Through this study we aimed to assess the educational level and employment status of adults with CHD in Germany.

Data were acquired from an online survey carried out in 2015 by the German National Register for Congenital Heart Defects. A total of 1458 adults with CHD participated in the survey (response rate: 37.6%). For 1198 participants, detailed medical information, such as main cardiac diagnosis and information from medical reports, was available.

Of the participants surveyed (n=1198), 54.5% (n=653) were female, and the mean age was 30 years. The majority of respondents (59.4%) stated that they had high education levels and that they were currently employed (51.1%). Patients with simple CHD had significantly higher levels of education (p<0.001) and were more likely to be employed (p=0.01) than were patients with complex CHD.

More than half of the participants had high education levels and the majority were employed. The association between CHD and its severity and individuals’ educational attainment should be investigated more closely in future studies.

One of the most important complications of Kawasaki disease is the development of giant coronary aneurysms. Risk factors for their development are still not clear.

A retrospective analysis was conducted at the National Institute of Paediatrics in Mexico City, Mexico. It included all patients with a diagnosis of acute Kawasaki disease between August, 1995 and August, 2015. Clinical and laboratory findings, as well as echocardiographic measurements, were recorded. Patients with giant coronary aneurysms (z-score⩾10) were compared with the rest of the patients. A value of p<0.05 was considered statistically significant. Odds ratios and their 95% confidence intervals were calculated to define risk factors.

During the study period, 416 patients were diagnosed with Kawasaki disease. Of them, 34 developed giant coronary aneurysms during the acute stage of the disease. In the multivariate analysis, patients younger than 1 year, those with a higher duration of illness at the time of diagnosis, and those who received additional intravenous immunoglobulin showed a significantly higher frequency of giant coronary aneurysms.

One of the main factors associated with the development of giant coronary aneurysms was the delay in the diagnosis of Kawasaki disease. This finding highlights the importance of maintaining a high suspicion of the disease, which would enable an early diagnosis and prompt treatment and decrease the risk for developing giant coronary aneurysms.

Optimal imaging is essential for catheter-based interventions in CHD. The three-dimensional models in volume-rendering technique currently in use are not standardised. This paper investigates the feasibility and impact of novel three-dimensional guidance with segmented and tessellated three-dimensional heart models in catheterisation of CHD. In addition, a nearly radiation-free two- to three-dimensional registration and a biplane overlay were used.

We analysed 60 consecutive cases in which segmented tessellated three-dimensional heart models were merged with live fluoroscopy images and aligned using the tracheal bifurcation as a fiducial mark. The models were generated from previous MRI or CT by dedicated medical software. We chose the stereo-lithography format, as this promises advantage over volume-rendering-technique models regarding visualisation. Prospects, potential benefits, and accuracy of the two- to three-dimensional registration were rated separately by two paediatric interventionalists on a five-point Likert scale. Fluoroscopy time, radiation dose, and contrast dye consumption were evaluated. Over a 10-month study period, two- to three-dimensional image fusion was applied to 60 out of 354 cases. Of the 60 catheterisations, 73.3% were performed in the context of interventions. The accuracy of two- to three-dimensional registration was sufficient in all cases. Three-dimensional guidance was rated superior to conventional biplane imaging in all 60 cases. We registered significantly smaller amounts of used contrast dye (p<0.01), lower levels of radiation dose (p<0.02), and less fluoroscopy time (p<0.01) during interventions concerning the aortic arch compared with a control group.

Two- to three-dimensional image fusion can be applied successfully in most catheter-based interventions of CHD. Meshes in stereo-lithography format are accurate and base for standardised and reproducible three-dimensional models.

Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term event-driven study.

Few studies compared the clinical efficacy and safety of switch from bosentan to macitentan only in adult patients with pulmonary arterial hypertension. We aimed to evaluate the clinical efficacy and safety of switch from bosentan to macitentan in children and young adults.

This is a single-institution, 24-week prospective study. Patients ⩾12 years of age with idiopathic/heritable pulmonary arterial hypertension or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan therapy were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors and inhaled prostanoids was allowed. Outcome measures included change from baseline to week 24, in the 6-minute walk distance, functional class, oxygen saturation at rest/after 6-minute walk distance test, systolic pulmonary artery pressure estimated by echocardiography, and brain natriuretic peptide levels. Safety end points included adverse events laboratory abnormalities.

