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Contains fulltext : 291299.pdf (Publisher’s version ) (Open Access) With the trend towards childhood surgery in patients with Ebstein anomaly (EA), thorough imaging is crucial for patient selection. This study aimed to assess biventricular function by echocardiography and cardiac magnetic resonance (CMR) and compare EA severity classifications. Twenty-three patients (8-17 years) underwent echocardiography and CMR. Echocardiographic parameters included tricuspid annular plane systolic excursions (TAPSE), fractional area change of the functional right ventricle (fRV-FAC), fRV free wall peak systolic myocardial velocity (fRVs'), and tricuspid regurgitation (TR). End-diastolic and end-systolic volume (EDV resp. ESV), fRV- and LV ejection fraction (EF) and TR were obtained by CMR. EA severity classifications included displacement index, Celermajer index and the total-right/left-volume index. Median fRV-FAC was 38% (IQR 33-42). TAPSE and fRVs' were reduced in 39% and 75% of the patients, respectively. Echocardiographic TR was visually graded as mild, moderate, or severe in nine, six and eight patients, respectively. By CMR, median fRVEF was 49% (IQR 36-58) and TR was graded as mild, moderate, or severe in nine, twelve and two patients, respectively. In 70% of cases, fRV-EDV was higher than LV-EDV. LVEF was decreased in 17 cases (74%). There was excellent correlation between echocardiography-derived fRV-FAC and CMR-derived fRVEF (rho = 0.812, p

Liquid injection in fluidized beds is used to add reactants or to improve the heat management in the reactor. This injection will increase the complexity of reactor due to the formation of agglomerates. In this work the effect of the injection on the particle temperature distribution in a fluidized bed of porous particles is determined experimentally using particle image velocimetry and infra-red thermography. The main property of the porous particles influencing the distribution is the specific surface area. In addition, the porosity has a large effect on the defluidization of the fluidized bed.

Background: Improving maternal lifestyle before conception may prevent the adverse effects of maternal obesity on their children’s future cardiovascular disease (CVD) risk. In the current study, we examined whether a preconception lifestyle intervention in women with obesity could alter echocardiographic indices of cardiovascular health in their children. Methods: Six years after a randomized controlled trial comparing the effects of a 6-month preconception lifestyle intervention in women with obesity and infertility prior to fertility care to prompt fertility care, 315 of the 341 children conceived within 24 months after randomization were eligible for this study. The intervention was aimed at weight loss (≥5% or until BMI < 29 kg/m 2). Children underwent echocardiographic assessment of cardiac structure and function, conducted by a single pediatric cardiologist, blinded to group allocation. Results were adjusted for multiple variables including body surface area, age, and sex in linear regression analyses. Results: Sixty children (32 girls, 53%) were included, mean age 6.5 years (SD 1.09). Twenty-four children (40%) were born to mothers in the intervention group. Children of mothers from the intervention group had a lower end-diastolic interventricular septum thickness (−0.88 Z-score, 95%CI −1.18 to −0.58), a lower left ventricle mass index (−8.56 g/m 2, 95%CI −13.09 to −4.03), and higher peak systolic and early diastolic annular velocity of the left ventricle (1.43 cm/s 95%CI 0.65 to 2.20 and 2.39 cm/s 95%CI 0.68 to 4.11, respectively) compared to children of mothers from the control group. Conclusions: Children of women with obesity, who underwent a preconception lifestyle intervention, had improved cardiac structure and function; a thinner interventricular septum, lower left ventricle mass, and improved systolic and diastolic tissue Doppler velocities. Despite its high attrition rates, our study provides the first experimental human evidence suggesting that preconception lifestyle interventions may present a method of reducing CVD risk in the next generation. Clinical trial registration: LIFEstyle study: Netherlands Trial Register: NTR1530 (https://www.trialregister.nl/trial/1461). This follow-up study was approved by the medical ethics committee of the University Medical Centre Groningen (METC code: 2008/284).

