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.

Recent changes in surgical education have had an impact on our congenital training programmes. The mandate of the 8-hour workweek, a rapidly expanding knowledge base, and a host of other mandates has had an impact on the readiness of the fellows who are entering congenital programmes. To understand these issues completely, we interviewed the top congenital experts in the United States of America. The purpose of this paper is to share their insight and offer suggestions to address these challenges.

We used a qualitative thematic analysis approach and performed phone interviews with the top five congenital experts in the United States of America.

Experts unanimously felt that duty-hour restrictions have negatively affected congenital training programmes in the following ways: current fellows do not seem as conditioned as fellows in the past, patient handoffs are not consistent with excellent performance, the mentor–mentee relationship has been affected by duty-hour restrictions, and fellows may be less prepared for real-world practice. Three positive themes emerged in response to duty-hour restrictions: fellows appear to be doing less menial task work, fellows are now better rested for learning, and we are attracting more individuals into the speciality. Experts agreed that congenital fellowships should be increased to 2 years. There was support for both the traditional and integrated residency pathways.

We are in a new era of education and must work together to overcome the challenges that have arisen in recent years.

Rheumatic heart disease causes substantial morbidity in children in low-income countries. School-based echocardiographic screening has been suggested as a means to identify children with latent disease; however, little is known about the experience of children and teachers participating in screenings. The aim of our study was to assess students’ and teachers’ experience of school-based echocardiographic screening and identify areas for improvement.

A school-based echocardiographic screening programme was conducted in five schools in Northern Uganda in 2013. After 8 months, an age- and gender-stratified population that included 5% of the participating students and teachers completed a questionnaire via an in-person interview. Responses were reviewed by question and coded to identify key themes.

A total of 255 students (mean 10.7 years; 48% male) and 35 teachers participated in our study. In total, 95% of the students and 100% of the teachers were happy to have participated in the screening; however, students reported feeling scared (35%) and nervous (48%) during the screening process. Programmatic strengths included the following: knowing one’s health status, opportunity to receive treatment, and staff interactions. Although 43% of the patients did not suggest a change with open-ended questioning, concerns regarding privacy, fear of the screening process, and a desire to include others in the community were noted.

School-based echocardiographic rheumatic heart disease screening was well received by students and teachers. Future programmes would likely benefit from improved pre-screening education regarding the screening process and diagnosis of rheumatic heart disease. Furthermore, education of teachers and students could improve screening perception and establish realistic expectations regarding the scope of screening.

National organisations in several countries have recently released more restrictive guidelines for infective endocarditis prophylaxis, including the American Heart Association 2007 guidelines. Initial studies demonstrated no change in infective endocarditis rates over time; however, a recent United Kingdom study suggested an increase; current paediatric trends are unknown.

Children (<18 years) hospitalised with infective endocarditis at 29 centres participating in the Pediatric Health Information Systems Database from 2003 to 2014 were eligible for inclusion. Our primary analysis focussed on infective endocarditis most directly related to the change in guidelines and included community-acquired cases in those >5 years of age. Interrupted time series analysis was used to evaluate rates over time indexed to total hospitalisations.

A total of 841 cases were identified. The median age was 13 years (interquartile range 9–15 years). In the pre-guideline period, there was a slight increase in the rate of infective endocarditis by 0.13 cases/10,000 hospitalisations per semi-annual period. In the post-guideline period, the rate of infective endocarditis increased by 0.12 cases/10,000 hospitalisations per semi-annual period. There was no significant difference in the rate of change in the pre- versus post-guidelines period (p=0.895). Secondary analyses in children >5 years of age with CHD and in children hospitalised with any type of infective endocarditis at any age revealed similar results.

We found no significant change in infective endocarditis hospitalisation rates associated with revised prophylaxis guidelines over 11 years across 29 United States children’s hospitals.

Percutaneous balloon valvuloplasty is the primary treatment for critical pulmonary valve stenosis in neonates. Thus far, a few studies have reported long-term results of this technique in neonatal critical pulmonary valve stenosis.

We carried out a retrospective study of all consecutive newborns with critical pulmonary valve stenosis subjected to percutaneous balloon valvuloplasty at a single centre, between 1994 and 2014, to assess its immediate and long-term safety and efficacy.

