Congenital Heart Disease Group Research Articles

24-Hour Movement Behaviors in Children with Chronic Disease and Their Healthy Peers: A Case-Control Study.

Background: Time spent in 24-h movement behaviors is important to health and wellbeing in childhood, but levels of these behaviors in children with chronic disease are unknown. Methods: A case-control-study included 80 children with chronic disease; 20 with type 1 diabetes mellitus (T1DM), 20 with juvenile idiopathic arthritis (JIA), 20 with congenital heart disease (CHD), 20 with cystic fibrosis (CF); pair-matched individually for age, sex, and timing of measures with 80 healthy children. Habitual time spent in movement behaviors and step counts were all measured with an activPAL accelerometer over 7 days. Comparisons against recommendations and differences between the groups were made. Results: Time spent in physical activity and step counts/day were significantly lower in T1DM and CHD groups compared to controls. Only 20/80 children with chronic disease and 29/80 controls met step count recommendations. Sedentary time was significantly higher in children with CF compared to controls. Time spent asleep was slightly greater in children with chronic disease, significant only for the JIA group. Sleep disruption was consistently greater in those with chronic disease, reaching significance for T1DM, CHD, and CF groups. Conclusions: For some groups of children with chronic disease, 24-h movement behaviors may differ substantially from recommendations, and slightly but systematically from their healthy peers. Optimizing levels of 24-h movement behaviors should confer a number of benefits for child health, development, and wellbeing.

Double gas transfer factors (DLCO-DLNO) at rest in patients with congenital heart diseases correlates with their ventilatory response during maximal exercise

AimExercise capacity is altered in congenital heart diseases (CHD) with potentially impaired pulmonary perfusion adaptation during exercise, such as in single ventricle or in significant pulmonary regurgitation. This study aimed to evaluate the value of double gas transfer factor analysis, at rest in conjunction with postural manoeuvres, to explore the various components of pulmonary gas transfer and its association with exercise capacity. MethodsA total of 40 subjects (24 CHD, 16 controls) underwent a combined measurement of lung diffusing capacity for carbon monoxide and nitric oxide (DLCO-DLNO) to determine pulmonary membrane diffusion (Dm) and Vcap, in sitting then supine position. CHD patients performed a maximal cardiopulmonary exercise test. ResultsCompared to normal controls, the CHD group's DLNO, DLCO, Vcap, and alveolar volume (AV) at rest, in the sitting position were depressed, whereas the DLCO/AV and DLNO/AV were similar. The magnitude of Dm and Vcap adaptation induced by postural change was similar in both groups, indicating a preserved pulmonary capillary recruitment capacity in CHD. In the CHD group, at rest, for each ml of postural-induced increase in Vcap we observed during exercise a VE/VCO2 slope decrease of 0.46 (95% CI[0.83; 0.098]), indicating a better ventilatory response to exercise. ConclusionCHD patients with impaired pulmonary circulation have a reduced Dm and Vcap mainly due to decreased pulmonary volume but maintain a normal capacity to adapt these parameters through a simple recruitment manoeuver. Vcap adaptation evaluated at rest predicts the level of ventilatory efficiency during exercise, which represents a main limiting factor in these CHD patients.

Expression and clinical significance of circular RNA hsa_circ_0003416 in pediatric pulmonary arterial hypertension associated with congenital heart disease.

