Despite the increasing utilisation of interventional electrophysiology in adults and older children with arrhythmias, there are few data reflecting the safety and efficacy of this procedure in the age group under 2 years.

We describe our experience in assessing the efficacy and safety with this group of children.

We undertook a retrospective review of all infants under 2 years of age who underwent an interventional electrophysiology procedure between 1995 and 2009 to determine indications, procedural details, short- and long-term success, and complication rate.

A total of 23 interventional electrophysiology procedures were performed in 17 patients initially under 2 years of age. Of these, three patients had congenital heart disease. The most common indication was arrhythmia resistant to pharmacological agents (59%), with the remaining cases being arrhythmia complicated by cardiovascular instability (41%). There was initial success in 15 patients after the first procedure, with early recurrence in four. Following six repeat procedures, there was long-term success in 15 patients (88%), with three repeat procedures being performed after 2 years of age. There was one non-procedural death related to persisting arrhythmia. There were three minor complications. In one patient, cryotherapy was used successfully.

The interventional electrophysiology procedure is a viable therapeutic option in infants under 2 years with arrhythmia resistant to other conventional medical management.

To evaluate the growth of children after repair of Tetralogy of Fallot, as well as the influence of residual lesions and socio-economic status.

A total of 17 children, including 10 boys with a median age of 16 months at surgery, were enrolled in a retrospective cohort, in a tertiary care university hospital. Anthropometric (as z-scores), clinical, nutritional, and social data were collected.

Weight-for-age and weight-for-height z-scores decreased pre-operatively and recovered post-operatively in almost all patients, most markedly weight for age. Weight-for-height z-scores improved, but were still lower than birth values in the long term. Long-term height-for-age z-scores were higher than those at birth, surgery, and 3 months post-operatively. Most patients showed catch-up growth for height for age (70%), weight for age (82%), and weight for height (70%). Post-operative residual lesions (76%) influenced weight-for-age z-scores. Despite the fact that most patients (70%) were from low-income families, energy intake was above the estimated requirement for age and gender in all but one patient. There was no influence of socio-economic status on pre- and post-operative growth. Bone age was delayed and long-term-predicted height was within mid-parental height limits in 16 children (93%).

Children submitted to Tetralogy of Fallot repair had pre-operative acute growth restriction and showed post-operative catch-up growth for weight and height. Acute growth restriction could still be present in the long term.

This study compares the developmental and functional outcomes at school entry between boys and girls born with a congenital cardiac defect who required early surgical correction.

A prospective cohort of 94 children, including 49 percent boys, were followed up to 5 years of age and assessed for developmental progress. Developmental measures included Wechsler Preschool and Primary Scale of Intelligence – cognitive; Peabody Picture Vocabulary Test – receptive language; Peabody Developmental Motor Scale – motor; and Child Behaviour Checklist – behaviour. Measures of function included the Vineland Adaptive Behavior Scale and Functional Independence Measure for Children (WeeFIM).

The mean scores of the boys on the WeeFIM subscales, such as self-care, mobility, cognition, were significantly lower than that of the girls. There was a trend for a greater proportion of boys to have abnormalities on neurological examination (boys 37.5 percent abnormal, girls 19.5 percent abnormal). Verbal, performance, and full scale Intellectual Quotients were 5–7 points lower in boys but did not reach significance (full scale Intellectual Quotient: boys 87.7 plus or minus 22.2; girls 93.9 plus or minus 19.3). Boys were more likely to have fine motor delays (50 percent, 82.7 plus or minus 16.5) compared with girls (28.2 percent, 87.0 plus or minus 15.8). There were no gender differences in receptive language or behavioural difficulties.

Boys born with congenital heart disease requiring early surgical repair appear to be at enhanced risk for neuromotor impairments and activity limitations. Findings support gender differences in the pathogenesis of early brain injury following hypoxic–ischaemic insults. This has implications for neuroprotective strategies to prevent brain injury.

Poor weight gain is common in infants after Stage I Norwood operation and can negatively impact outcomes.

The purpose of this study was to examine the impact of feeding strategy on interstage weight gain.

In a multi-centre study, 158 infants discharged following the Norwood operation were enrolled prospectively. Weight and feeding data were obtained at 2-week intervals. Differences between feeding regimens in average daily weight gain and change in weight-for-age z-score between Stage I discharge and Stage II surgery were examined.

Discharge feeding regimens were oral only in 52%, oral with tube supplementation in 33%, and by nasogastric/gastrostomy tube only in 15%. There were significant differences in the average daily interstage weight gain among the feeding groups – oral only 25.0 grams per day, oral/tube 21.4 grams per day, and tube only 22.3 grams per day – p = 0.019. Tube-only-fed infants were significantly older at Stage II (p = 0.004) and had a significantly greater change in weight-for-age z-score (p = 0.007). The overall rate of weight gain was 16–32 grams per day, similar to infant norms. The rate of weight gain declined over time, with earlier decline observed for oral- and oral/tube-fed infants (less than 15 grams per day at 5.4 months) in comparison with tube-only-fed infants (less than 15 grams per day at 8.6 months).

