We aimed to compare the procedural and mid-term performance of a specifically designed self-expanding stent with balloon-expandable stents in patients undergoing hybrid palliation for hypoplastic left heart syndrome and its variants.

The lack of specifically designed stents has led to off-label use of coronary, biliary, or peripheral stents in the neonatal ductus arteriosus. Recently, a self-expanding stent, specifically designed for use in hypoplastic left heart syndrome, has become available.

We carried out a retrospective cohort comparison of 69 neonates who underwent hybrid ductal stenting with balloon-expandable and self-expanding stents from December, 2005 to July, 2014.

In total, 43 balloon-expandable stents were implanted in 41 neonates and more recently 47 self-expanding stents in 28 neonates. In the balloon-expandable stents group, stent-related complications occurred in nine patients (22%), compared with one patient in the self-expanding stent group (4%). During follow-up, percutaneous re-intervention related to the ductal stent was performed in five patients (17%) in the balloon-expandable stent group and seven patients (28%) in self-expanding stents group.

Hybrid ductal stenting with self-expanding stents produced favourable results when compared with the results obtained with balloon-expandable stents. Immediate additional interventions and follow-up re-interventions were similar in both groups with complications more common in those with balloon-expandable stents.

The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management.

Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug.

Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

We carried out a cross-sectional study to assess cognitive function in a sample of adult CHD patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined.

A total of 310 patients participated in this study. Patients were classified into four structural complexity groups – tetralogy of Fallot, transposition of the great arteries, single ventricle, and simple conditions. Each patient underwent neuropsychological assessment to evaluate cognitive function, including memory and executive function, and completed questionnaires to assess depression and anxiety.

Among all, 41% of the sample showed impaired performance (>1.5 SD below the normative mean) on at least three tests of cognitive function compared with established normative data. This was higher than the 8% that was expected in a normal population. The sample exhibited significant deficits in divided attention, motor function, and executive functioning. There was a significant group difference in divided attention (F=5.01, p=0.002) and the mean total composite score (F=5.19, p=0.002) between different structural complexity groups, with the simple group displaying better cognitive function.

The results indicate that many adult CHD patients display impaired cognitive function relative to a healthy population, which differs in relation to disease complexity. These findings may have implications for clinical decision making in this group of patients during childhood. Possible mechanisms underlying these deficits and how they may be reduced or prevented are discussed; however, further work is needed to draw conclusive judgements.

We aimed to investigate whether early postoperative extubation following the Fontan operation is universally feasible and can be used as a management tool in unstable patients.

All patients undergoing the Fontan operation in our centre between 2004 and 2013 (n=253) were analysed. Until 2008, patients were extubated according to standard criteria and comprised group 1. Group 2 included all patients presenting after 2009, when early extubation was always aimed regardless of the haemodynamic status. Patients who exceeded the 75th percentiles for volume requirements and inotrope scores for the respective group were defined as unstable. Comparisons of outcomes between groups and subgroups and analysis of the changes in haemodynamic and treatment parameters with extubation in unstable patients after 2009 were performed.

Compared with group 1, patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.50), and needed less volume (p=0.01). In group 2, the unstable patients were not ventilated for longer durations (p=0.19), but had higher re-intubation rates (p=0.03) than the stable patients. Compared with the unstable patients from group 1, the unstable patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.66), and needed less volume (p=0.006). There was a significant acute and sustained increase in mean arterial pressure with extubation and a parallel reduction in volume requirements and inotrope scores in the unstable patients from group 2.

Timely extubation is universally applicable following the Fontan operation. Early postoperative extubation can be valuable for improving Fontan haemodynamics.

Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.

We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.

A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).

The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

In Kawasaki disease, although coronary dilatation is attributed to vasculitis, the effect of myocardial inflammation is underestimated. Coronary dilatations are determined by Z-scores, which do not take into account dominance. The aim of the present study was to describe the impact of coronary dominance on dilatation in Kawasaki disease.

We performed a retrospective analysis of coronary dilatations according to angiography categorisation of dominance.

Of 28 patients (2.6 [0.2–10.1] years), right dominance was present in 15 patients and left in 13. Early dilatation was present in all patients, of whom 11 were ipsilateral to the dominant segment and 17 contralateral. Ipsilateral dilatations were present at diagnosis (9/11 versus 6/17, p=0.02) compared with contralateral dilatations, which developed 2 weeks after diagnosis (9/11 versus 16/17, p=0.29). Coronary artery Z-scores of patients with contralateral dilatation increased at 2 weeks, before returning to baseline values (2.0±2.2 at diagnosis, 4.1±1.8 at 2 weeks, 1.8±1.2 at 3–6 months, p=0.001), compared with patients with ipsilateral dilatation in whom Z-scores were maximal at diagnosis and remained stable (3.0±0.9, 2.7±1.1 and 2.6±1.5, respectively, p=0.13). Dominant coronary artery Z-scores were higher compared with non-dominant segments at diagnosis (3.0±0.9 versus 1.0±0.8, p<0.001) and at late follow-up (2.6±1.5 versus 0.4±1.4, p=0.002) in patients with ipsilateral dilatation.