BackgroundCircular RNAs (circRNAs) have been found to be involved in the development of pulmonary arterial hypertension (PAH). However, their diagnostic value in pediatric PAH remains unclear. This study aimed to examine the characteristic expression of the circRNA hsa_circ_0003416 in the plasma of children with PAH caused by congenital heart disease (CHD); the potential of hsa_circ_0003416 as a diagnostic biomarker was also investigated.MethodsThe plasma expression levels of hsa_circ_0003416 were determined via quantitative reverse transcription–polymerase chain reaction in 50 CHD patients, 50 PAH patients, and 20 healthy subjects; the associations between hsa_circ_0003416 levels and clinical data were analyzed thereafter. Receiver operating characteristic curves were employed to determine the diagnostic capacity of this circRNA.ResultsExpression levels of hsa_circ_0003416 in plasma were lower in the PAH‐CHD group than in the CHD and healthy control groups (p = 0.009 vs. healthy control group, p = 0.026 vs. CHD group). Moreover, hsa_circ_0003416 was found to be negatively associated with B‐type natriuretic peptide (r = −0.342, p = 0.013). In addition, the area under the curve of hsa_circ_0003416 levels in plasma was 0.721 (95% confidence intervals = 0.585–0.857, p = 0.004), suggesting that it has a promising diagnostic value.ConclusionsOverall, hsa_circ_0003416 was found to be significantly downregulated in children with PAH‐CHD and to be potent as a biomarker for PAH‐CHD diagnosis.

Fetal Brain Volume Predicts Neurodevelopment in Congenital Heart Disease.

Neurodevelopmental impairment is common in children with congenital heart disease (CHD), but postnatal variables explain only 30% of the variance in outcomes. To explore whether the antecedents for neurodevelopmental disabilities might begin in utero, we analyzed whether fetal brain volume predicted subsequent neurodevelopmental outcome in children with CHD. Fetuses with isolated CHD and sociodemographically comparable healthy control fetuses underwent fetal brain magnetic resonance imaging and 2-year neurodevelopmental evaluation with the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) and the Adaptive Behavior Assessment System, Third Edition (ABAS-3). Hierarchical regression evaluated potential predictors of Bayley-III and ABAS-3 outcomes in the CHD group, including fetal total brain volume adjusted for gestational age and sex, sociodemographic characteristics, birth measures, and medical history. The CHD group (n=52) had lower Bayley-III cognitive, language, and motor scores than the control group (n=26), but fetal brain volumes were similar. Within the CHD group, larger fetal total brain volume correlated with higher Bayley-III cognitive, language, and motor scores and ABAS-3 adaptive functioning scores (r=0.32-0.47; all P<0.05), but this was not noted in the control group. Fetal brain volume predicted 10% to 21% of the variance in neurodevelopmental outcome measures in univariate analyses. Multivariable models that also included social class and postnatal factors explained 18% to 45% of the variance in outcome, depending on developmental domain. Moreover, in final multivariable models, fetal brain volume was the most consistent predictor of neurodevelopmental outcome across domains. Small fetal brain volume is a strong independent predictor of 2-year neurodevelopmental outcomes and may be an important imaging biomarker of future neurodevelopmental risk in CHD. Future studies are needed to support this hypothesis. Our findings support inclusion of fetal brain volume in risk stratification models and as a possible outcome in fetal neuroprotective intervention studies.

Major Adverse Cardiovascular Events in Korean Congenital Heart Disease Patients: A Nationwide Age- and Sex-Matched Case-Control Study.

Congenital heart disease (CHD) is a known risk factor for acquired cardiovascular and cerebrovascular diseases. However, available evidence on CHD is limited mostly to Western populations. This study aimed to evaluate the prevalence of vascular events and all-cause mortality in Korean patients with CHD and to further corroborate CHD as a predictor of vascular events and all-cause mortality. The claims data of the Korean National Health Insurance Service (NHIS) were retrospectively reviewed. Information regarding diagnostic codes, comorbidities, medical services, income level, and residential area was also collected. Outcomes of interest included stroke, myocardial infarction (MI), all-cause mortality, and major adverse cardiovascular events (MACE). We included 232203 patients with CHD and 3024633 individuals without CHD as a control group through age- and sex-matched 1:10 random sampling. The prevalences of hypertension, congestive heart failure, ischemic heart disease, hyperlipidemia, and atrial fibrillation were significantly higher in the CHD group, which had a more than two-fold higher incidence of vascular events and all-cause mortality, than in the group without CHD. Multivariable models demonstrated that CHD was a significant risk factor for stroke, MI, all-cause mortality, and MACE. In conclusion, this nationwide study demonstrates that Korean patients with CHD have a high incidence of comorbidities, vascular events, and mortality. CHD has been established as an important predictor of cardiovascular events. Further studies are warranted to identify high-risk patients with CHD and related factors to prevent vascular events.