A total of 13 patients – 5 male and 8 female – completed the study. The mean age was 20.3±6.5 years (12–35) and weight was 54.0±14.5 kg (27–75). Five patients were ⩽18 years of age. Macitentan improved 6-minute walk distance from baseline (mean: 466±35 m (300–590)), at 12 weeks (mean: 494±78 m (325–590), +28 m) (p<0.05), and at 24 weeks (mean: 507±58 m (325–625), +41 m) (p<0.05). Macitentan did not significantly change functional class, oxygen saturation at rest/after 6-minute walk distance test, brain natriuretic levels, and systolic pulmonary artery pressure (p>0.05). None of the patients had anaemia, hepatotoxicity, and peripheral oedema.

Our study is the first study that showed that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension and is well tolerated without any adverse events.

Maternal obesity is associated with an increased risk for adverse perinatal outcomes. Obesity is also associated with a chronic inflammatory state and metabolic derangements that affect the newborn. The additional use of cardiopulmonary bypass during the neonatal period could impact the systemic inflammatory response in the immediate postoperative period that manifests as cardiac depression and multi-organ dysfunction. This study aimed to determine the association of maternal obesity and excessive weight gain during pregnancy with the immediate postoperative morbidity of neonatal patients undergoing cardiopulmonary bypass.

A retrospective review of neonates who underwent cardiopulmonary bypass within the first 30 days of life at our institution between 2011 and 2013 was conducted. Postoperative variables investigated included the duration of length of mechanical ventilation, length of stay in the ICU, peak vasoactive inotrope scores, and peak lactate level. Maternal obesity was defined as 1st trimester body mass index ⩾30 kg/m2. Excessive weight gain was defined as ⩾12 kg gained during pregnancy. In order to determine the association between maternal obesity or excessive weight gain and postoperative variables, we used multiple linear regression, adjusting for birth weight and risk adjustment for congenital heart surgery score.

Records from 58 mother–baby dyads were examined. After controlling for birth weight and risk adjustment for congenital heart surgery score, there were no significant associations between maternal obesity and excessive weight gain during pregnancy versus all postoperative outcomes measured.

Despite the known negative impact of maternal obesity on perinatal outcomes, we were unable to find associations between maternal obesity and excessive weight gain during pregnancy versus postoperative outcomes.

The objective of this research was to study lung function, physical capacity, and effect of endurance training in children and adolescents after Fontan palliation compared with healthy matched controls.

Fontan patients (n=30) and healthy matched control patients (n=25) performed dynamic and static spirometry, and pulmonary diffusing capacity and maximal oxygen uptake tests, before and after a 12-week endurance training programme and at follow-up after 1 year.

Fontan patients had a restrictive lung pattern, reduced pulmonary diffusing capacity (4.27±1.16 versus 6.61±1.88 mmol/kPa/minute, p<0.001), and a reduced maximal oxygen uptake (35.0±5.1 versus 43.7±8.4 ml/minute/kg, p<0.001) compared with controls. Patients had air trapping with a higher portion of residual volume of total lung capacity compared with controls (26±6 versus 22±5%, p<0.05). Vital capacity increased for patients, from 2.80±0.97 to 2.91±0.95 L, p<0.05, but not for controls after endurance training. The difference in diffusing capacity between patients and controls appeared to be greater with increasing age.

Fontan patients have a restrictive lung pattern, reduced pulmonary diffusing capacity, and reduced maximal oxygen uptake compared with healthy controls. Endurance training may improve vital capacity in Fontan patients. The normal increase in pulmonary diffusing capacity with age and growth was reduced in Fontan patients, which is concerning. Apart from general health effects, exercise may improve lung function in young Fontan patients and should be encouraged.

Children with CHD and acquired heart disease have unique, high-risk physiology. They may have a higher risk of adverse tracheal-intubation-associated events, as compared with children with non-cardiac disease.

