It is unclear whether cryoablation or radiofrequency ablation offers better value for treating atrioventricular nodal re-entrant tachycardia in children. We aimed to compare the value of these procedures for treating atrioventricular nodal re-entrant tachycardia in children, with value being outcomes relative to costs.

We performed a retrospective cohort study of all atrioventricular nodal re-entrant tachycardia ablations for children (age⩽18 years) from July, 2009 to June, 2011 at our institution. Costs included fixed costs, miscellaneous hospital costs, and labour costs, and key outcomes were acute and long-term success (6 months) of the ablations. We conducted T-tests and regression analyses to investigate the associations between the ablation procedure type and the cost and success of the ablations.

Of 96 unique cases performed by three paediatric electrophysiologists, 48 were cryoablation only, 42 radiofrequency ablation only, and six were a combination. Acute success was 100% for the cryoablation only and radiofrequency ablation only cases and 83% for the combination cases. There were no notable adverse events. The average total cost was $9636 for cryoablation cases, $9708 for radiofrequency ablation cases, and $10,967 for combination cases (p=0.51 for cryoablation only versus radiofrequency ablation only). The long-term success rate was 79.1% for cryoablation only, 92.8% for radiofrequency ablation only, and 66.7% for the combination (p=0.01 for cryoablation only versus radiofrequency ablation only), but long-term success varied notably by provider.

Cryoablation and radiofrequency ablation offer similar value in the short term for the treatment of atrioventricular nodal re-entrant tachycardia in children. Differences in long-term success may vary substantially by physician, and thus may lead to differences in long-term value.

P-wave dispersion is a new and simple electrocardiographic marker that has been reported to be associated with inhomogeneous and discontinuous propagation of sinus impulses. In the present study, we evaluated P-wave dispersion in obese adolescents and investigated the relationship between P-wave dispersion, cardiovascular risk factors, and echocardiographic parameters.

We carried out a case–control study comparing 150 obese adolescents and 50 healthy controls. Maximum and minimum P-wave durations were measured using a 12-lead surface electrocardiogram, and P-wave dispersion was calculated as the difference between these two measures. Echocardiographic examination was also performed for each subject. Multivariate linear regression analysis with stepwise variable selection was used to evaluate parameters associated with increased P-wave dispersion in obese subjects.

Maximum P-wave duration and P-wave dispersion were significantly higher in obese adolescents than control subjects (143±19 ms versus 117±20 ms and 49±15 ms versus 29±9 ms, p<0.0001 for both). P-wave dispersion was positively correlated with body mass index, waist and hip circumferences, systolic and diastolic blood pressures, total cholesterol, serum levels of low-density lipoprotein cholesterol, triglycerides, glucose, and insulin, homoeostasis model assessment for insulin resistance score, left ventricular mass, and left atrial dimension. P-wave dispersion was negatively correlated with high-density lipoprotein cholesterol levels. By multiple stepwise regression analysis, left atrial dimension (β: 0.252, p=0.008) and homoeostasis model assessment for insulin resistance (β: 0.205; p=0.009) were independently associated with increased P-wave dispersion in obese adolescents.

Insulin resistance is a significant, independent predictor of P-wave dispersion in obese adolescents.

The growing number of women with CHD presents unique challenges, including those related to pregnancy, which can lead to significant morbidity and mortality. We sought to evaluate the perception of paediatric cardiologists towards the reproductive health of women with CHD.

Paediatric cardiologists in the United States of America were invited to participate in a cross-sectional, anonymous survey. Information solicited included knowledge of contraceptive methods, experience caring for pregnant women with CHD, and referral patterns including the utilisation of high-risk obstetric and adult CHD specialists.

A total of 110 cardiologists responded – 90% with an academic affiliation and 70% with ⩾10 years’ clinical experience. Although 95% reported an understanding of available contraceptive options, 32% did not feel comfortable recommending birth control. Pregnant women with CHD were seen by 83% of responders, and 37% of the responders reported a low level of comfort in doing so. Among all respondents, 73% indicated that they would refer a pregnant CHD patient to a high-risk obstetrician and 60% to an adult CHD specialist – almost all respondents would not transfer care to a non-adult CHD cardiologist. Among paediatric cardiologists, 81% indicated that they would resume their patient’s care following delivery.

Our results illustrate a gap in what physicians feel should be done and the care that they feel comfortable providing pregnant women with CHD. As this population continues to grow, training adult CHD cardiologists with specific skills in reproductive health in women with CHD is the first step to closing the care gap that exists in the management of such patients.