Results of aortopulmonary collateral arteries coil embolization in pre-fontan patients

Background: Aortopulmonary collateral arteries (APCAs) are common pulmonary blood supply in the group of congenital heart disease with functionally single ventricle. APCAs causes loss of aortic outflow, increase in pulmonary arterial hydrostatic pressure, reducing blood flow from the SVC and IVC to the pulmonary artery (in patients after Glenn and Fontan operations). Therefore, the closure of APCAs is vital in the management of single ventricle patients before and during Fontan operation. The technique of percutaneous APCAs occlusion with coils is a new, effective and better method of control of APCAs, excluding the need for surgical ligation of the APCAs..&#x0D; Objectives: To evaluate results of aortopulmonary collateral arteries coil embolization in pre-fontan patients&#x0D; Methods: This is the descriptive clinical and retrospective study&#x0D; Result: 29 patients with average age of 7.9 underwent cardiac catheterization with APCAs coil embolization successfully before the Fontan surgery. Complication rate was very low. This method effectively reduced mean pulmonary artery pressure with statistical significance. The results of Fontan surgery of this group were better than that in other studies in the rate of diaphragmatic paralysis and chylous effusion&#x0D; Conclusions: The technique of percutaneous APCAs occlusion with coils before Fontan surgery is safe, effective in reducing mean pulmonary artery pressure and improving results of Fontan surgery.

Aerobic fitness in overweight/obese children with congenital heart disease: Benefit of adapted VO2 reference values

Overweight/obesity is a major public health problem in the general paediatric population with a prevalence of 21% in Europe. This prevalence is more important in children with chronic diseases (including congenital heart disease (CHD)). We know that children with CHD have lower aerobic fitness than “healthy” children. Overweight/obesity also has a negative impact on this aerobic fitness. Overweight/Obese children with CHD should have lower aerobic fitness than healthy obese children, but until now there have been no reference values to assess aerobic fitness in this overweight subpopulation. Our team recently provided paediatric reference values with adapted Z scores for extreme weights. The main objective of this study is to assess whether these new reference values discriminate healthy obese children from obese children with CHD compared to the usual refence values of Cooper et al. This cross-sectional study was carried out between November 2010 and November 2020 in two tertiary care paediatric cardiology reference centres and included both “healthy” and CHD children with BMI > 85th percentiles. 344 children were included (185 boys), 244 controls and 100 CHD. Using the usual standards (Cooper et al.), no significant difference in VO2 max between CHD and control groups was found (76 ± 18% vs. 77 ± 21%; P = 0.7). According to our new reference values, a significant difference was found for the Z-score of VO2 max between the CHD and control groups (−0.46 ± 1.31 vs. −0.10 ± 1.03; P = 0.006). The CHD subgroups in according with ACC-CHD classification found a lowest mean VO2 max Z-score in single ventricle malformations, anomalies of the atrioventricular junctions and complex anomalies of the right ventricle outflow tract. This study reveals the benefit of these new references values adapted to extreme weights by discriminating a pathological group (CHD) from a control group.

Contemporary pregnancy outcomes for women with moderate and severe congenital heart disease.

Women with congenital heart disease (CHD) are surviving into adulthood, with more undergoing pregnancy. Retrospective review of the Vizient database from 2017-2019 for women 15-44 years old with moderate, severe or no CHD and vaginal delivery or caesarean section. Demographics, hospital outcomes and costs were compared. There were 2,469,117 admissions: 2,467,589 with no CHD, 1277 with moderate and 251 with severe CHD. Both CHD groups were younger than no CHD, there were fewer white race/ethnicity in the no CHD group and more women with Medicare in both CHD groups compared to no CHD. With increasing CHD severity there was an increase in length of stay, ICU admission rates and costs. There were also higher rates of complications, mortality and caesarean section in the CHD groups. Pregnant women with CHD have more problematic pregnancies and understanding this impact is important to improve management and decrease healthcare utilization.