We sought to evaluate the occurrence of adverse tracheal-intubation-associated events in children with cardiac disease compared to children with non-cardiac disease. A retrospective analysis of tracheal intubations from 38 international paediatric ICUs was performed using the National Emergency Airway Registry for Children (NEAR4KIDS) quality improvement registry. The primary outcome was the occurrence of any tracheal-intubation-associated event. Secondary outcomes included the occurrence of severe tracheal-intubation-associated events, multiple intubation attempts, and oxygen desaturation.

A total of 8851 intubations were reported between July, 2012 and March, 2016. Cardiac patients were younger, more likely to have haemodynamic instability, and less likely to have respiratory failure as an indication. The overall frequency of tracheal-intubation-associated events was not different (cardiac: 17% versus non-cardiac: 16%, p=0.13), nor was the rate of severe tracheal-intubation-associated events (cardiac: 7% versus non-cardiac: 6%, p=0.11). Tracheal-intubation-associated cardiac arrest occurred more often in cardiac patients (2.80 versus 1.28%; p<0.001), even after adjusting for patient and provider differences (adjusted odds ratio 1.79; p=0.03). Multiple intubation attempts occurred less often in cardiac patients (p=0.04), and oxygen desaturations occurred more often, even after excluding patients with cyanotic heart disease.

The overall incidence of adverse tracheal-intubation-associated events in cardiac patients was not different from that in non-cardiac patients. However, the presence of a cardiac diagnosis was associated with a higher occurrence of both tracheal-intubation-associated cardiac arrest and oxygen desaturation.

IL-10, as a proinflammatory and anti-inflammatory cytokine, has been thought to have an important role in the development of Kawasaki disease. Variation in the IL-10 gene might lead to altered protein production, which may result in Kawasaki disease. Several studies have been performed to investigate the IL-10 -592 A/C polymorphism and Kawasaki disease risk. Unfortunately, the results of previous studies were inconsistent. Therefore, we performed a meta-analysis to derive a more precise estimation of the association between the IL-10 -592 A/C polymorphism and Kawasaki disease risk.

The association between the IL-10 -592 A/C polymorphism and Kawasaki disease risk was assessed by odds ratios (ORs) together with their 95% confidence intervals (CIs). Six studies were enrolled in the present meta-analysis.

Overall, no significant association between IL-10 -592 A/C polymorphism and Kawasaki disease risk was found under allele contrast (A versus C: OR=0.95, 95% CI=0.77–1.18, p=0.668), homozygote comparison (AA versus CC: OR=0.86, 95% CI=0.56–1.31, p=0.475), heterozygote comparison (CA versus CC: OR=0.88, 95% CI=0.65–1.19, p=0.479), recessive genetic model (AA versus CA/CC: OR=0.96, 95% CI=0.73–1.28, p=0.801), or dominant genetic model (AA/CA versus CC: OR=0.85, 95% CI=0.64–1.13, p=0.275).

We conclude that IL-10 -592 A/C polymorphism was not associated with Kawasaki disease risk in the Chinese population. However, more primary large-scale and well-designed studies are still required to further evaluate the interaction of IL-10 -592 A/C polymorphism with Kawasaki disease risk.

We have previously shown that the minor alleles of vascular endothelial growth factor A (VEGFA) single-nucleotide polymorphism rs833069 and superoxide dismutase 2 (SOD2) single-nucleotide polymorphism rs2758331 are both associated with improved transplant-free survival after surgery for CHD in infants, but the underlying mechanisms are unknown. We hypothesised that one or both of these minor alleles are associated with better systemic ventricular function, resulting in improved survival.

This study is a follow-up analysis of 422 non-syndromic CHD patients who underwent neonatal cardiac surgery with cardiopulmonary bypass. Echocardiographic reports were reviewed. Systemic ventricular function was subjectively categorised as normal, or as mildly, moderately, or severely depressed. The change in function was calculated as the change from the preoperative study to the last available study. Stepwise linear regression, adjusting for covariates, was performed for the outcome of change in ventricular function. Model comparison was performed using Akaike’s information criterion. Only variables that improved the model prediction of change in systemic ventricular function were retained in the final model.

Genetic and echocardiographic data were available for 335/422 subjects (79%). Of them, 33 (9.9%) developed worse systemic ventricular function during a mean follow-up period of 13.5 years. After covariate adjustment, the presence of the VEGFA minor allele was associated with preserved ventricular function (p=0.011).