The impact of solution stoichiometry, upon formation of BaSO4 crystals in 0.02 M NaCl suspensions, on the development of particle size was investigated using Dynamic Light Scattering (DLS). Measurements were performed on a set of suspensions prepared with predefined initial supersaturation (Ωbarite = {Ba2+}{SO42-}/Ksp = 1000) and dissolved ion activity stoichiometries (raq = {Ba2+}:{SO42-} = 0.01, 0.1, 1, 10 and 100), at a pH of 5.5 to 6.0, and ambient temperature and pressure. At this Ωbarite and set of raq, the average apparent hydrodynamic particle size of the largest population present in all suspensions grew from ~ 200 nm to ~ 700 nm within 10 to 15 minutes. This was independently confirmed by TEM imaging. Additional DLS measurements conducted at the same conditions in flow confirmed that the BaSO4 formation kinetics were very fast for our specifically chosen conditions. The DLS flow measurements, monitoring the first minute of BaSO4 formation, showed strong signs of aggregation of prenucleation clusters forming particles with a size in the range of 200 – 300 nm for every raq. The estimated initial bulk growth rates from batch DLS results show that BaSO4 crystals formed fastest at near stoichiometric conditions and more slowly at non-stoichiometric conditions. Moreover, at extreme SO4-limiting conditions barite formation was slower compared to Ba-limiting conditions. Our results show that DLS can be used to investigate nucleation and growth at carefully selected experimental and analytical conditions. Additional SEM imaging on formed BaSO4 crystals for a range of initial conditions of Ωbarite (i.e. 31, 200, 1000 and 6000), raq (0.01, 0.1, 1, 10 and 100) and different background electrolytes (i.e. NaCl, KCl, NaNO3, MgSO4 and SrCl2) confirms that {Ba2+}:{SO42-} impacts the growth rate significantly in different directions for the different background electrolytes at the different Ωbarite-values. Furthermore, the BaSO4 crystal morphology varies with raq and the type of background electrolyte. The combined DLS, TEM and SEM results imply that solution stoichiometry should be considered when optimizing antiscalant efficiency to regulate BaSO4 (scale) formation processes.

In patients with congenital heart disease (CHD), reduced exercise capacity can be a predictor for late complications and may be used to guide interventions. Yet, the interpretation of exercise capacity is challenged by changes in body composition during growth. Our aim was to create an overview of disease-specific exercise capacity in children with CHD. We performed a multicentre retrospective study of exercise capacity of CHD patients, aged 6–18 years, tested between January 2001 and October 2018. Sex-specific distribution graphs were made using the LMS method and height to relate to body size. We included all CHD with N > 50, including severe defects (e.g., univentricular heart, tetralogy of Fallot) and “simple” lesions as ventricular septum defect and atrial septum defect. We included 1383 tests of 1208 individual patients for analysis. The peak oxygen uptake (VO2peak, 37.3 ml/min/kg (25th–75th percentile 31.3–43.8)) varied between specific defects; patients with univentricular hearts had lower VO2peak compared with other CHD. All groups had lower VO2peak compared to healthy Dutch children. Males had higher VO2peak, Wpeak and O2pulsepeak than females. Sex- and disease-specific distribution graphs for VO2peak, Wpeak and O2pulsepeak showed increase in variation with increase in height. Conclusion: Disease-specific distribution graphs for exercise capacity in children with CHD from a large multicentre cohort demonstrated varying degrees of reduced VO2peak and Wpeak. The distribution graphs can be used in the structured follow-up of patients with CHD to predict outcome and identify patients at risk. What is Known:• Children with congenital heart disease (COnHD) are at risk to develop heart failure, arrhytmia’s and other complications. Exercise capacity may be an important predictor for outcome in children with ConHD. In children, the interpretation of exercise capacity poses an additional challenge related to physical changes during growth. What is New:• In this report of a multi-center cohort >1300 childrewn with ConHD, we related the changes in exercise capacity to length. We demonstrated that exercise capacity was reduced as compared with healthy children and we observed variation between disease groups. Patients with a univentricular circulation (Fontan) had worse exercise capacity. We constructed disease specific charts of development of exercise capacity throughout childhood, accessible via a web-site. These graphs may help practitioner to guide children with ConHD.

Introduction We present a case of dual drainage of the right upper lobe of the lung into the left atrium and via partial anomalous venous pulmonary return (PAPVR) into a persistent left superior vena cava (SVC). Discussion It is only in the minority of PAPVR cases where the anomalous pulmonary veins cross the midline. We provide a review of current literature on this topic and an explanatory embryological model. Knowledge of embryonic development and possible anatomic variations, including the concept of dual venous drainage of the lung, leads to better interpretation of imaging, with more accurate description of the morphology at hand. High-resolution multidetector computed tomography (MDCT) helps to delineate the exact vascular anatomy. This will enhance a better understanding of and anticipation on the patient’s disease status, with more accurate planning of intervention, and possibly less complications.