Progression of coronary dilatation after diagnosis may be a sign of dilatation secondary to vasculitis, as opposed to regression of Z-scores in ipsilateral dilatations, probably related to physiological vasodilatation in response to carditis. This needs to be validated in larger studies against vasculitic and myocardial inflammatory markers.

The aim of the present study was to determine general paediatrician knowledge, practices, and attitudes towards electrocardiogram (ECG) screening in school athletes during pre-participation screening exam (PPSE).

Paediatricians affiliated with a tertiary children’s hospital completed a survey about ECGs for PPSE.

In total, 205/498 (41%) responded; 92% of the paediatricians did not include an ECG as part of PPSE; 56% were aware of a case in which a student athlete in their own community had died of sudden unexplained death; 4% had an athlete in their practice die. Only 16% of paediatricians perform all 12 American Heart Association recommended elements of the PPSE. If any of these screening elements are abnormal, 69% obtain an ECG, 36% an echocardiogram, and 30% restrict patients from sports activity; 73% of them refer the patient to a cardiologist.

Most of the general paediatricians surveyed did not currently perform ECGs for PPSE. In addition, there was a low rate of adherence to performing the 12 screening elements recommended by the American Heart Association. They have trouble obtaining timely, accurate ECG interpretations, worry about potential unnecessary exercise restrictions, and cost-effectiveness. The practical hurdles to ECG implementation emphasise the need for a fresh look at PPSE, and not just ECG screening. Improvements in ECG performance/interpretation would be necessary for ECGs to be a useful part of PPSE.

Anomalous aortic origin of a coronary artery is a congenital cardiac condition that can be associated with increased risk of sudden death. To date, quality of life and exercise performance have not been evaluated in patients with this condition who do not undergo surgical repair.

We carried out a cross-sectional analysis of patients with unoperated anomalous aortic origin of a coronary artery at our institution from 1 January, 2000 to 31 January, 2016. We prospectively assessed quality of life using standardised questionnaires. Medical records were reviewed for clinical and exercise stress test data. Statistical analyses were performed using Student’s t-tests and Spearman’s correlation coefficients.

In total, 56 families completed the questionnaires. The average age at enrolment was 14.7±6 years. The majority were male (n=44, 78.6%) and had interarterial anomalous right coronary artery (n=38, 67.9%). Patients had normal quality of life on the PedsQL 4.0 Report, Child Health Questionnaire Child Form 87, and SF-36v2. Their parents had normal quality of life on the PedsQL 4.0 Parent Report, but parents of exercise-restricted patients had decreased Physical Functioning, General Health Perception, Emotional Impact on Parent, and Physical Summary scores (p<0.001–0.048) on the Child Health Questionnaire Parent Form 50.

Patients with unoperated anomalous aortic origin of a coronary artery appear to have normal quality of life, but parents of exercise-restricted patients have decreased general health and emotional and physical quality of life scores. Improved counselling of families may be beneficial in this group. Future studies with more patients should evaluate quality of life and exercise performance over time.

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.

Previous studies have identified risk factors for femoral arterial thrombosis after paediatric cardiac catheterisation, but none of them have evaluated the clinical and economic significance of this complication at the population level. Therefore, we examined the national prevalence and economic impact of femoral arterial thrombosis after cardiac catheterisation in children.

Patients⩽18 years of age who underwent cardiac catheterisation were identified in the 2003–2009 Kids’ Inpatient Database. Patients were stratified by age as follows: <1 year of age or 1–18 years of age. The primary outcome was arterial thrombosis of the lower extremity during the same hospitalisation as cardiac catheterisation. Propensity score matching was used to determine the impact of femoral arterial thrombosis on hospital length of stay, cost, and mortality.

Among the 11,497 paediatric cardiac catheterisations identified, 4558 catheterisations (39.6%) were performed in children <1 year of age. This age group experienced a higher prevalence of reported femoral arterial thrombosis, compared with children aged 1–18 years (1.3 versus 0.3%, p<0.001). After matching, femoral arterial thrombosis in children <1 year of age was associated with similar mortality (5.4 versus 1.8%, p=0.28), length of stay (8 versus 5 days, p=0.11), and total hospital cost ($27,135 versus $28,311, p=0.61), compared with absence of thrombosis.

Femoral arterial thrombosis is especially prevalent in children <1 year of age undergoing cardiac catheterisation. Clinicians should be vigilant in monitoring femoral arterial patency in neonates and infants after cardiac catheterisation.

Sickle cell disease is known to cause various degrees of vasculopathy, including impact on heart function. The aims of this single-centre, retrospective study were to assess cardiac chamber size and function and the relationship with haematological indices such as haemoglobin, aspartate aminotransferase, reticulocytosis and bilirubin, lactate dehydrogenase in sickle cell disease.