These data support the hypothesis that the mechanism by which the VEGFA single-nucleotide polymorphism rs833069 minor allele improves survival may be the preservation of ventricular function. Further studies are needed to validate this genotype–phenotype association and to determine whether this mechanism is related to increased vascular endothelial growth factor production.

The Single Ventricle Reconstruction trial randomised neonates with hypoplastic left heart syndrome to a systemic-to-pulmonary-artery shunt strategy. Patients received care according to usual institutional practice. We analysed practice variation at the Stage II surgery to attempt to identify areas for decreased variation and process control improvement.

Prospectively collected data were available in the Single Ventricle Reconstruction public-use database. Practice variation across 14 centres was described for 397 patients who underwent Stage II surgery. Data are centre-level specific and reported as interquartile ranges across all centres, unless otherwise specified.

Preoperative Stage II median age and weight across centres were 5.4 months (interquartile range 4.9–5.7) and 5.7 kg (5.5–6.1), with 70% performed electively. Most patients had pre-Stage-II cardiac catheterisation (98.5–100%). Digoxin was used by 11/14 centres in 25% of patients (23–31%), and 81% had some oral feeds (68–84%). The majority of the centres (86%) performed a bidirectional Glenn versus hemi-Fontan. Median cardiopulmonary bypass time was 96 minutes (75–113). In aggregate, 26% of patients had deep hypothermic circulatory arrest >10 minutes. In 13/14 centres using deep hypothermic circulatory arrest, 12.5% of patients exceeded 10 minutes (8–32%). Seven centres extubated 5% of patients (2–40) in the operating room. Postoperatively, ICU length of stay was 4.8 days (4.0–5.3) and total length of stay was 7.5 days (6–10).

In the Single Ventricle Reconstruction Trial, practice varied widely among centres for nearly all perioperative factors surrounding Stage II. Further analysis may facilitate establishing best practices by identifying the impact of practice variation.

Families of children born with CHD face added stress owing to uncertainty about the magnitude of the financial burden for medical costs they will face. This study seeks to assess the family responsibility for healthcare bills during the first 12 months of life for commercially insured children undergoing surgery for severe CHD.

The MarketScan® database from Truven was used to identify commercially insured infants in 39 states from 2010 to 2012 with an ICD-9 diagnosis code for transposition of the great arteries, tetralogy of Fallot, or truncus arteriosus, as well as the corresponding procedure code for complete repair. Data extraction identified payment responsibilities of the patients’ families in the form of co-payments, deductibles, and co-insurance during the 1st year of life.

There were 481 infants identified who met the criteria. Average family responsibility for healthcare bills during the 1st year of life was $2928, with no difference between the three groups. The range of out-of-pocket costs was $50–$18,167. Initial hospitalisation and outpatient care accounted for the majority of these responsibilities.

Families of commercially insured children with severe CHD requiring corrective surgery face an average of ~$3000 in out-of-pocket costs for healthcare bills during the first 12 months of their child’s life, although the amount varied considerably. This information provides a framework to alleviate some of the uncertainty surrounding healthcare financial responsibilities, and further examination of the origination of these expenditures may be useful in informing future healthcare policy discussion.

We reviewed all patients who were supported with extracorporeal membrane oxygenation and/or ventricular assist device at our institution in order to describe diagnostic characteristics and assess mortality.

A retrospective cohort study was performed including all patients supported with extracorporeal membrane oxygenation and/or ventricular assist device from our first case (8 October, 1998) through 25 July, 2016. The primary outcome of interest was mortality, which was modelled by the Kaplan–Meier method.