Many Fontan patients with and without systolic ventricular dysfunction are being treated with angiotensin-converting enzyme (ACE) inhibitors, despite its effectiveness remaining unclear. In the present study, we evaluated the short-term effect of enalapril on exercise capacity, vascular and ventricular function in pediatric Fontan patients with moderategood systolic ventricular function. Fontan patients between 8 and 18 years with moderategood systolic ventricular function and without previous ACE inhibitor treatment were included and were treated with enalapril for 3 months. During the first 2 weeks, the dosage was titrated according to systolic blood pressure (SBP). Exercise tests, ventricular function assessed by echocardiography, arterial stiffness measurements, and plasma levels of N-terminal pro-B-type natriuretic peptide assessed before and after a 3-month enalapril treatment period was compared. A total of 28 Fontan patients (median age 13.9 years, 6 to 15 years after Fontan operation) completed the study with a mean dosage of 0.3 +/- 0.1 mg/ kg/d. A total of 6 patients (21 %) experienced a significant drop in SBP and 6 others (21%) experienced other adverse events. Enalapril treatment lowered the SBP (from 110 to 104 mmHg, p = 0.003) and levels of N-terminal pro-B-type natriuretic peptide (from 80 to 72 ng/L, p = 0.036). However, enalapril treatment did not improve exercise capacity, ventricular function, or arterial stiffness. In conclusion, short-term ACE inhibition has no beneficial effect in Fontan patients with moderate-good systolic ventricular function. (C) 2021 The Authors. Published by Elsevier Inc.

A 14-week-old infant with an abnormal pronated position of the right arm was referred to the surgeon by the pediatrician. On physical examination, pro- and supination of the forearm was limited. Conventional radiography of the elbow showed a bony fusion of the proximal radius and ulna, a radioulnar synostosis.

Pathogenic heterozygous NEXN variants are associated with progressive dilated cardiomyopathy (DCM) usually presenting around 50 years of age. We describe an asymptomatic boy who had transient DCM at 3 months of age, that resolved by 4 months. Presently, at 11 years of age, he has normal cardiac function with signs of mild DCM on cardiac MRI. Genetic diagnostics revealed a paternally derived, heterozygous 1949_1951del class 4 variant in NEXN. His father had mild DCM with mildly reduced systolic function. The second patient presented with fetal hydrops at 33 weeks gestation requiring emergency caesarian delivery. Postnatally she required ventilation and continuous inotropic support for left ventricle systolic dysfunction. She died after 2 weeks when therapy was withdrawn. Homozygous c.1174C > T,p.(R392*) class 4 variants in the NEXN gene were found via WES. Microscopic investigation showed endomyocardial fibroelastosis. Her parents, both heterozygous carriers, had normal cardiac function and the family history was normal. These patients show a new clinical spectrum of pediatric cardiac disease seen in heterozygous and homozygous NEXN variants, ranging from mild, transient DCM to a severe, fatal neonatal DCM. These patients support the inclusion of the NEXN gene in the investigation of pediatric patients with DCM, even in cases with transient DCM.

The mass transfer between a rising bubble and the surrounding liquid is mainly determined by an extremely thin layer of dissolved gas near the bubble interface. Resolving this concentration boundary layer in numerical simulations is computationally expensive and limited to low Péclet numbers. Subgrid-scale models mitigate the resolution requirements by approximating the mass transfer near the interface. In this contribution, we validate an improved subgrid-scale model with a single-phase simulation approach, which solves only the liquid phase at a highly-resolved mesh. The mass transfer during the initial transient rise of moderately deformed bubbles in the range Re = 72–569 and Sc = 102–104 is carefully validated. The single-phase approach is able to mirror the two-phase flow field. The time-dependent local and global mass transfer of both approaches agree well. The difference in the global Sherwood number is below than 2.5%. The improved subgrid-scale model predicts the mass transfer accurately and shows marginal mesh dependency.