Following Stage I Norwood, infants discharged on oral feeding had better average daily weight gain than infants with tube-assisted feeding. The overall weight gain was within the normal limits in all feeding groups, but the rate of weight gain decreased over time, with an earlier decline in infants fed orally.

To describe the experiences of children and adolescents being screened positive for hypertrophic cardiomyopathy and how this impacts their daily life.

Hypertrophic cardiomyopathy is a hereditary disease and the most common medical cause of sudden death in childhood and adolescence. This is the reason for recommending screening in children with an affected first-degree relative. A diagnosis of hypertrophic cardiomyopathy implies lifestyle modifications, restrictions that may bring profound changes to the daily life of the affected individual.

This is a descriptive qualitative interview study.

We interviewed 13 asymptomatic children or adolescents diagnosed with hypertrophic cardiomyopathy through family screening 12–24 months after the diagnosis. Analysis was conducted with qualitative content analysis.

Children described an involuntary change, which affected their daily life with limitations and restrictions in life, both in the individual and social context. Lifestyle recommendations had the most severe impact on daily life and affected their social context. They tried to navigate in a world with new references, and after reorientation they felt hope and had faith in the future.

Children diagnosed with hypertrophic cardiomyopathy through family screening went through an involuntary change resulting in limitations and restrictions in life. This study indicates that there is a need for support and that healthcare professionals have to consider the specific needs in these families. Our findings thus give guidance in how best to improve support to the patients and their family. Diagnosis in asymptomatic children should be accompanied by ideally multi-professional follow-up, focusing not only on medical issues.

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.

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.

In spite of tremendous progress in the medical and surgical treatment of children with congenital heart disease and dilated cardiomyopathy achieved during the past few decades, for some children a heart transplant remains the only option. Clinically relevant benefits of intracoronary injection of autologous stem cells on cardiac function and remodelling have been demonstrated in adult patients with acute myocardial infarction. Experience with autologous stem cell therapy in children with severe congenital or acquired pump failure is limited to a small number of case reports.

Between 2006 and 2010, nine severely ill children were treated with intracoronary infusion of autologous bone marrow-derived mononuclear cells as part of a compassionate therapy in our centre. No procedure-related unexpected adverse events occurred. There was one patient on extracorporeal membrane oxygenation who died of haemorrhage unrelated to the procedure; three patients proceeded to heart transplantation once a donor heart became available. The other five patients showed an improvement with respect to New York Heart Association classification (greater than or equal to 1), brain natriuretic peptide serum levels, and ejection fraction.

Similar to adults, intracoronary injection of autologous bone marrow cell is technically feasible and safe for children. On the basis of our data, we propose to perform a pilot study for children with congestive heart failure, to formally assess the efficacy of intracoronary autologous bone marrow cell therapy.

To analyse the outcome of children with left ventricular dysfunction placed on Highly Active Antiretroviral Therapy.

This study is a retrospective review of records of Human Immunodeficiency Virus-positive children with left ventricular dysfunction. Demographic data were collected. Left ventricular fractional shortening, CD4 percentage, viral load, and nutritional status were compared before and during antiretroviral therapy.

We reviewed the records of 34 Human Immunodeficiency Virus-positive children with left ventricular dysfunction. In all, 18 patients received antiretroviral therapy (group one) and 16 were antiretroviral therapy naive (group two). The median age of group one at initial visit was 94 months, with a male-to-female ratio of 1:1. Of those, 17 children showed improved left ventricular function on treatment, with an increase in fractional shortening (median: 17–33.5%; p less than 0.0001). There was no significant statistical difference between the groups regarding initial fractional shortening. In group one, the CD4 percentage improved (median: 12% to 30.5%; p less than 0.0001), with viral load suppression (median: 24,900 copies per millilitre to less than 25 copies per millilitre; p less than 0.0001). There was weight gain in group one (median z-score: −1.70 to −1.32; p equal to 0.0083). Proper statistical analysis in group two was not possible because of poor follow-up of patients.

The findings are in keeping with other reports that have shown improvement in left ventricular function in patients with Human Immunodeficiency Virus-associated cardiomyopathy treated with Highly Active Antiretroviral Therapy. Recovery of myocardial function is associated with improvement in immunological and nutritional statuses.

To describe the gross motor development of infants who had undergone cardiac surgery in the neonatal or early infant period.