Abstract 9192: Efficacy and Safety of Dabigatran in the Treatment and Secondary Prophylaxis of Children With Congenital Heart Disease

Introduction: Congenital heart disease (CHD) is associated with an increased frequency of thrombotic events in children, particularly in cyanotic conditions (i.e., single ventricles). Phase IIb/III clinical trials in children with venous thromboembolism (VTE) reported the noninferiority of dabigatran etexilate (DE) versus standard of care in treating acute VTE (DIVERSITY, NCT01895777) and a favorable safety profile for VTE prevention (NCT02197416). Aim: To evaluate the efficacy and safety of DE in the treatment and secondary prophylaxis of VTE in children with CHD, including subsets with cyanotic CHD. Methods: The primary composite efficacy endpoint in DIVERSITY was complete thrombus resolution and freedom from VTE recurrence or VTE-related death; safety endpoints included bleeding events (BEs). Primary endpoints in the VTE prevention trial were VTE recurrence and BEs. CHD status was confirmed locally using standard diagnostic modalities. Results: In DIVERSITY, 48/267 (18.0%) children had CHD. DE was noninferior to standard of care in both the CHD and non-CHD groups for the composite primary endpoint ( Table ), irrespective of the presence of cyanotic or noncyanotic CHD ( Table ). BEs (mainly minor) appeared to be lower in the CHD (8.3%) than the non-CHD group (25.7%), regardless of treatment. In the secondary VTE prevention trial, 17/211 (8.1%) children had CHD. Recurrent VTE at 12 months occurred in 0 and 3 (1.5%) children in the CHD and non-CHD groups respectively ( Table ). Conclusions: In children with CHD, DE has an efficacy and safety profile consistent with the overall DIVERSITY trial. Furthermore, our results confirm the safety profile for secondary VTE prevention, suggesting that DE is a viable anticoagulant alternative for this population.

Abstract 10072: Stroke Topology and Neurodevelopment in Infants with Congenital Heart Disease: Does it Differ by Cardiac Physiology?

Objectives: Vulnerability to brain injury in infants with congenital heart disease (CHD) may differ by CHD lesion. However, the link between arterial ischemic stroke (AIS) and neurodevelopment, and whether it differs by CHD lesion, is unknown. We sought to determine whether: (1) Stroke topology differs between infants with Transposition of the Great Arteries (TGA) and single ventricle physiology (SVP), and relationship with neurodevelopment;(2) Associations between stroke volume and neurodevelopment are CHD lesion specific. Methods: 64 of 312 CHD infants (TGA n=38, SVP n=26) studied prospectively with pre- and/or post-operative brain MRIs had AIS. AIS were segmented on 3DT1 and/or ADC images. 39 infants completed 18-month neurodevelopmental assessments with Bayley Scales (2 nd or 3 rd Edition); scores were adjusted to account for differences between versions. Adverse neurodevelopment was defined as &lt;85 points. We used multivariable linear regression models to study associations between AIS volume and neurodevelopment, stratifying by CHD lesion and adjusting for study site. Probability maps demonstrating areas vulnerable to AIS and odds ratio maps reflecting likelihood of a lesion predicting adverse outcomes were developed. Results: Most AIS were in MCA territories, with a left-sided predominance (Fig 1A). Stroke volume did not differ between CHD groups (p=0.8). Basal ganglia lesions were most predictive of cognitive (max OR=11) and motor (max OR=6) outcomes (Fig 1B). Stratifying by CHD lesion, AIS volume predicted 18-month cognitive outcomes in infants with TGA (β=-0.6, 95%CI -1.0-(-0.1), p=0.02) but not SVP (β=7.9, 95%CI -72-88). AIS volume did not predict motor outcomes in infants with TGA (β=-0.6, 95%CI -1.6-0.3) or SVP (β=-19.7, 95%CI -91-52). Conclusions: Neonatal AIS topology does not differ between infants with TGA and SVP. AIS location and size are important predictors of neurodevelopment at 18 months though this association differs by CHD lesion.