The aim of this study was to investigate the psychosocial needs of both parents of children with CHD (aged 0–18 years) and patients themselves (aged 8–18 years) in the week before cardiac surgery or a catheter intervention.

Eligible participants included all consecutive patients (0–18 years) scheduled to undergo cardiac surgery or a catheter intervention in our hospital between March, 2012 and July, 2013. Psychosocial needs were assessed using a disease-specific questionnaire designed for this study, consisting of a 83-item parent version and a 59-item child version (for children ⩾8 years), each covering five domains: physical/medical, emotional, social, educational/occupational, and health behaviour; two items assessed from whom and in what format psychosocial care was preferred. Quality of life was also assessed.

If parents/patients reported a need for psychosocial care, referral to adequate mental health-care professionals was arranged.

More than 40% of participating parents and >50% of participating children reported a need for psychosocial care on each of the five domains. Needs for psychosocial care for parents themselves were highest for those with children aged 0–12 years. Parents and patients report clear preferences when asked from whom and in what format they would like to receive psychosocial care. Quality of life was relatively high for both parents and patients. Psychosocial care interventions in our hospital increased significantly after the implementation of this study.

Results show that psychosocial care is rated as (very) important by both parents and children during an extremely stressful period of their life.

The present study aims to identify the role of inflammatory markers such as C-reactive protein, interleukin-6, and fractalkine in CHD-associated pulmonary hypertension in children.

This is a prospective review of 37 children with CHD-related pulmonary hypertension, 21 children with congenital heart defects, and 22 healthy children.

Serum C-reactive protein and interleukin-6 levels were significantly higher in the children with CHD-related pulmonary hypertension (respectively, p=0.049 and 0.026). Serum C-reactive protein concentrations correlated negatively with ejection fraction (r=−0.609, p=0.001) and fractional shortening (r=−0.452, p=0.007) in the pulmonary hypertension group. Serum fractalkine concentrations correlated negatively with ejection fraction (r=−0.522, p=0.002) and fractional shortening (r=−0.395, p=0.021) in the children with pulmonary hypertension. Serum interleukin-6 concentrations also correlated negatively with Qs (r=−0.572, p=0.021), positively with Rs (r=0.774, p=0.001), and positively with pulmonary wedge pressure (r=0.796, p=0.006) in the pulmonary hypertension group. A cut-off value of 2.2 IU/L for C-reactive protein was able to predict pulmonary hypertension with 77.5% sensitivity and 77.5% specificity. When the cut-off point for interleukin-6 concentration was 57.5 pg/ml, pulmonary hypertension could be predicted with 80% sensitivity and 75% specificity.

Inflammation is associated with the pathophysiology of pulmonary hypertension. The inflammatory markers C-reactive protein and interleukin-6 may have a role in the clinical evaluation of paediatric pulmonary hypertension related to CHDs.

Knowledge regarding factors that influence deviation of the QRS axis is important when seeking to differentiate between physiological and pathological changes. We hypothesised that, in contrast to those patients with an atrioventricular septal defect and common atrioventricular junction permitting only atrial shunting, those associated with ventricular shunting would show no relationship between the positions of the papillary muscles and the degree of the leftward deviation of the QRS axis.

We compared the positions of endocardial origin of the papillary muscles, and the frontal plane QRS axis, in patients with atrioventricular septal defects and common atrioventricular junction permitting exclusively atrial as opposed to atrial and ventricular shunting.

We analysed 18 patients with atrial and ventricular shunting and 23 patients with exclusively atrial shunting. The correlation coefficient between the ratio of distances of the papillary muscles from the mid-septum and the amount of leftward deviation in the frontal plane QRS axis was 0.1 (p=0.4) in those with ventricular shunting and 0.26 (p=0.01) in those with exclusively atrial shunting.

In contrast to patients with the so-called primum form of atrioventricular septal defect, in whom the locations of the papillary muscles correlate with the degree of QRS axis, such relationships are lacking in patients with defects permitting both atrial and ventricular shunting. It may be, therefore, that the presence of ventricular shunting and/or their younger age causes pressure overload, which negates the leftward QRS forces caused by the abnormally positioned papillary muscles.

In 2007, a partnership was initiated between a small-volume paediatric cardiac surgery unit located in Las Palmas de Gran Canaria, Spain, and a large-volume cardiac surgery unit located in Milan, Italy. The main goal of this partnership was to provide surgical treatment to children with CHD in the Canary Islands.