A total of 24 neonates presented with critical pulmonary valve stenosis. The mean diameter of the pulmonary annulus was 7 mm (±1.19); 33.3% had a dysplastic pulmonary valve, and 92% were started on prostaglandin E1 treatment. Percutaneous balloon valvuloplasty was performed at a mean age of 4.0±4.3 days using, on average, a balloon-to-pulmonary annulus ratio of 1.18 mm (with a range from 0.9 to 1.43). Immediate success was achieved in 22/24 patients (92%) with a reduction in the pulmonary transvalvular peak gradient (p<0.05) and in the right ventricle/systemic pressure ratio (p<0.05). There was one death (4%) 6 days after the procedure, and 29.2% of them had transient rhythm complications. For a mean follow-up time of 8.4 years, the re-intervention rate was 42.9%. In total, 14 re-interventions were performed in nine neonates, including surgery in six. Freedom from re-intervention was 50% at 8 years and 43% at 10 and 15 years.

This series, to the best of our knowledge, has had the longest follow-up of neonates with critical pulmonary valve stenosis. Percutaneous balloon valvuloplasty is a safe and effective treatment, and in our study 75% of the patients were exclusively treated using this technique.

Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population.

We performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death.

A total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease.

Women with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

Sequential nephron blockade using intravenous chlorothiazide is often used to enhance urine output in patients with inadequate response to loop diuretics. A few data exist to support this practice in critically ill infants.

We included 100 consecutive patients <1 year of age who were administered intravenous chlorothiazide while receiving furosemide therapy in the cardiac ICU in our study. The primary end point was change in urine output 24 hours after chlorothiazide administration, and patients were considered to be responders if an increase in urine output of 0.5 ml/kg/hour was documented. Data on demographic, clinical, fluid intake/output, and furosemide and chlorothiazide dosing were collected. Multivariable regression analyses were performed to determine variables significant for increase in urine output after chlorothiazide administration.

The study population was 48% male, with a mean weight of 4.9±1.8 kg, and 69% had undergone previous cardiovascular surgery. Intravenous chlorothiazide was initiated at 89 days (interquartile range 20–127 days) of life at a dose of 4.6±2.7 mg/kg/day (maximum 12 mg/kg/day). Baseline estimated creatinine clearance was 83±42 ml/minute/1.73 m2. Furosemide dose before chlorothiazide administration was 2.8±1.4 mg/kg/day and 3.3±1.5 mg/kg/day after administration. A total of 43% of patients were categorised as responders, and increase in furosemide dose was the only variable significant for increase in urine output on multivariable analysis (p<0.05). No graphical trends were noted for change in urine output and dose of chlorothiazide.

Sequential nephron blockade with intravenous chlorothiazide was not consistently associated with improved urine output in critically ill infants.

Systemic hypertension is increasingly recognised in premature infants. There is limited evidence regarding treatment, and most published treatment recommendations are based solely on expert opinions.

We identified all infants born ⩽32 weeks of gestation and ⩽1500 g birth weight discharged from one of 348 neonatal ICUs managed by the Pediatrix Medical Group between 1997 and 2013. We defined antihypertensive drugs as vasodilators, angiotensin-converting enzyme inhibitors, β receptor blockers, calcium channel blockers, and central α2 receptor agonists. We compared characteristics between infants who were treated with at least one antihypertensive drug during their initial hospitalisation and infants who were not prescribed antihypertensive drugs using Wilcoxon’s ranked sum test or Pearson’s χ2-test.

We identified 2504/119,360 (2.1%) infants who required at least one antihypertensive drug. The median postnatal age of first exposure was 48 days (25th, 75th percentile 15, 86), and the median length of therapy was 6 days (1, 16). Hydralazine was the most commonly prescribed antihypertensive with 1280/2504 (51.1%) treated infants exposed to the drug. More than two antihypertensive drugs were administered in 582/2504 (23.2%) infants, and 199/2097 (9.5%) of the treated infants were discharged home on antihypertensive therapy. Infants who received antihypertensive drugs were of lower gestational age (p<0.001) and birth weight (p<0.001) compared with infants not prescribed antihypertensive drugs.

Our study is the largest to describe current antihypertensive drug exposure in a cohort of exclusively premature infants born ⩽32 weeks of gestation. We found wide variations in practice for treating hypertension in premature infants.

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.

The aim of the present study was to explore the predictive value of red cell distribution width as a means to differentiate between neurally mediated syncope and arrhythmic syncope in children.