Longitudinal Trends in Body Mass Index for Adults with Congenital Heart Disease.

Obesity is a modifiable, independent risk factor for adverse long-term outcomes in children and adults. Our objective was to determine the prevalence of overweight and obese status in a cohort of adults with congenital heart disease (CHD) as well as to assess longitudinal trends over a 20-year period. The study group consisted of patients 18years of age and older followed at our adult CHD clinic. Body mass index (BMI) data were collected from our index period, consisting of patient encounters from 2009 to 2012 (Period 2), as well as during 2000-2003 (Period 1) and 2017-2020 (Period 3) when available. The study cohort was subdivided into three groups per published guidelines: simple, moderate, and greater CHD complexity. The prevalence of obesity and overweight status was compared among the different groups as well as with published data (NHANES). Our cohort in Period 2 consisted of 261 subjects. The median age (25-75% interquartile range) for Period 2 was 27.6 (21.1-35.9) years and BMI was 25.2 (21.7-30.0) kg/m2 with 8.0% underweight, 40.0% with normal weight, 27.0% overweight, and 25% obese. 95 patients had follow-up data from each time period, with 96% of patients having moderate or greater complexity of CHD. The combined percentage of overweight and obese patients for the moderate and greater complex CHD groups increased from 42 and 37% in period 1 to 60% and 65% in period 3, respectively. The percentage of obese patients with moderate and greater CHD complexity increased by 250% and 55%, respectively, from Period 1 to 3. Our study cohort had a high prevalence of overweight and obese weight status. Given adults with CHD have high baseline cardiovascular morbidity, the presence of obesity can increase their risk for poor outcomes, highlighting the need for prevention of this modifiable risk factor.

Pulmonary arterial hypertension associated with congenital heart disease in children: clinical characterisation, outcomes and changes in demographics over time

Abstract Background/Introduction Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) in children and is associated with significant morbidity and mortality. The impact of different phenotypes on management and survival remains unclear. Purpose To examine the clinical features, management and outcomes of paediatric PAH-CHD patients based on the 20-year experience of the UK National Paediatric Pulmonary Hypertension (PH) Service. Methods Consecutive PAH-CHD patients entering the service between January 2001 and January 2021 were included and classified into: Eisenmenger syndrome (ES, group A), PAH related to a significant systemic-pulmonary shunt (group B), PAH with small or co-incidental CHD (group C) and PAH following defect repair (group D). Incident patients (without pre-existing PAH) were included in survival analysis. Results Of the overall PH paediatric cohort of 1104 patients, 819 (74.2%) had co-existing CHD and 354 (32.1%) patients received a diagnosis of PAH-CHD: 57.1% female, median [IQR] 4.6 [1.7–10.9] years. Group D PAH-CHD was the commonest subgroup, accounting for 36%, while the least frequent subtype was group C (14%). Group A and group B PAH-CHD represented 26% and 24%, respectively. Down syndrome was present in over one third (122, 34.5%) of PAH-CHD patients and was more commonly associated with ES (p=0.02). PAH therapy was started in 79.9% of PAH-CHD patients. At the end of follow-up, patients with group C PAH-CHD were more likely to be on combination therapy than any other group (64.6% vs. 28.4%, p&amp;lt;0.0001). Prostanoid therapy was used in a minority (11%) of patients. The subgroup distribution of PAH-CHD at diagnosis changed from the early (2000–2005) to late (2015–2020) period (Figure 1). The proportion of ES patients decreased from 43.4% to 14.6% of PAH-CHD (p&amp;lt;0.0001). The proportion of group B PAH-CHD patients increased (9.4% vs. 33.3%, p&amp;lt;0.0001), with the majority (59.3%) deemed “operable” on specialist assessment. There was a trend for an increase in repaired PAH-CHD patients between the early and late era (31.1% vs. 43.8%, p=0.09). Transplant-free survival in PAH-CHD was 90.9% (95% CI: 87.8–94%) at 1 year, 77.9% (95% CI: 73.1–83.1%) at 5 years, and 74.9% (95% CI: 69.6–80.7%) at 10 years (Figure 2). Group C PAH-CHD had a lower transplant-free survival than the other 3 groups (HR 2.54, 95% CI: 1.51–4.28, p=0.0005). There was no difference in outcome between group A and group D PAH-CHD (HR 1.19, 95% CI: 0.62–2.28, p=0.6). Conclusions Repaired PAH-CHD, not ES, was the most common subtype in this large paediatric cohort. Over time, there were fewer ES patients and more “operable” patients with left-right shunts, suggesting an improvement in early diagnosis and management. Despite widespread use of PAH therapy, PAH-CHD remains a life-limiting disease with the poorest outcomes in PAH with co-incidental CHD. Funding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Dr Constantine received a personal PhD fellowship grant (CHAMPION PhD Fellowship) Figure 1Figure 2

