An association of heart disease and its treatment with biliary calculi is popularly accepted. We sought determine the prevalence and risk factors of paediatric gallstone disease in the presence of CHD and analyse the treatment options. We evaluated the role of open-heart surgery in the development of gallstones in patients with CHD.

In a 10-year, retrospective, chart review (2005–2014), patients with CHD and cholelithiasis were identified and reviewed.

In all, 19 of 4729 children with CHD had cholelithiasis (0.4%); eight patients underwent cardiac surgery before diagnosis of cholelithiasis (group 1), whereas 11 of them had not (group 2). The prevalence was 0.3% in group 1 and 0.5% in group 2.

In nine asymptomatic patients, gallstones were found incidentally. Children with cholecystolithiasis (n=17) received ursodeoxycholic acid. A resolution of gallstones was found in four cases; two patients underwent biliary surgery, and the others (15/17) were successfully managed non-operatively.

Despite an accumulation of risk factors, prevalence of gallstones is not as high as expected in children with CHD. Open-heart surgery with a heart–lung machine plays a minor role as an aetiological factor. In about half of the cases, cholelithiasis is an incidental finding and patients stay asymptomatic. Prophylactic administration of ursodeoxycholic acid is not indicated in children undergoing open-heart surgery for CHDs. Biliary surgery is reserved for patients with recurrent symptoms or cholestasis.

In children with CHD, cholelithiasis is a minor and manageable co-morbid condition.

A number of case reports show various outcomes of premature closure of the ductus arteriosus in utero, including persistent pulmonary hypertension of the newborn and fetal or neonatal death; however, no study clarifies the clinical observations that are related to their prognoses. We aimed to clarify the prognostic factors of intrauterine ductal closure by a systematic literature review.

We searched PubMed database (1975–2014) to identify case reports and studies on intrauterine closure of the ductus arteriosus, including maternal, fetal, and neonatal clinical information and their prognoses.

We analysed the data of 116 patients from 39 articles. Of these, 12 (10.3%) died after birth or in utero. Fetal or neonatal death was significantly correlated with fetal hydrops (odds ratio=39.6, 95% confidence interval=4.6–47.8) and complete closure of the ductus arteriosus (odds ratio=5.5, 95% confidence interval=1.2–15.1). Persistent pulmonary hypertension was observed in 33 cases (28.4%), and was also correlated with fetal hydrops (odds ratio=4.2, 95% confidence interval=1.3–4.6) and complete closure of the ductus arteriosus (odds ratio=5.5, 95% confidence interval=1.6–6.0). Interestingly, maternal drug administration was not correlated with the risk of death and persistent pulmonary hypertension.

Fetal hydrops and complete ductal closure are significant risk factors for both death and persistent pulmonary hypertension. Cardiac or neurological prognoses could be favourable if the patients overcome right heart failure during the perinatal period.

“Heterotaxy syndrome”, best segregated as isomerism, is characterised by laterality defects of the thoraco-abdominal organs, causing functional impairment. In particular, the spleen is frequently affected, increasing susceptibility to bacteraemia. This study explored factors that may increase the risk of bacteraemia in patients with isomerism.

We identified patients with CHD and isomerism. Review of outpatient, inpatient, and surgical records was conducted to collect data and determine trends in the cohort. A Cox regression analysis was conducted to determine factors influencing freedom from bacteraemia (Fig 1).

We identified 83 patients with CHD and isomerism – 17 (20%) who had documented episodes of bacteraemia with a total of 21 episodes. A majority (86%) were nosocomial. The median age at the time of bacteraemia was 4 months. Although splenic anatomy did appear to influence the risk of bacteraemia in univariate analysis, this significance was lost with multivariate analysis. None of the other factors was significantly associated in either univariate or multivariate analysis.

Specific factors such as splenic anatomy, atrial appendage isomerism, and antibiotic prophylaxis status are not significantly associated with the risk of bacteraemia in patients with CHD and isomerism. Nosocomial infections represent a majority of bacteraemia in these patients.

Current guidelines recommend that patients with CHD receive age-appropriate counselling on reproduction, pregnancy, and risk of heredity. Our aim was to examine patient knowledge of reproductive health and explore the association between patient knowledge of CHD transmission risk and earlier physician counselling in adults with CHD.

We performed a cross-sectional survey of patients with CHD aged 18 years and older in a paediatric hospital.

Of the 100 patients who completed the questionnaire, most did not report counselling on heredity (66%) or contraception (71%). Of the 54 women, 25 (46%) identified their contraceptive options correctly; 42 (78%) women were classified as being at significantly increased risk for an adverse outcome during pregnancy, and of these 20 (48%) identified this risk correctly. Of all patients surveyed, 72% did not know that having CHD placed them at increased risk for having a child with CHD. On multivariate analysis, factors associated with correct knowledge about risk of recurrence were correct identification of CHD diagnosis (p=0.04) and patient-reported counselling (p=0.001).