A total of 223 patients underwent 241 extracorporeal membrane oxygenation runs. Median support time was 4.0 days, ranging from 0.04 to 55.8 days, with a mean of 6.4±7.0 days. Mean (±SD) age at initiation was 727.4 days (±146.9 days). Indications for extracorporeal membrane oxygenation were stratified by primary indication: cardiac extracorporeal membrane oxygenation (n=175; 72.6%) or respiratory extracorporeal membrane oxygenation (n=66; 27.4%). The most frequent diagnosis for cardiac extracorporeal membrane oxygenation patients was hypoplastic left heart syndrome or hypoplastic left heart syndrome-related malformation (n=55 patients with HLHS who underwent 64 extracorporeal membrane oxygenation runs). For respiratory extracorporeal membrane oxygenation, the most frequent diagnosis was congenital diaphragmatic hernia (n=22). A total of 24 patients underwent 26 ventricular assist device runs. Median support time was 7 days, ranging from 0 to 75 days, with a mean of 15.3±18.8 days. Mean age at initiation of ventricular assist device was 2530.8±660.2 days (6.93±1.81 years). Cardiomyopathy/myocarditis was the most frequent indication for ventricular assist device placement (n=14; 53.8%). Survival to discharge was 42.2% for extracorporeal membrane oxygenation patients and 54.2% for ventricular assist device patients. Kaplan–Meier 1-year survival was as follows: all patients, 41.0%; extracorporeal membrane oxygenation patients, 41.0%; and ventricular assist device patients, 43.2%. Kaplan–Meier 5-year survival was as follows: all patients, 39.7%; extracorporeal membrane oxygenation patients, 39.7%; and ventricular assist device patients, 43.2%.

This single-institutional 18-year review documents the differential probability of survival for various sub-groups of patients who require support with extracorporeal membrane oxygenation or ventricular assist device. The indication for mechanical circulatory support, underlying diagnosis, age, and setting in which cannulation occurs may affect survival after extracorporeal membrane oxygenation and ventricular assist device. The Kaplan–Meier analyses in this study demonstrate that patients who survive to hospital discharge have an excellent chance of longer-term survival.

Severe fatigue and cognitive dysfunction are frequent symptoms in patients with postural orthostatic tachycardia syndrome. They can be debilitating, and often do not resolve despite improvement in haemodynamic symptoms. Our analysis was intended to assess clinical outcomes of medication treatment for these symptoms in a large, single-centre paediatric programme.

We performed a retrospective review of patients treated for fatigue and cognitive dysfunction. Patients aged 18 years or younger at the time of initial diagnosis were included. Patients who had a specific medication ordered five or more times for these symptoms were confirmed by chart review for clinical improvement. Percentage of patients with clinical improvement for each medication and overall for all medications, as well as the number of medications per patient required to achieve improvement, were assessed. Data were analysed based on gender as well. t-Test and χ2 analyses were used to assess for differences between means in variables, or specific variables.

A total of 708 patients met study criteria, of whom 517 were treated for fatigue or brain fog. Overall efficacy was 68.8%, with individual medication effectiveness ranging from 53.1 (methylphenidate) to 16.5% (atomoxetine). There was no significant difference in efficacy with respect to gender. The median number of medications used per patient was 2, without gender difference. Therapy was limited by side effects or lack of efficacy.

Medications are effective in the improvement of fatigue and cognitive dysfunction in these patients. However, trials of multiple medications may be needed before achieving clinical improvement.

Our primary goal was to decrease time to resolution of postoperative chylothorax as demonstrated by total days of chest tube utilisation through development and implementation of a management protocol.

A chylothorax management protocol was implemented as a quality improvement project at a tertiary-care paediatric hospital in July, 2015. Retrospective analysis was completed on patients aged 0–17 years diagnosed with chylothorax within 30 days of cardiac surgery in a pre-protocol cohort (February, 2014 to June, 2015, n=20) and a post-protocol cohort (July, 2015 to March, 2016, n=22).

Patient characteristics were similar before and after protocol implementation. Duration of mechanical ventilation and cardiac ICU and hospital lengths of stay were unchanged between cohorts. Following protocol implementation, total duration of chest tube utilisation decreased from 12 to 7 days (p=0.047) with a decrease in maximum days of chest tube utilisation from 44 to 13 days. Duration of medium-chain triglyceride feeds decreased from 42 days to 31 days (p=0.01). In total, three patients in the post-protocol cohort underwent additional surgical procedures to treat chylothorax with subsequent resolution of chylothorax within 24 hours. There were no chest tube re-insertions or re-admissions related to chylothorax in either the pre- or post-protocol cohorts. Protocol compliance was 81%.

Adoption of a chylothorax management protocol is feasible, and in our small cohort of patients implementation led to a significant decrease in the duration of chest tube utilisation, while eliminating practice variability among providers.