An operative algorithm for performing surgery in elective, urgent, and emergency cases was adopted by the this joint programme. Demographic and in-hospital variables were collected from the medical records of all the patients who had undergone surgical intervention for CHD from January, 2009 to March, 2013. Data were introduced into the congenital database of the European Congenital Heart Surgeons Association Congenital Database and the database was interrogated.

In total, 65 surgical mission trips were performed during the period of this study. The European Congenital Heart Surgeons Association Congenital Database documented 214 total patients with a mean age at operation of 36.45 months, 316 procedures in total with 198 cardiopulmonary bypass cases, 46 non-cardiopulmonary bypass cases, 26 cardiovascular cases without cardiopulmonary bypass, 22 miscellaneous other types of cases, 16 interventional cardiology cases, six thoracic cases, one non-cardiac, non-thoracic procedure on a cardiac patient with cardiac anaesthesia, and one extracorporeal membrane oxygenation case. The 30-day mortality was 6.07% (13 patients).

A joint programme between a small-volume centre and a large-volume centre may represent a valid and reproducible model for safe paediatric cardiac surgery in the context of a peripheral region.

Adults with tetralogy of Fallot experience atrial tachyarrhythmias; however, there are a few data on the outcomes of radiofrequency ablation. We examined the characteristics, outcome, and predictors of recurrence of atrial tachyarrhythmias after radiofrequency ablation in tetralogy of Fallot patients.

Retrospective data were collected from 2004 to 2013. In total, 56 ablations were performed on 37 patients. We identified two matched controls per case: patients with tetralogy of Fallot but no radiofrequency ablation and not known to have atrial tachyarrhythmias. Acute success was 98%. Left atrial arrhythmias increased in frequency over time. The mean follow-up was 41 months; 78% were arrhythmia-free. Number of cardiac surgeries, age, and presence of atrial fibrillation were predictors of recurrence. Lone cavo-tricuspid isthmus-dependent flutter reduced the likelihood of atrial fibrillation. Right and left atria in patients with tetralogy of Fallot were larger in ablated cases than controls. NYHA class was worse in cases and improved after ablation; baseline status predicted death. Of matched non-ablated controls, a number of them had atrial fibrillation. These patients were excluded from the case–control study but analysed separately. Most of them had died during follow-up, whereas of the matched ablated cases all were alive and the majority in sinus rhythm.

Patients with tetralogy of Fallot and atrial tachyarrhythmias have more dilated atria than those without atrial tachyarrhythmias. Radiofrequency ablation improves functional status. Left atrial ablation is more commonly required with repeat procedures. There is a high prevalence of atrial tachyarrhythmias, particularly atrial fibrillation, in patients with tetralogy of Fallot; early radiofrequency ablation may have a protective effect against this.

Infective endocarditis has been reported post Melody percutaneous pulmonary valve implant; the incidence and risk factors, however, remain poorly defined. We identified four cases of endocarditis from our first 25 Melody implants. Our aim was to examine these cases in the context of postulated risk factors and directly compare endocarditis rates with local surgical valves.

We conducted a retrospective review of patients post Melody percutaneous pulmonary valve implant in New Zealand (October, 2009–May, 2015) and also reviewed the incidence of endocarditis in New Zealand among patients who have undergone surgical pulmonary valve implants.

In total, 25 patients underwent Melody implantation at a median age of 18 years. At a median follow-up of 2.9 years, most were well with low valve gradient (median 27 mmHg) and only mild regurgitation. Two patients presented with life-threatening endocarditis and obstructive vegetations at 14 and 26 months post implant, respectively. Two additional patients presented with subacute endocarditis at 5.5 years post implant. From 2009 to May, 2015, 178 surgical pulmonic bioprostheses, largely Hancock valves and homografts, were used at our institution. At a median follow-up of 2.9 years, four patients (2%) had developed endocarditis in this group compared with 4/25 (16%) in the Melody group (p=0.0089). Three surgical valves have been replaced.

The Melody valve offers a good alternative to surgical conduit replacement in selected patients. Many patients have excellent outcomes in the medium term. Endocarditis, however, can occur and if associated with obstruction can be life threatening. The risk for endocarditis in the Melody group was higher in comparison with that in a contemporaneous surgical pulmonary implant cohort.

Absent arterial valve leaflets are rare anomalies. On the basis of our understanding of the normal development of the arterial valves, we draw inferences that might offer clues to their morphogenesis.

We describe the findings from four human fetal autopsies with so-called “absent” arterial valvar leaflets. We then make inferences relative to these finding on the basis of our current understanding of normal development, the latter obtained by analysis of episcopic data sets from a large series of mouse embryos.