In patients with congenital heart disease (CHD), reduced exercise capacity can be a predictor for late complications and may be used to guide interventions. Yet, the interpretation of exercise capacity is challenged by changes in body composition during growth. Our aim was to create an overview of disease-specific exercise capacity in children with CHD. We performed a multicentre retrospective study of exercise capacity of CHD patients, aged 6-18 years, tested between January 2001 and October 2018. Sex-specific distribution graphs were made using the LMS method and height to relate to body size. We included all CHD with N 50, including severe defects (e.g., univentricular heart, tetralogy of Fallot) and "simple" lesions as ventricular septum defect and atrial septum defect. We included 1383 tests of 1208 individual patients for analysis. The peak oxygen uptake (VO

Magnetic particle tracking (MPT) was employed to study a rotating drum filled with cork particles, using both air and water as interstitial medium. This noninvasive monitoring technique allows for the tracking of both particle translation and rotation in dry granular and liquid–solid systems. Measurements on the dry and floating bed rotating drum were compared and detailed analysis of the bed shape and velocity profiles was performed. It was found that the change of particle–wall and particle–particle interaction caused by the presence of water significantly affects the bed behavior. The decreased friction leads to slipping of the particles with respect to the wall, rendering the circulation rate largely insensitive to increased drum speed. It was also found that the liquid–particle interaction is determining for the behavior of the flowing layer. The well-defined experiments and in-depth characterization performed in this study provide an excellent validation case for multiphase flow models.

Background: Due to the improved survival rates of children and adolescents with congenital heart disease (CHD), more attention is now being directed towards their health-related quality of life (HRQoL), emotional and behavioral problems. Ebstein anomaly (EA) is a rare CHD with a broad clinical spectrum. The aim of the current study is to evaluate self- and proxy-reported HRQoL and emotional and behavioral problems in children and adolescents with EA. Methods: In this cross-sectional, multicenter study, we included EA patients (aged 8-17 years), who underwent routine clinical assessments in Dutch university hospitals between May 2017 and March 2019. The Generic Pediatric Quality of Life Inventory 4.0 (for ages 8-12/13-17 years) was used to assess HRQoL. The Child Behavior Checklist, Youth Self-Report and Teacher's Report Form were completed to measure emotional and behavioral problems. Results: Questionnaires for thirty-eight patients (median age: 13 years; 48% male) were completed by patients themselves, parents and teachers. Compared to normative data, self-reported HRQoL was not significantly impaired, except for social functioning in children aged 8-12 years. Gender, severity of tricuspid regurgitation or previous surgery did not predict HRQoL. Parents reported significantly more internalizing (anxiety/depression), attention and thought problems in children with EA. These problems, however, were not reported by adolescents themselves. School teachers reported significantly fewer externalizing problems compared to the norm. Conclusions: Overall, despite satisfactory HRQoL, children and adolescents show emotional and behavioral problems. We recommend routine HRQoL and psychological assessment to screen for less apparent internalizing and psychosocial problems.

With the trend towards childhood surgery in patients with Ebstein anomaly (EA), thorough imaging is crucial for patient selection. This study aimed to assess biventricular function by echocardiography and cardiac magnetic resonance (CMR) and compare EA severity classifications. Twenty-three patients (8-17 years) underwent echocardiography and CMR. Echocardiographic parameters included tricuspid annular plane systolic excursions (TAPSE), fractional area change of the functional right ventricle (fRV-FAC), fRV free wall peak systolic myocardial velocity (fRVs'), and tricuspid regurgitation (TR). End-diastolic and end-systolic volume (EDV resp. ESV), fRV- and LV ejection fraction (EF) and TR were obtained by CMR. EA severity classifications included displacement index, Celermajer index and the total-right/left-volume index. Median fRV-FAC was 38% (IQR 33-42). TAPSE and fRVs' were reduced in 39% and 75% of the patients, respectively. Echocardiographic TR was visually graded as mild, moderate, or severe in nine, six and eight patients, respectively. By CMR, median fRVEF was 49% (IQR 36-58) and TR was graded as mild, moderate, or severe in nine, twelve and two patients, respectively. In 70% of cases, fRV-EDV was higher than LV-EDV. LVEF was decreased in 17 cases (74%). There was excellent correlation between echocardiography-derived fRV-FAC and CMR-derived fRVEF (rho = 0.812, p 0.001). While echocardiography is a versatile tool in the complex geometry of the Ebstein heart, it has limitations. CMR offers a total overview and has the advantage of reliable volume assessment of both ventricles. Comprehensive evaluation of pediatric patients with EA may therefore require a synergistic implementation of echocardiography and CMR.