The Effects of Physical Inactivity and Exercise at Home in Young Patients with Congenital Heart Disease during the COVID-19 Pandemic.

Background: The COVID-19 pandemic had a significant impact on the population’s ability to be physically active. Purpose: Evaluate the effect of the COVID-19 mitigation measures on exercise tolerance in patients with congenital heart disease (CHD). Materials and methods: All subjects (880, 6–18 years old) who performed a stress test at our hospital from October 2020 to February 2021 and had a similar test one year earlier were enrolled. A questionnaire on the degree of physical activity carried out in 2020 concerning the period prior to the pandemic was compiled. Exercise tolerance and the main anthropometric parameters between the first and second tests were compared. Results: 110 subjects (11.9 ± 4.1 years) were included in the study. The percentage of patients engaged in regular physical activity (RPA) decreased significantly during the pandemic (p < 0.001), and BMI increased significantly (p < 0.001), except among the subjects who began RPA during the lockdown, whereas test duration did not decrease significantly overall but increased in this last subgroup (p < 0.05) Conclusions: The COVID-19 lockdown led to a less active lifestyle with a significant increase in BMI in our group of CHD. These data could have negative effects on the risk profile of this population. RPA practiced at home seems to be effective in counteracting such effects.

Microbiome Analysis of Carious Lesions in Pre-School Children with Early Childhood Caries and Congenital Heart Disease.

Oral bacteria have been associated with several systemic diseases. Moreover, the abundance of bacteria associated with caries has been found to be higher in patients with congenital heart disease (CHD) than in healthy control groups (HCGs). Therefore, this study aimed to evaluate the dental microbiota in children with CHD compared to a HCG. The aim was to describe and compare the carious microbiome regarding the composition, diversity, and taxonomic patterns in these two groups. Twenty children with CHD and a HCG aged between two and six years participated. All of them were affected by early childhood caries. Microbiome profiling indicated that Fusobacterium, Prevotella, Capnocytophaga, and Oribacterium were more abundant in the CHD group, whereas Lactobacillus and Rothia were predominant in the HCG. Furthermore, microbiome analysis revealed three distinct clusters for the CHD and HCG samples. In the first cluster, we found mainly the genera Lactobacillus and Coriobacteriaceae. The second cluster showed a higher relative abundance of the genus Actinomyces and a more diverse composition consisting of more genera with a smaller relative lot. The third cluster was characterized by two genera, Streptococcus and Veillonella. These data can help us to understand the oral microbial community structures involved in caries and endodontic infections of pre-school children in relation to the general health of these high-risk patients.