The fetuses had died between 12 and 15 weeks of gestation. In two cases, we found absence of the leaflets of the pulmonary valve, with patency of the arterial duct, but otherwise normal hearts. In a third case, there was absence of the leaflets of both arterial valves, along with a perimembranous ventricular septal defect and a “window-type” arterial duct. This fetus had a completely muscular subaortic infundibulum. The last fetus had a pulmonary dominant common arterial trunk, with absence of the truncal valvar leaflets, but again with a muscular subtruncal infundibulum. Findings from the analysis of the mouse embryos reveal that the arterial valvar leaflets are formed from the distal outflow cushions, but that the cushions have a separate function in septating the arterial roots and the proximal outflow tracts.

When interpreting the fetal findings in the light of development, we conclude that there had been normal fusion of the major outflow cushions, but failure in excavation of their peripheral margins in three of the cases. In the fourth case, however, the cushions had not only failed to excavate but had also failed to separate the arterial roots.

The aim of the present study was to determine the role of toxic elements and trace elements in the pathogenesis of conotruncal heart defects by measuring their concentrations in the first meconium specimens of the affected newborns.

Concentrations of lead, cadmium, iron, zinc, and copper were measured in 1st-day meconium specimens that were collected from 60 newborns with conotruncal heart defects (Group I) and 72 healthy newborns (Group II).

The newborns with conotruncal defects and the healthy newborns had statistically similar demographic and clinical characteristics. When compared with healthy newborns, mean concentrations of lead, cadmium, iron, zinc, and copper were significantly higher in newborns with conotruncal heart defects (p=0.001 for each). In total, 51 newborns with conotruncal heart defects had normal karyotype. These newborns had significantly higher concentrations of lead, cadmium, iron, zinc, and copper when compared with healthy newborns. There were significant and positive correlations between the concentrations of lead and cadmium (r=0.618, p=0.001), lead and iron (r=0.368, p=0.001), lead and zinc (r=0.245, p=0.005), lead and copper (r=0.291, p=0.001), cadmium and iron (r=0.485, p=0.001), cadmium and zinc (r=0.386, p=0.001), and cadmium and copper (r=0.329, p=0.001).

Toxic metals and trace elements may disturb DNA repair mechanisms by impairing DNA methylation profiles, and thus have a role in the pathogenesis of conotruncal heart defects.

Use of hydrocortisone to treat refractory haemodynamic instability in patients recovering from surgery for congenital heart disease is common practice at many centres. We aimed to determine whether there is a relationship between total serum cortisol concentrations and haemodynamic response to this therapy.

We retrospectively reviewed patients <21 years who underwent cardiac surgery from 2011 to 2013, received hydrocortisone within 72 hours postoperatively, and had total serum cortisol measurements contemporaneous with its administration. Favourable responders were defined as patients in whom, at 24 hours after hydrocortisone initiation, either (1) systolic blood pressure was increased or unchanged and vasoactive-inotrope score was decreased or (2) systolic blood pressure increased by ⩾10% of baseline and vasoactive-inotrope score was unchanged. Variables were compared using t-tests or Mann–Whitney U tests as appropriate.

In total, 24 patients were reviewed, with a median age of 1.4 months and range of 0.1–232 months. Among them, 14 (58%) patients responded favourably to hydrocortisone. At 24 hours, the median change in vasoactive-inotrope score was −18% in favourable responders and +31% in those who did not respond favourably, p=0.001. The mean pre-hydrocortisone total serum cortisol in favourable responders was 17.4±10.9 µg/dl compared with 46.1±44.7 µg/dl in those who did not respond favourably, p=0.03.

Total serum cortisol obtained before initiation of hydrocortisone was significantly lower in patients who responded favourably to this therapy. Total serum cortisol may therefore be helpful in identifying children recovering from cardiac surgery who may or may not haemodynamically improve with hydrocortisone.

Oxidative stress refers to an imbalance between reactive oxidative species and antioxidants. In this case-controlled, prospective, observational study, we investigated the total oxidant status, total antioxidant status, oxidative stress index, and albumin and C-reactive protein levels of children with cyanotic and acyanotic congenital heart diseases who had undergone on-pump cardiac surgery.

The study groups consisted of 60 patients with congenital heart disease, who were operated under cardiopulmonary bypass, and a control group of 30 healthy individuals. The patients were classified into two groups. Among them, one was a patient group that consisted of 30 patients with acyanotic congenital heart disease and the other group consisted of 30 patients with cyanotic congenital heart disease. In the patient groups, blood samples were collected before surgery and at one and 24 hours following surgery. In control groups, blood samples were collected once during hospital admission.