Objective: To assess whether ‘treatment day’ is a significant predicting factor in Kawasaki disease and imposes a risk for coronary artery aneurysms (CAAs) in a per-day analysis. CAA formation can be reduced from 25% to 10% when treated with intravenous immunoglobulin (IVIG). Study design: Patient data from (n = 1016) a single center were collected for an observational cohort study. After exclusions, we retrospectively analyzed 776 patients in total. A multivariate analysis was performed with treatment day as a continuous variable (n = 691). Patients were categorized as no enlargement, small CAA, medium CAA, and giant CAA. Results: Late treatment per-day was a significant predicting factor for the development of larger CAAs. ORs for medium and giant CAAs per delayed day were 1.1 (95% CI 1.1-1.2) P < .05 and 1.2 (95% CI 1.1-1.2) P < .05, respectively. Conclusion: We showed that every day of delay in treatment of patients with Kawasaki disease inherently carries a risk of medium and giant aneurysm formation. There was no cut-off point for treatment day that could mark a safe zone.

Improving maternal lifestyle before conception may prevent the adverse effects of maternal obesity on their children's future cardiovascular disease (CVD) risk. In the current study, we examined whether a preconception lifestyle intervention in women with obesity could alter echocardiographic indices of cardiovascular health in their children.Six years after a randomized controlled trial comparing the effects of a 6-month preconception lifestyle intervention in women with obesity and infertility prior to fertility care to prompt fertility care, 315 of the 341 children conceived within 24 months after randomization were eligible for this study. The intervention was aimed at weight loss (≥5% or until BMI 29 kg/mSixty children (32 girls, 53%) were included, mean age 6.5 years (SD 1.09). Twenty-four children (40%) were born to mothers in the intervention group. Children of mothers from the intervention group had a lower end-diastolic interventricular septum thickness (-0.88 Z-score, 95%CI -1.18 to -0.58), a lower left ventricle mass index (-8.56 g/mChildren of women with obesity, who underwent a preconception lifestyle intervention, had improved cardiac structure and function; a thinner interventricular septum, lower left ventricle mass, and improved systolic and diastolic tissue Doppler velocities. Despite its high attrition rates, our study provides the first experimental human evidence suggesting that preconception lifestyle interventions may present a method of reducing CVD risk in the next generation.LIFEstyle study: Netherlands Trial Register: NTR1530 ( https://www.trialregister.nl/trial/1461 ). This follow-up study was approved by the medical ethics committee of the University Medical Centre Groningen (METC code: 2008/284).

In patients after Fontan completion exercise capacity is significantly reduced. Although peak oxygen consumption (VO2peak) is a strong prognostic factor in many cardiovascular diseases, it requires the achievement of a maximal effort. Therefore, submaximal exercise parameters such as oxygen uptake efficiency slope (OUES) may be of value. In the present observational study we evaluated the exercise capacity with maximal and submaximal parameters in a group of Fontan patients with an extracardiac conduit and determined their prognostic value. Sixty Fontan patients followed up in the Leiden University Medical Center who have performed an exercise test were included in this retrospective study. Exercise tests were performed at a median age of 11 years. Fontan patients showed on average lower values for all exercise parameters compared to reference values from a healthy dataset as shown by the %predicted values: VO2peak%:mean 66%(95%CI:64 to 74) and OUES%:mean 72%(95%CI:67 to 77). Twenty percent of the patients were not able to achieve an RER>1.0. RER showed a moderate positive correlation with VO2peak but not with OUES. There was a deterioration of VO2peak% and OUES% over time. OUES was significantly lower in patients with cardiac events in the follow up period. Fontan patients have an impaired exercise performance even at young ages and it deteriorates with age. An important percentage of Fontan patients is not able to reach maximal effort so the use of submaximal parameters, like OUES, should be considered as part of the evaluation. Moreover, OUES could have a prognostic value in this group of patients.