Aerobic fitness in overweight/obese children with congenital heart disease: Benefit of adapted VO2 reference values

Overweight/obesity is a major public health problem in the general paediatric population with a prevalence of 21% in Europe. This prevalence is more important in children with chronic diseases [including congenital heart disease (CHD)]. We know that children with CHD have lower aerobic fitness than “healthy” children. Overweight/obesity also has a negative impact on this aerobic fitness. Overweight/Obese children with CHD should have lower aerobic fitness than healthy obese children, but until now there have been no reference values to assess aerobic fitness in this overweight subpopulation. Our team recently provided paediatric reference values with adapted Z scores for extreme weights. The main objective of this study is to assess whether these new reference values discriminate healthy obese children from obese children with CHD compared to the usual reference values of Cooper et al. This cross-sectional study was carried out between November 2010 and November 2020 in two tertiary care paediatric cardiology reference centres and included both “healthy” and CHD children with BMI > 85th percentiles. In total, 344 children were included (185 boys), 244 controls and 100 CHD. Using the usual standards (Cooper et al.), no significant difference in VO2 max between CHD and control groups was found (76 ± 18% vs. 77 ± 21%; P = 0.7). According to our new reference values, a significant difference was found for the Z-score of VO2 max between the CHD and control groups (−0.46 ± 1.31 vs. −0.10 ± 1.03; P = 0.006). The CHD subgroups in according with ACC-CHD classification found a lowest mean VO2 max Z-score in the single ventricle malformations, anomalies of the atrioventricular junctions and complex anomalies of the right ventricle outflow tract. This study reveals the benefit of these new references values adapted to extreme weights by discriminating a pathological group (CHD) from a control group.

Infective endocarditis in children: A 10-year multicentric study

Congenital heart disease (CHD) represents a predisposing condition for the development of infective endocarditis (IE). In recent years, the reduction in the incidence of rheumatic heart disease, advances in cardiac surgery and the increased use of long-term central venous catheters in subjects without CHD have changed significantly the epidemiology of the disease. We sought to evaluate the epidemiology of EI in children in the north of France. We retrospectively included all children < 18 years hospitalized for EI in 2008–2018 in 4 tertiary centres. Demographic, underlying conditions, echocardiography, microbiology and outcomes were collected. We identified 64 episodes of IE in 60 children. Incidence was 3.07 cases for 10,000 paediatric hospitalisations ( Table 1 ). Median age was 8 years [0–13] and distribution was bimodal with peaks in infancy and adolescence. An organism pathogen was isolated in 87.5% of cases. The most common was Staphylococcus aureus (34%), then streptococcus (25%). At echocardiography, anomalies were vegetations ( n = 32), new valvular regurgitations ( n = 10), peri-valvular abscesses ( n = 2). Among cohort, 45 had a CHD (70%), 7 were preterm birth (11%), 2 had long-term central venous catheters and 10 no predisposition. No CHD group was significatively younger than CHD group. Among CHD patients, 38 (84%) had previous heart surgery and 10 presented an IE < 2 months after surgery. Pulmonary bioprosthetic valve were involved in 22 cases and mechanical valve in 3. Cardiac surgery was needed in 26 cases (41%) including valve replacement in half of them and were significatively higher in CHD group ( P < 0.05). Complications of IE occurred in 35 (58%) including emboli, mycotic aneurysm, cerebral haemorrhage and myocardial abscess. IE mostly occurred in predisposed children. Morbidity is important with neurological impairment and valvular replacement in this young population at risk for recurrence.

High maternal blood lipid levels during early pregnancy are associated with increased risk of congenital heart disease in offspring.

IntroductionThis study aimed to investigate whether maternal blood lipid levels during early pregnancy are associated with the occurrence of congenital heart disease (CHD) in their offspring.Material and methodsIn this single‐center case–control study, mothers of offspring with CHD (n = 230) and without CHD (n = 381) were included. Maternal lipid levels were determined on fasting blood samples taken in the first trimester. Relevant demographic and clinical data were extracted from the medical records. Maternal lipid profile was compared between the two groups, and regression analysis was performed to evaluate the association between lipid profile and CHD risk in offspring.ResultsCompared with the control group, levels of triglyceride, apolipoprotein‐A1, and apolipoprotein‐B in early pregnancy were significantly higher in the CHD group. Multivariate analyses showed that triglyceride (odds ratio [OR] 2.46, 95% CI 1.62–3.73, p < 0.01), total/high‐density lipoprotein cholesterol (OR 2.10, 95% CI 1.07–4.13, p = 0.03), and apolipoprotein‐A1 (OR 2.73, 95% CI 1.16–6.40, p = 0.02) were positively associated with CHD risk in offspring.ConclusionsElevated maternal lipid profile was associated with increased risk of CHD in offspring.