Right ventricle and left ventricle diastolic diameters, left ventricle mass estimate, left ventricle shortening fraction, myocardial performance index, and an index of myocardial relaxation (E/E’) were calculated and correlated with haematological parameters.

A total of 110 patients (65% haemoglobin SS, 29% haemoglobin SC) were studied at a mean age of 12.14±5.26 years. Right ventricle dilatation and left ventricle dilatation were present in 61.5 and 42.9%, respectively. Left ventricle mass was abnormal in 21.9%; all patients had normal myocardial performance index, 31.4% had abnormal E/E’, and left ventricle shortening fraction was low in 38.1%. Cardiac dilatation was best correlated with haemoglobin, aspartate aminotransferase, reticulocytosis and bilirubin. Best subset regression analysis yielded significant additional prediction for right ventricle or left ventricle dilatation with haemoglobin, bilirubin, and lactate dehydrogenase. Abnormal E/E’ was solely predictable with haemoglobin level. Hydroxyurea-treated patients had improved diastolic function.

Right ventricle dilatation was more prevalent than left ventricle dilatation. The long-term consequences of right ventricular dilatation, clinical consequences, and association with pulmonary vasculopathy need to be further determined.

The surgical treatment for complex forms of d-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction remains controversial. In this study, we describe the classical surgical options – namely, the Rastelli procedure and the “réparation à l’étage ventriculaire” – and present our experience with the modified Nikaidoh procedure with early and short-term follow-up results.

Between 2007 and 2014, four patients with d-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction underwent surgical repair at our institution by means of a modified Nikaidoh procedure.

With a mean follow-up of 4.5 years, survival was 100%, and none of the patients required re-intervention or mechanical circulatory support. There was no recurrence of left ventricular outflow tract obstruction and no aortic valve regurgitation classified as more than mild. Left ventricular function was preserved.

Aortic translocation with the modified Nikaidoh procedure is a safe and effective surgical treatment for certain complex forms of transposition of the great arteries, particularly those associated with ventricular septal defect and left ventricular outflow tract obstruction. It is associated with less need for re-intervention and better morbidity and mortality results in the short- and mid-term follow-up, when compared with the classical alternatives such as the Rastelli procedure.

Physical activity and exercise have important health benefits for children and adolescents with CHD. The objective of this study was to survey the provision of advice and recommendations in United Kingdom paediatric CHD clinics.

A three-page questionnaire was sent out to paediatric cardiac consultants in the United Kingdom, paediatric consultants with expertise in cardiology, and nursing staff (Paediatricians with Expertise in Cardiology Special Interest Group), as well as all members of the British Congenital Cardiovascular Association. The aim of this questionnaire was to determine the extent and scope of current information provision and to assess the importance that clinicians place on this advice.

There were 68 responses in total, and the data showed that, of these, 24 (36%) clinicians had never provided paediatric CHD patients with written advice about exercise. Only 27 (39%) clinicians provided physical activity advice at every appointment. Lack of time during consultation (n=39, 56.9%), lack of training (n=38, 55.2%), and uncertainty about appropriate recommendations (n=38, 55.2%) were identified as the main factors preventing clinicians from providing patients with advice about physical activity.

Although healthcare providers consider physical activity to be very important, the provision of clear, specific advice and recommendations is underutilised; therefore, more education and provision of resources to support the promotion of exercise need to be provided to clinicians and their support teams.

Expert knowledge of cardiac malformations is essential for paediatric cardiologists. Current cardiac morphology fellowship teaching format, content, and nomenclature are left up to the discretion of the individual fellowship programmes. We aimed to assess practices and barriers in morphology education, perceived effectiveness of current curricula, and preferences for a standardised fellow morphology curriculum.

A web-based survey was developed de novo and administered anonymously via e-mail to all paediatric cardiology fellowship programme directors and associate directors in the United States of America; leaders were asked to forward the survey to fellows.

A total of 35 directors from 32 programmes (51%) and 66 fellows responded. Curriculum formats varied: 28 (88%) programmes utilised pathological specimens, 25 (78%) invited outside faculty, and 16 (50%) utilised external conferences. Director nomenclature preferences were split – 6 (19%) Andersonian, 8 (25%) Van Praaghian, and 18 (56%) mixed. Barriers to morphology education included time and inconsistent nomenclature. One-third of directors reported that <90% of recent fellow graduates had adequate abilities to apply segmental anatomy, identify associated cardiac lesions, or communicate complex CHD. More structured teaching, protected time, and specimens were suggestions to improve curricula. Almost 75% would likely adopt/utilise an online morphology curriculum.

Cardiac morphology training varies in content and format among fellowships. Inconsistent nomenclature exists, and inadequate morphology knowledge is perceived to contribute to communication failures, both have potential patient safety implications. There is an educational need for a common, online cardiac morphology curriculum that could allow for fellow assessment of competency and contribute to more standardised communication in the field of paediatric cardiology.