Purpose: After surgery for congenital heart disease in children and adolescents, an active lifestyle is important to achieve and maintain good physical fitness. This study aimed to describe physical activity, as well as the use of patients’ and parents’ preferences for rehabilitative care after surgery for congenital heart disease. Methods: This cross-sectional study included patients aged 4–16 years old, who had undergone surgery for congenital heart disease 2 years prior to the study onset. Patients and/or parents were invited to complete questionnaires on physical activity, and on their preferences for, and -use of, rehabilitative care after surgery. Results: Forty-five of the 92 eligible patients and/or parents completed the questionnaires. The median age of the children was 12.2 years (IQR 7.8–16.1). Two children (4%) met the recommendations for taking part in moderate physical activity and 13 (29%) having done so for vigorous physical activity. Postoperatively 15 children (33%) had had physical therapy in primary care. More than 50% of the patients/parents would have preferred to have had more information on physical activity and to have taken part in an individual exercise program delivered during their primary care. Conclusion: The majority of eligible patients do not meet public health recommendations for engaging in physical activity and do not use physical therapy after surgery for congenital heart disease. The majority of patients and parents preferred more information as well as individual physical therapy treatments.Implications for rehabilitations Two years after surgery for congenital heart disease children and adolescents are less active compared to typically developing peers. Patients with a congenital heart disease and their parents need more information and support regarding stimulation of physical activity and physical fitness after heart surgery. In the postoperative process, a well-structured and supportive (individual) rehabilitation program should be introduced to improve physical activity and physical fitness.

OBJECTIVES We evaluated the outcomes of biventricular repair after initial hybrid palliation performed in small infants with various forms of left ventricle hypoplasia. METHODS Between September 2010 and January 2020, a total of 27 patients had biventricular repair after hybrid palliation at a median age of 11 days. Indications for the hybrid approach included growth promotion of the left ventricle outflow tract and/or the aortic valve in 14 patients and that of the left ventricle in 13 patients. Seven reinterventions and 7 reoperations were performed during the interstage period. Significant growth of left ventricle parameters was noted during the median interstage period of 62 days. Sixteen subjects had aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients had the Ross–Konno procedure; 5 patients underwent the Yasui procedure; and 1 patient had unbalanced atrioventricular septal defect and aortic arch repair. RESULTS Twenty-three patients (85.2%) are alive at a median follow-up of 3.3 years. Two and 3 patients died early and late after achieving biventricular circulation, respectively. There were 22 reinterventions and 15 reoperations after biventricular repair. CONCLUSIONS Hybrid palliation can stimulate left heart growth in some patients with left ventricle hypoplasia. More patients may eventually achieve biventricular circulation than was initially thought. Additional interventions and operations are foreseeable. Despite ventricular rehabilitation, some patients with borderline left ventricles may develop restrictive physiology.

OBJECTIVES The objective of this study was to assess our 43-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) by analysing cardiac outcome measures (hospital and late mortality, reoperations and catheter interventions, significant coronary artery obstruction) and to identify risk factors for reoperation and catheter interventions. METHODS A total of 490 patients who underwent ASO for TGA from 1977 to 2020 were included in this retrospective, single-centre study. Data on reoperation and catheter intervention of hospital survivors were estimated by the Kaplan–Meier method and compared using a long-rank test. Risk factors for reoperation and/or catheter intervention were assessed by multivariate Cox regression analysis. RESULTS Hospital mortality occurred in 43 patients (8.8%), late death in 12 patients (2.9%) and 43 patients were lost to follow-up. Median follow-up time of 413 hospital survivors was 15.6 (interquartile range 7.0–22.4) years. Reoperations were performed in 83 patients (117 reoperations). Neoaortic valve regurgitation with root dilatation was the second most common indication for reoperation (15/83 patients, 18.1%) after right ventricular outflow tract obstruction (50/83 patients, 60.2%). Risk factors for any reoperation on multivariable analysis were: TGA morphological subtype [TGA with ventricular septal defect: hazard ratio (HR) = 1.99, 95% confidence interval (CI) 1.18–3.36; P = 0.010 and Taussig-Bing: HR = 2.17, 95% CI 1.02–4.64; P = 0.045], aortic arch repair associated with ASO (HR = 3.03, 95% CI 1.62–5.69; P = 0.001) and a non-usual coronary artery anatomy (HR = 2.41, 95% CI 1.45–4.00; P = 0.001). One hundred and one catheter interventions were performed in 54 patients, usually for relief of supravalvular pulmonary stenosis (44/54 patients, 81.5%) or arch obstruction (10/54 patients, 18.5%). Main risk factor for catheter intervention on multivariable analysis was aortic arch repair associated with ASO (HR = 2.95, 95% CI 1.37–6.36; P = 0.006). Significant coronary artery stenosis was relatively uncommon (9/413 patients, 2.2%) but may be underrepresented. CONCLUSIONS Patients after ASO typically have good long-term clinical outcomes but reoperations and interventions remain necessary in some patients. Neoaortic valve regurgitation with root dilatation is the second most common indication for reoperation after right ventricular outflow tract obstruction and an increasing need for neoaortic valve and root redo surgery in future is to be expected.

