nearest-age sibling control group (18 participants). Comparisons were made between sub-groups of children and families who had and had not participated in an early intervention trial.

Problems with attention, thought and social problems, and limitations in activity and school competencies, were found in comparison with siblings. Teacher reports were consistent with parents, although problems were of a lower magnitude. Disease, surgical, and neurodevelopmental functioning in infancy were related to competence outcomes but not behaviour problems. The latter were mediated by family and maternal mental health profiles from infancy. Limited, but encouraging, gains were maintained in the sub-group that had participated in the early intervention programme.

The present study is strengthened by its longitudinal design, use of teacher informants, and sibling control group. The patterns of problems and limitations discerned, and differential determinants thereof, have clear implications for interventions. We consider these in the light of our previously reported intervention trial with this sample and current outcomes at the 7-year follow-up.

Foetal aortic valvuloplasty has been proposed as a strategy to improve left heart growth and function in foetuses with severe aortic stenosis at risk of progression to hypoplastic left heart syndrome. We report our experience with this intervention.

Between 2005 and 2010, five foetuses with aortic stenosis and at risk of progression to hypoplastic left heart syndrome underwent ultrasound-guided percutaneous foetal aortic valvuloplasty. There were no associated maternal complications or foetal demise. In one case, the pregnancy was terminated a couple of weeks after the intervention, one foetus evolved to hypoplastic left heart syndrome, and three did not.

Foetal aortic valvuloplasty seems to be a safe and feasible procedure. It has been reported that it has the potential to prevent progression to hypoplastic left heart syndrome in selected foetuses with severe aortic stenosis. Further investigation regarding physiological and clinical aspects of this disease both prenatally and postnatally will probably allow to improve therapeutic strategies and clinical outcome.

Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. So far, the presence of endothelial dysfunction in patients with no coronary lesions has not been demonstrated. Peripheral arterial tonometry (Endo-PAT) measures the microvascular function in response to local ischaemia and has been validated in adult population, but its use in children is scarce.

To evaluate endothelial dysfunction in children as a long-term complication after Kawasaki disease using Endo-PAT.

We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed for >5 years, with no coronary lesions, or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically accessed. Endo-PAT was performed to determine reactive hyperaemia index and augmentation index.

A total of 35 individuals (21 males, age 21 ± 6 years) were evaluated (group 1: 19; controls: 16). Kawasaki disease patients presented significant lower reactive hyperaemia index (1.68 ± 0.49 versus 2.31 ± 0.53; p = 0.001). Augmentation index was similar in both groups (−10 ± 7 versus −11 ± 5; p > 0.005). Most patients with Kawasaki disease disclosed endothelial dysfunction (68%) compared with only 12% in controls.

Endo-PAT is feasible and reproducible in the child population. Endothelial dysfunction is a frequent long-term complication in patients after Kawasaki disease with normal appearing coronary arteries. However, these results need validation in a larger population.

The aim of our study was to compare the blood levels of adhesion molecules in children with different heart diseases and pulmonary flow rates.

In this study, we evaluated the levels of soluble intercellular adhesion molecule-1 and soluble vascular cellular adhesion molecule-1 in blood samples of 65 children with different congenital heart diseases. The patients were divided into four groups according to their pulmonary blood flow. The first group had increased pulmonary blood flow with pulmonary hypertension and left-to-right shunt. The second group had increased pulmonary blood flow without pulmonary hypertension and left-to-right shunt. The third group had decreased pulmonary blood flow with cyanotic congenital heart disease and the fourth group had normal pulmonary blood flow with left ventricle outflow tract obstruction and aortic stenosis.

The highest soluble intercellular and vascular cellular adhesion molecule-1 levels with the mean values of 420.2 nanograms per millilitre and 1382.1 nanograms per millilitre, respectively, were measured in the first group and the lowest levels with the mean values of 104.4 and 358.6 nanograms per millilitre, respectively, were measured in the fourth group. The highest pulmonary blood pressure levels were found in the first group.

Endothelial activity is influenced not only by left-to-right shunt with pulmonary hypertension, but also by decreased pulmonary blood flow in cyanotic heart diseases. Adhesion molecules are valuable markers of endothelial activity in congenital heart diseases, and they are influenced by pulmonary blood flow rate.

To compare potential risk factors for complications and recurrence after radiofrequency catheter ablation in symptomatic atrioventricular reentrant tachycardia in children and adolescents.

We retrospectively reviewed the data of 213 consecutive patients with symptomatic atrioventricular reentrant tachycardia who underwent both electrophysiological study and radiofrequency catheter ablation, divided these patients into two groups, children (age <12 years) and adolescents (12 ≤ age < 18 years), and compared the location of the accessory pathway, success rate, recurrence rate, complications, presence of congenital heart disease, presence of intermittent ventricular pre-excitation, and presence of Wolff–Parkinson–White syndrome in the two groups.

The position of the accessory pathway was mostly right sided in children (61.3%) and left sided in adolescents (61.5%). Children had significantly more congenital heart disease than adolescents (6.4% versus 0.8%). Univariate analysis showed children or adolescents with right-sided accessory pathways to be 6.84 times and those with accessory pathways on both sides of the septum 25 times more likely to relapse than those with a single accessory pathway. Multivariate analysis indicated that children or adolescents with two accessory pathways were six times, and those with intermittent ventricular pre-excitation nine times more at risk of relapsing following radiofrequency ablation than those with single accessory pathways. All five complications occurred in children.

The findings suggest that the position and number of accessory pathways and presence of intermittent ventricular pre-excitation are related to risks of recurrence of atrioventricular reentrant tachycardia in children and adolescents.

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.

Chest pain is a common presenting complaint to paediatrics, paediatric cardiology, and paediatric emergency departments. In this study, we prospectively evaluated clinical characteristics and causes of chest pain in children referred to our paediatric cardiology unit.

A total of 380 children were included. Associated symptoms and past and family histories were evaluated. All patients underwent physical examination. The following studies were performed: complete blood count in all patients; fasting lipid profiles in overweight and obese children and children with a family history of premature cardiovascular disease; and electrocardiogram, chest X-ray, and echocardiogram in all patients. If necessary, 24-hour electrocardiogram monitoring or exercise stress tests were performed. Patients with a history of positive psychological findings were evaluated by a child psychiatrist.

The most common causes of chest pain were musculoskeletal disorders (37.1%), idiopathic chest pain (29.2%), and miscellaneous disorders, for example precordial catch syndrome (15%), respectively. Only 1 of 380 (0.3%) patients had chest pain due to a cardiac disorder. Electrocardiograms were abnormal in 4 of 380 (1.1%) patients. A total of 9 of 380 patients (2.3%) had dyslipidaemia.

Although a paediatric cardiology referral may provide reassurance to the primary care and emergency department physicians, our results show that cardiac aetiologies for paediatric chest pain are very rare. We think that many patients in our study were adequately evaluated only by careful history, and physical examination. Therefore, we suggest that it may not be necessary to use echocardiogram in the routine evaluation of children with chest pain.