The aim of this study was to evaluate the reduction of frame rate from 7.5 to 4 frames per second on radiation exposure and to provide new standards of radiation exposure.

Frame rate is a large contributor to radiation exposure. The use of 4 frames per second for closure of atrial septal defects has been reported not to affect the level of radiation exposure.

We retrospectively reviewed radiation data from all patients referred to our catheterisation laboratory for closure of an atrial septal defect between January, 2015 and June, 2017. Fluoroscopic time, dose area product (μGy.m2), and total air kerma (mGy) were collected. These values were compared according to the frame rate used for closure of atrial septal defects.

A total of 49 atrial septal defects were closed using 7.5 frames per second and 85 using 4 frames per second. Baseline characteristics were similar in both groups. Procedural success was similar in both groups (100 versus 98.8%). Median total air kerma and dose area product were statistically lower in the 4 frames per second group (4 versus 1.3 mGy [p=0.00012]), 43.7 versus 13.1 μGy.m2 [p<0.00001]). There was no increase in median procedure and fluoroscopic times (respectively, 10 and 1.1 min for 7.5 and 4 frames per second), or complications (4.1 versus 2.3%, p>0.05).

Reduction of frame rate allows reducing significantly the radiation exposure while maintaining excellent clinical results in transcatheter closure of atrial septal defects. We recommend implementing this little change in every laboratory in order to achieve drastic reduction of radiation exposure to the patients and laboratory personnel.

Despite improvements in the medical and surgical management of infants with CHD, growth failure before surgery in many infants continues to be a significant concern. A nutritional pathway was developed, the aim of which was to provide a structured approach to nutritional care for infants with CHD awaiting surgery.

The modified Delphi process was development of a nutritional pathway; initial stakeholder meeting to finalise draft guidelines and develop questions; round 1 anonymous online survey; round 2 online survey; regional cardiac conference and pathway revision; and final expert meeting and pathway finalisation.

Paediatric Dietitians from all 11 of the paediatric cardiology surgical centres in the United Kingdom contributed to the guideline development. In all, 33% of participants had 9 or more years of experience working with infants with CHD. By the end of rounds 1 and 2, 76 and 96% of participants, respectively, were in agreement with the statements. Three statements where consensus was not achieved by the end of round 2 were discussed and agreed at the final expert group meeting.

Nutrition guidelines were developed for infants with CHD awaiting surgery, using a modified Delphi process, incorporating the best available evidence and expert opinion with regard to nutritional support in this group.

The aim of this study was to identify and evaluate demographic and clinical features of paediatric patients with postural orthostatic tachycardia syndrome in a tertiary hospital speciality clinic.

This is a retrospective review of clinical data obtained during initial outpatient evaluation.

A total of 708 patients met the evaluation criteria. Female patients outnumbered males, 3.45:1. Caucasians were over-represented at 94.1% of patients. Median age at diagnosis was 15.7 years. Joint hypermobility occurred in 57.3% of patients; 22.4% had hypermobile Ehlers–Danlos syndrome; and 34.9% had hypermobility spectrum disorder. Median age of onset of symptoms was 12.6 years in patients with hypermobility versus 13.7 years in those without (p=0.0001). Median duration of symptoms was 3.3 years with hypermobility versus 1.5 years without (p<0.00001). Putative triggers included infection in 23.6% of patients, concussion in 11.4%, and surgery/trauma in 2.8%. Concurrent inflammatory disorders were noted in 5.2% of patients. Six symptoms comprised 80% of initial patient complaints. Overall, 66% of patients subsequently had at least 10 symptoms, 50% had at least 14 symptoms, and 30% reported at least 26 symptoms. Symptoms were largely cardiovascular, gastrointestinal, and neurological. Paediatric patients with postural orthostatic tachycardia syndrome seen in a large speciality clinic are predominantly female, are mostly Caucasian, have onset of symptoms in early adolescence, and have symptoms for over two years before diagnosis. Over half of patients have joint hypermobility. More than one-third of patients have a possible autoimmune or inflammatory trigger, including infection, concussion, or surgery/trauma. Patients experience symptoms that are highly variable and multi-system in origin over the course of illness.