Knowledge about heredity, pregnancy risk, and contraceptive options is inadequate among adults with CHD followed-up in a paediatric subspecialty clinic. The majority of patients did not report a history of counselling about reproductive health. There is a strong correlation between history of counselling by the patient’s cardiologist and correct knowledge about recurrence risk, suggesting that effective reproductive counselling can positively impact this knowledge gap.

The aim of this study was to describe the epidemiology of Ebstein’s anomaly in Europe and its association with maternal health and medication exposure during pregnancy.

We carried out a descriptive epidemiological analysis of population-based data.

We included data from 15 European Surveillance of Congenital Anomalies Congenital Anomaly Registries in 12 European countries, with a population of 5.6 million births during 1982–2011.

Cases included live births, fetal deaths from 20 weeks gestation, and terminations of pregnancy for fetal anomaly.

We estimated total prevalence per 10,000 births. Odds ratios for exposure to maternal illnesses/medications in the first trimester of pregnancy were calculated by comparing Ebstein’s anomaly cases with cardiac and non-cardiac malformed controls, excluding cases with genetic syndromes and adjusting for time period and country.

In total, 264 Ebstein’s anomaly cases were recorded; 81% were live births, 2% of which were diagnosed after the 1st year of life; 54% of cases with Ebstein’s anomaly or a co-existing congenital anomaly were prenatally diagnosed. Total prevalence rose over time from 0.29 (95% confidence interval (CI) 0.20–0.41) to 0.48 (95% CI 0.40–0.57) (p<0.01). In all, nine cases were exposed to maternal mental health conditions/medications (adjusted odds ratio (adjOR) 2.64, 95% CI 1.33–5.21) compared with cardiac controls. Cases were more likely to be exposed to maternal β-thalassemia (adjOR 10.5, 95% CI 3.13–35.3, n=3) and haemorrhage in early pregnancy (adjOR 1.77, 95% CI 0.93–3.38, n=11) compared with cardiac controls.

The increasing prevalence of Ebstein’s anomaly may be related to better and earlier diagnosis. Our data suggest that Ebstein’s anomaly is associated with maternal mental health problems generally rather than lithium or benzodiazepines specifically; therefore, changing or stopping medications may not be preventative. We found new associations requiring confirmation.

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.

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.

Shone’s syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone’s syndrome and the factors associated with left heart growth.

All patients diagnosed with Shone’s syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed.

A total of 121 patients with Shone’s syndrome presented at a median age of 28 days (0–17.3 years) and were followed-up for 7.2 years (0.01–35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time.

Shone’s syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.

“Athlete’s heart” is a cardiac adaptation to long-term intensive training. The aims of this study were to show the prevalence of left ventricular hypertrophy in teenagers who participate in sports, to define the different types of cardiac re-modelling, and to differentiate between physiological and pathological hypertrophy.

Echocardiographic measurements were obtained by M-mode, two dimensional, and Doppler techniques of participants from sports and control groups.

The echocardiographic examinations included 100 healthy teenagers taking part in dynamic sports such as football and basketball and 100 healthy teenagers taking part in static sports such as karate and judo. The control group (n=100) included healthy, sedentary teenagers. Sports participants had significantly higher left ventricular mass when compared with the control group, (p<0.001). In the dynamic sports group, 29% of the respondents had left ventricular mass above the 95th percentile, whereas 71% had left ventricular mass below the 95th percentile (p<0.001). The cardiac re-modelling was eccentric (79.4 versus 20.6%, p<0.001). In the group of static sports participants, 37% had left ventricular mass above the 95th percentile, whereas 63% had left ventricular mass below the 95th percentile (z score 0.74±0.82, p<0.001). The prevalence of concentric and eccentric types of re-modelling was equally manifested (54.05 versus 45.95%, p>0.05). Respondents from both groups had E/A ratios (transmitral flow velocity ratio)>1, preserved diastolic function, and statistically they did not differ from the control group.

Echocardiographic parameters show that physiological hypertrophy and cardiac re-modelling are present in teenagers who play sports. Unexpectedly, the prevalence of concentric and eccentric types of re-modelling is equally possible in the group of static sports participants.

This study aimed to (a) examine eHealth literacy, beliefs, and behaviours in parents of children with complex CHD, and (b) identify parents’ preferences for the content, format, features, and functions of eHealth resources for CHD.

Families (n=198) of children born between 2008 and 2011 and diagnosed with CHD requiring surgery were mailed a survey assessing a range of variables including eHealth literacy, beliefs, and behaviours as well as preferences for the format, functions, features, and content of eHealth resources for CHD.