Gross motor performance was assessed when infants were 4, 8, 12, and 16 months of age with the Alberta Infant Motor Scale. This scale is a discriminative gross motor outcome measure that may be used to assess infants from birth to independent walking. Infants were videotaped during the assessment and were later evaluated by a senior paediatric physiotherapist who was blinded to each infant's medical history, including previous clinical assessments. Demographic, diagnostic, surgical, critical care, and medical variables were considered with respect to gross motor outcomes.

A total of 50 infants who underwent elective or emergency cardiac surgery at less than or up to 8 weeks of age, between July 2006 and January 2008, were recruited to this study and were assessed at 4 months of age. Approximately, 92%, 84%, and 94% of study participants returned for assessment at 8, 12, and 16 months of age, respectively. Study participants had delayed gross motor development across all study time points; 62% of study participants did not have typical gross motor development during the first year of life. Hospital length of stay was associated with gross motor outcome across infancy.

Active gross motor surveillance of all infants undergoing early cardiac surgery is recommended. Further studies of larger congenital heart disease samples are required, as are longitudinal studies that determine the significance of these findings at school age and beyond.

Syncope is common in children and adolescents and most commonly represents neurocardiogenic syncope. No information has been reported regarding the effect of syncope on health-related quality of life in children.

This was a retrospective cohort study of patients seen in the Heart Institute Syncope Clinic at Cincinnati Children's Hospital Medical Center between July, 2009 and June, 2010. Health-related quality of life was assessed using the PedsQL™ tool. PedsQL™ scores were compared with both healthy historical controls and historical controls with chronic illnesses.

A total of 106 patients were included for analysis. In all, 90% were Caucasian and 63% were girls. The median age was 15.1 years (8.2–21.6). Compared with healthy controls, patients had lower PedsQL™ scores: Total score (75.2 versus 83.8, p < 0.0001); Physical Health Summary (78.8 versus 87.5, p < 0.0001); Psychosocial Health Summary (73.9 versus 81.9, p < 0.001), Emotional Functioning (68.9 versus 79.3, p < 0.001); and School Functioning (66.4 versus 81.1, p < 0.001). No difference was seen in Social Functioning (86.2 versus 85.2, p = 0.81). Patients also had lower PedsQL™ Total scores than patients with diabetes mellitus (p < 0.0001) and similar scores to patients with asthma, end-stage renal disease, obesity, and structural heart disease.

Children with syncope, although typically benign in aetiology, can have low health-related quality of life.

To evaluate the results of balloon dilatation of stenotic homografts in children, adolescents, and young adults and to identify factors that might influence or predict the effect of the dilatation.

Homografts are widely used in congenital cardiac surgery; however, the longevity remains a problem mostly because of stenosis in the homograft. The effect of treatment by balloon dilatation is unclear.

In a retrospective study, the effect of balloon dilatation was determined by the percentage of reduction of the peak systolic pressure gradient over the homograft during catheterisation and the postponement of re-intervention or replacement of the homograft in months. Successful dilatations – defined in this study as a reduction of more than 33% and postponement of more than 18 months – were compared with unsuccessful dilatations in search of factors influencing or predicting the results.

The mean reduction of the peak systolic pressure gradient was 30% in 40 procedures. Re-intervention or replacement of the homograft was postponed by a mean of 19 months. In all, 14 balloon dilatations (35%) were successful; the mean reduction was 49% and the mean postponement was 34 months. The time since homograft implantation, the presence of calcification, the homograft/balloon ratio, and the pressure applied during dilatation all tended to correlate with outcome, but were not statistically significant.

Balloon dilatation is able to reduce the peak systolic pressure gradient over homografts in a subgroup of patients and can be of clinical significance to postpone re-intervention or pulmonary valve replacement.

To describe endovascular stent placement using partially covered stents to preserve flow in head and neck vessels.

Endovascular stent placement has become established as a first-line therapy for native coarctation of the aorta or re-coarctation in older children and adults. Increasingly covered stents are becoming the preferred option over bare-metal stents because of the perceived lower risk of aneurysm formation. Open-cell bare-metal stents are chosen when there is a high likelihood of jailing a head and neck vessel. Here we describe partial uncovering of a covered stent before implantation to allow flow through the uncovered portion of the stent to the branch vessel but preserve the covering over the majority of the remaining stent.

We describe two cases with aortic arch hypoplasia and re-coarctation, both of which required two partially uncovered stents for a satisfactory result.

Endovascular stent placement is becoming the preferred option in the management of coarctation of the aorta in older children and adults. Strategies to deal with transverse arch hypoplasia and multiple levels of aortic arch obstruction frequently involving branch vessels or aneurysms need to be considered before these procedures are embarked upon. Partially uncovering stents may afford more protection than using bare-metal stents in the transverse and distal arch while preserving flow in head and neck branches, and is a technically straightforward procedure.