No statistically significant differences were found between the groups in terms of baseline total oxidant status, total antioxidant status, and oxidative stress index values. Regarding the postoperative first-hour and 24-hour total oxidant status and total antioxidant status levels as well as oxidative stress index values, there were no significant differences between the groups, except for an increase in total antioxidant status levels (p=0.002) 24 hours after surgery in cyanotic patients.

There was no difference between oxidative stress status of cyanotic and acyanotic congenital heart disease patients and healthy individuals. Oxidative stress status of cyanotic and acyanotic patients does not change after cardiac surgery under cardiopulmonary bypass.

As survival of children with CHD needing surgery has improved significantly, the need for follow-up in terms of health-related quality of life has become increasingly important. In this study, we sought to describe health-related quality of life in children with CHD in relation to cardiac surgery.

A retrospective Swedish National Registry for Congenital Heart Disease survey measured using DISABKIDS chronic generic measure-short version included 337 children (age 9–17 years; 39% girls). The majority (n=319, 95%) of children had a biventricular heart, whereas the remaining had a univentricular heart. Cardiac surgery was performed in 197 (58%) children. Health-related quality of life was expressed as total score (100 highest) and given as medians and 10–90th percentiles.

The overall total score was 95 (88–100). Children with a biventricular heart who had undergone three or more surgeries (n=31; 9%) had the lowest total score of 81 (61–97; p<0.001). Children with two or more surgeries and those with univentricular heart were classified in NYHA II more frequently than children with one or no cardiac surgery (p=0.005 and <0.001, respectively). Children with three or more surgeries and those with univentricular heart needed more help at school (p<0.001). Compared with children with other chronic diseases, children with CHD had a high total score except for children with three or more surgeries who had comparable total scores with children with other chronic diseases.

Children with three or more cardiac surgeries and those with a univentricular heart appear to have lower health-related quality of life, cognitive ability, and NYHA classification.

The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD.

A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention.

The need for urgent intervention and/or PGE1 infusion within the first 48 hours of life was 47% (n=507/1080): 72% (n=248) for CHD at risk for a Rashkind procedure, 77% (n=72) for CHD with ductal-dependent pulmonary flow, 13% (n=22) for CHD with potentially ductal-dependent pulmonary flow, 94% (n=62) for CHD with ductal-dependent systemic flow, 29% (n=88) for CHD with potentially ductal-dependant systemic flow, 50% (n=4) for total anomalous pulmonary venous connection, and 17% (n=1) for CHD with atrio-ventricular block. In all, 34% of the patients received PGE1 infusion and 21.4% underwent urgent catheter-based or surgical interventions; 10% of patients without anticipated risk (n=10) underwent an early intervention; 6.7% (n=73) of the patients died; and 55% (n=589) had an intervention before discharge from hospital.

Half of the neonates with foetal CHD benefited from an urgent intervention or PGE1 infusion at birth. We recommend scheduled delivery and in utero transfer for transposition of the great arteries, double-outlet right ventricle with sub-pulmonary ventricular septal defect, total anomalous pulmonary venous connection, CHD with atrio-ventricular block with heart rate <50, all ductal-dependant lesions, and CHD with potentially ductal-dependant systemic flow.

The spatial peaks QRS-T angle accurately distinguishes children with hypertrophic cardiomyopathy from their healthy counterparts. The spatial peaks QRS-T angle is also useful in risk stratification for ventricular arrhythmias. We hypothesised that the spatial peaks QRS-T angle would be useful for the prediction of ventricular arrhythmias in hypertrophic cardiomyopathy patients under 23 years of age.

Corrected QT interval and spatial peaks QRS-T angles were retrospectively assessed in 133 paediatric hypertrophic cardiomyopathy patients (12.4±6.6 years) with versus without ventricular arrhythmias of 30 seconds or longer. Significance, positive/negative predictive values, and odds ratios were calculated based on receiver operating characteristic curve cut-off values.

In total, 10 patients with ventricular arrhythmias were identified. Although the corrected QT interval did not differentiate those with versus without ventricular arrhythmias, the spatial peaks QRS-T angle did (151.4±19.0 versus 116.8±42.6 degrees, respectively, p<0.001). At an optimal cut-off value (124.1 degrees), the positive and negative predictive values of the spatial peaks QRS-T angle were 15.4 and 100.0%, respectively, with an odds ratio of 25.9 (95% CI 1.5–452.2).

In children with hypertrophic cardiomyopathy, the spatial peaks QRS-T angle is associated with ventricular arrhythmia burden with high negative predictive value and odds ratio.