A total of 132 parents (83 mothers, 49 fathers) completed the survey (response rate: 50%). Mothers (96%) were more likely to access eHealth resources than fathers (83%, χ2=6.74, p=0.009). Despite high eHealth resource use, eHealth literacy was relatively low, with results demonstrating considerable and widespread gaps in awareness of, access to, and communication about eHealth resources. Over 50% of parents reported that decisions regarding their child’s healthcare were influenced, to some extent, by web-based resources. Barriers to doctor–patient communication about eHealth included limited consultation time and concern about doctors’ disapproval. Participants demonstrated a strong desire for “eHealth prescriptions” from their child’s healthcare team, and perceived a wide range of eHealth topics as highly important, including treatment-related complications as well as physical, cognitive, and emotional development in children with CHD.

Results suggest a need for stronger, more proactive partnerships between clinicians, researchers, educators, technologists, and patients and families to bring about meaningful innovations in the development and implementation of eHealth interventions in paediatric cardiology.

Patients with hypoplastic left heart syndrome and its variants following palliation surgery are at risk for thrombosis. This study examines variability of antithrombotic practice, the incidence of interstage shunt thrombosis, and other adverse events following Stage I and Stage II palliation within the National Pediatric Cardiology Quality Improvement Collaborative registry.

We carried out a multicentre, retrospective review using the National Pediatric Cardiology Quality Improvement Collaborative registry including patients from 2008 to 2013 across 52 surgical sites. Antithrombotic medications used at Stage I and Stage II discharge were evaluated. Variability of antithrombotics use at the individual patient level and intersite variability, incidence of shunt thrombosis, and other adverse events such as cardiac arrest, seizure, stroke, and need for cardiac catheterisation intervention in the interstage period were identified. Antithrombotic strategies for hybrid Stage I patients were evaluated but they were excluded from the variability and outcomes analysis.

A total of 932 Stage I and 923 Stage II patients were included in the study: 93.8% of Stage I patients were discharged on aspirin and 4% were discharged on no antithrombotics, and 77% of Stage II patients were discharged on aspirin and 17.5% were discharged on no antithrombotics. Only three patients (0.2%) presented with interstage shunt thrombosis. The majority of patients who died during interstage or required shunt dilation and/or stenting were discharged home on aspirin.

Aspirin is the most commonly used antithrombotic following Stage I and Stage II palliation. There is more variability in the choice of antithrombotics following Stage II compared with Stage I. The incidence of interstage shunt thrombosis and associated adverse events was rare.

Morbidity is defined as a state of being unhealthy or of experiencing an aspect of health that is “generally bad for you”, and postoperative morbidity linked to paediatric cardiac surgery encompasses a range of conditions that may impact the patient and are potential targets for quality assurance.

As part of a wider study, a multi-disciplinary group of professionals aimed to define a list of morbidities linked to paediatric cardiac surgery that was prioritised by a panel reflecting the views of both professionals from a range of disciplines and settings as well as parents and patients.

We present a set of definitions of morbidity for use in routine audit after paediatric cardiac surgery. These morbidities are ranked in priority order as acute neurological event, unplanned re-operation, feeding problems, the need for renal support, major adverse cardiac events or never events, extracorporeal life support, necrotising enterocolitis, surgical site of blood stream infection, and prolonged pleural effusion or chylothorax. It is recognised that more than one such morbidity may arise in the same patient and these are referred to as multiple morbidities, except in the case of extracorporeal life support, which is a stand-alone constellation of morbidity.

It is feasible to define a range of paediatric cardiac surgical morbidities for use in routine audit that reflects the priorities of both professionals and parents. The impact of these morbidities on the patient and family will be explored prospectively as part of a wider ongoing, multi-centre study.

The aim of the present study was to investigate the relationships between red blood cell distribution width, platelet distribution width, and mean platelet volume and the presence and severity of valvular involvement in patients with rheumatic heart disease.

Between April, 2012 and December, 2015, 151 patients who were admitted to the Pediatric Cardiology Unit with diagnosis of rheumatic heart disease and 148 healthy children were included to our study. Transthoracic echocardiography for all children was performed, and the values of red blood cell distribution width, platelet distribution width, and mean platelet volume, besides other blood count parameters, erythrocyte sedimentation rate, and C-reactive protein levels were recorded.

Red blood cell distribution width, platelet distribution width, mean platelet volume, and C-reactive protein levels were significantly higher in patients with rheumatic heart disease when compared with healthy controls (p<0.01). Red blood cell distribution width was positively correlated with both C-reactive protein (r=0.271, p=0.035) and erythrocyte sedimentation rate (r=0.308, p=0.006). When single valve involvement was compared with both aortic valve and mitral valve involvement in the study group, red blood cell distribution width and platelet distribution width were higher in patients with double valve involvement; however, this was not statistically significant (p>0.05).

This is the first study in children with rheumatic heart disease that demonstrated significantly increased red blood cell distribution width, platelet distribution width, and mean platelet volume levels, as well as evaluated all three parameters together. Furthermore, red blood cell distribution width values in the chronical period of acute rheumatic fever, due to the positive correlation with the other chronic inflammatory markers, may help make the diagnosis in children.