Background Although various determinants of exercise limitation in Fontan patients have been studied, most research has been performed in patients who underwent different surgical procedures with differing haemodynamic characteristics. The aim of the current study was to evaluate non-invasively measured cardiovascular parameters and their influence on exercise performance in paediatric Fontan patients with an extracardiac conduit and moderate-good systolic ventricular function. Methods Fontan patients, between 8 and 18 years of age, with moderate to good systolic ventricular function and an extracardiac conduit were included. Exercise performance and cardiovascular assessment, comprising echocardiography, aortic stiffness measurement and ambulatory measurement of cardiac autonomous nervous activity were performed on the same day. Healthy subjects served as controls. Results Thirty-six Fontan patients (age 14.0 years) and thirty-five healthy subjects (age 12.8 years) were included. Compared to controls, Fontan patients had reduced diastolic ventricular function and increased arterial stiffness. No differences were found in heart rate (HR) and cardiac parasympathetic nervous activity. In Fontan patients, maximal as well as submaximal exercise capacity was impaired, with the percentage of predicted capacity ranging between 54 and 72%. Chronotropic competence, however, was good with a peak HR of 174 (94% of predicted). Lower maximal and submaximal exercise capacity was correlated with a higher HR at rest, higher pulse wave velocity of the aorta and a lower ratio of early and late diastolic flow velocity. Conclusion Contemporary paediatric Fontan patients have an impaired exercise capacity with preserved chronotropic competence. Exercise performance correlates with heart rate at rest, diastolic function and aortic stiffness.

The aim of the study is to compare the efficacy of flecainide, beta-blockers, sotalol, and verapamil in children with frequent PVCs, with or without asymptomatic VT. Frequent premature ventricular complexes (PVCs) and asymptomatic ventricular tachycardia (VT) in children with structurally normal hearts require anti-arrhythmic drug (AAD) therapy depending on the severity of symptoms or ventricular dysfunction; however, data on efficacy in children are scarce. Both symptomatic and asymptomatic children (≥ 1 year and N = 10; p = 0.032), compared to the control group and other groups receiving beta-blockers (− 1.7 percentage points; N = 18), sotalol (+ 1.0 percentage points; N = 7), or verapamil (− 3.9 percentage points; N = 11). The efficacy of anti-arrhythmic drug therapy on frequent PVCs or asymptomatic VTs in children is very limited. Only flecainide appears to be effective in lowering the PVC burden.

Background Kawasaki disease (KD) is a vasculitis with formation of coronary artery aneurysms (CAAs) that can lead to myocardial ischemia. Echocardiography is the primary imaging modality for the coronary arteries despite limited visualization. Coronary angiography (CAG) is the gold standard yet invasive with high-radiation exposure. To date however, state-of-the-art CT scanners enable high-quality low-dose coronary computed tomographic angiography (cCTA) imaging. The aim of our study in KD is to report (i) the diagnostic yield of cCTA compared to echocardiography, and (ii) the radiation dose. Methods and results We collected data of KD patients who underwent cCTA. cCTA findings were compared with echocardiography results. In 70 KD patients (median age 15.1 years [0.5–59.5 years]; 78% male; 38% giant CAA), the cCTA identified 61 CAAs, of which 34 (56%, with a Z score > 3, in 22 patients) were not detected by echocardiography. In addition, the left circumflex (aneurysmatic in 6 patients) was always visible upon cCTA and not detected upon echocardiography. Calcifications, plaques, and/or thrombi were visualized by cCTA in 25 coronary arteries (15 patients). Calcifications were seen as early as 2.7 years after onset of disease. In 5 patients, the cCTA findings resulted in an immediate change of treatment. The median effective dose (ED) in millisievert differed significantly (p n = 56) vs 3.8 [1.7–20.0] (n = 14)). Conclusions The diagnostic yield of third-generation dual-source cCTA combined with reduced radiation exposure makes cCTA a favorable diagnostic modality to complete the diagnosis and long-term treatment indications for KD. Key Points • cCTA is a favorable diagnostic modality to complete the diagnosis and long-term treatment indications for Kawasaki disease. • Kawasaki disease patients with proven coronary artery involvement on echocardiography require additional imaging.