Patients were divided into a neurally mediated syncope group (n=72) and an arrhythmic syncope group (n=21) on the basis of clinical history, results of the head-up tilt test, electrocardiography, and 24-hour ambulatory electrocardiography. As controls, we recruited 55 healthy children. Red cell distribution width was determined for children in all groups. A receiver operating characteristic curve was drawn to study the predictive effect of red cell distribution width to differentiate between neurally mediated syncope and arrhythmic syncope.

Red cell distribution width was significantly higher in children with neurally mediated syncope than in children with arrhythmic syncope and the control group. A receiver operating characteristic curve on the predictive value of red cell distribution width in differentiating neurally mediated syncope from arrhythmic syncope showed that the area under the curve was 0.841 (95% confidence interval: 0.737–0.945, p<0.05). A red cell distribution width value of 12.8% as the cut-off value yielded a sensitivity of 80.6% and a specificity of 76.2% in discriminating between patients with neurally mediated syncope and arrhythmic syncope.

Red cell distribution width value of ⩾12.8% might be a useful adjunct for primary-care physicians to differentiate neurally mediated syncope from arrhythmic syncope in children.

Right heart function is an important predictor of morbidity and mortality in pulmonary arterial hypertension and many CHD. We investigated whether treatment with the prostacyclin analogue treprostinil could prevent pressure overload-induced right ventricular hypertrophy and failure.

Male Wistar rats were randomised to severe pulmonary trunk banding with a 0.5-mm banding clip (n=41), moderate pulmonary trunk banding with a 0.6-mm banding clip (n=36), or sham procedure (n=10). The banded rats were randomised to 6 weeks of treatment with a moderate dose of treprostinil (300 ng/kg/minute), a high dose of treprostinil (900 ng/kg/minute), or vehicle.

Pulmonary trunk banding effectively induced hypertrophy, dilatation, and decreased right ventricular function. The severely banded animals presented with decompensated heart failure with extracardial manifestations. Treatment with treprostinil neither reduced right ventricular hypertrophy nor improved right ventricular function.

In the pulmonary trunk banding model of pressure overload-induced right ventricular hypertrophy and failure, moderate- and high-dose treatment with treprostinil did not improve right ventricular function neither in compensated nor in decompensated right heart failure.

Most patients born with CHD nowadays reach adulthood, and thus quality of life, life situation, and state of medical care aspects are gaining importance in the current era. The present study aimed to investigate whether patients’ assessment depends on their means of occupation. The findings are expected to be helpful in optimising care and for developing individual treatment plans.

The present study was based on an online survey conducted in cooperation with patient organisations. Participants were recruited from the database of the German National Register for Congenital Heart Defects. In total, 1828 individuals (777 males, 1051 females) took part. Participants were asked to rate aspects such their state of health on a six-tier scale (1=worst specification). Response behaviour was measured against the background of occupational details.

Training for or pursuing a profession was found to be significantly associated with participants’ rating of five of the six examined aspects (p<0.05). Sex seemed to play an important part in four of the six aspects.

An optimal treatment plan for adults with CHD should always consider aspects such as sex and employment status. To work out such an optimal and individual treatment plan for each adult CHD patient, an objective tool to measure patients’ actual CHD-specific knowledge precluding socially accepted response bias would be very useful.

The aim of this study was to share the mid-term results of percutaneous ventricular septal defect closure using Amplatzer Duct Occluder-II in children.

Nowadays, percutaneous ventricular septal defect closure is accepted as an alternative to surgery, but so far no ideal device has been developed for this procedure.

In the study centre, between April, 2011 and October, 2016, the ventricular septal defect of 49 patients was closed percutaneously using the Amplatzer Duct Occluder-II device, and seven of them were <1 year old.

The mean age of patients was 86.8±52.6 months. The mean weight of the patients was 24.3±16 kg. The mean diameter of the defect was 3.7±1.4 mm. Mean fluoroscopy time and total procedure time were 37±19.3 and 74.1±27 minutes, respectively. The types of ventricular septal defects were muscular in six patients, and were perimembranous in the rest of them. We did not face any major complications during the procedure, but one patient was admitted with a complete atrioventricular block in the 6th month of follow-up. The total follow-up period was 66 months.

To our knowledge, our study includes the largest series of paediatric patients whose ventricular septal defect was closed using Amplatzer Duct Occluder-II percutaneously. When the complications during the 66-month follow-up period are taken into consideration, we can state that Amplatzer Duct Occluder-II is a safe and effective device, even in infants aged <1 year.