Association between Objectively Measured Physical Activity and Arterial Stiffness in Children with Congenital Heart Disease.

Background: The association between physical activity (PA) and arterial stiffness is particularly important in children with congenital heart disease (CHD) who are at risk for arterial stiffening. The aim of this study was to examine the association between objectively measured PA and arterial stiffness in children and adolescents with CHD. Methods: In 387 children and adolescents with various CHD (12.2 ± 3.3 years; 162 girls) moderate-to-vigorous PA (MVPA) was assessed with the “Garmin vivofit jr.” for 7 consecutive days. Arterial stiffness parameters including pulse wave velocity (PWV) and central systolic blood pressure (cSBP) were non-invasively assessed by oscillometric measurement via Mobil-O-Graph®. Results: MVPA was not associated with PWV (ß = −0.025, p = 0.446) and cSBP (ß = −0.020, p = 0.552) in children with CHD after adjusting for age, sex, BMI z-score, peripheral systolic blood pressure, heart rate and hypertensive agents. Children with CHD were remarkably active with 80% of the study population reaching the WHO recommendation of average 60 min of MVPA per day. Arterial stiffness did not differ between low-active and high-active CHD group after adjusting for age, sex, BMI z-score, peripheral systolic blood pressure, heart rate and hypertensive agents (PWV: F = 0.530, p = 0.467; cSBP: F = 0.843, p = 0.359). Conclusion: In this active cohort, no association between PA and arterial stiffness was found. Longer exposure to the respective risk factors of physical inactivity might be necessary to determine an impact of PA on the vascular system.

Neurocognitive functioning in young adults with congenital heart disease: insights from a case-control study

While there is evidence that cognitive impairment of children with congenital heart disease (CHD) may persist into adolescence, little is known about the spectrum of neurocognitive functioning of young adults with this disorder. The aim of this study was to assess neurocognitive functioning in a population of young adults with different types of CHD. Cross-sectional cohort study in young adults with CHD and a group-matched healthy control group. We assessed neurocognitive and general intellectual functioning with a comprehensive battery of standardised neuropsychological tests. In addition to task-based assessments, questionnaire data of executive dysfunctions in everyday life were measured with the Behaviour Rating Inventory of Executive Function - Adult Version. A total of 67 patients (55% men) with CHD and 55 healthy controls (51% men) were included for analysis. Mean age at assessment was 26.9 (3.68) and 26.0 (3.32) years, respectively. The CHD group performed poorer in the domains of Executive Functions, Memory, Attention & Speed, and general intellectual functioning. Patients with a CHD of severe complexity were more affected than patients with simple or moderate complexity. Behaviour Rating Inventory of Executive Function - Adult Version scores indicated that patients' self-rated deficits in behaviour regulation in everyday life was higher compared with healthy controls. Our findings indicate lower neurocognitive functioning in young adults with a CHD, particularly in those with severe defect complexity. In view of the potentially enhanced risk for cerebrovascular and neurodegenerative disease in this patient group as reported in the literature, systematic longitudinal monitoring of cognitive functioning is recommended.

Congenital Heart Diseases Impair Female Fertility.

Background: The objective of this research was to evaluate the fertility of Taiwanese women with diagnoses of congenital heart diseases (CHDs). The study also investigated how different forms of CHDs may have variously influenced fertility.Methods: We directed this nationwide, population-based and retrospective matched-cohort research by using data from the Taiwan National Health Insurance Research Database. The CHD group (n = 6602) included women with congenital structural heart diseases, aged 16–45 years in 2000. The non-CHD group (n = 6602) was matched according to urbanization and income. The outcomes, involving live birth, abortion, and fertility rates, were followed until the end of 2013. Poisson regression was used to evaluate the incidence rate ratios (IRRs).Results: The CHDs had an inferior rate of live births (IRR 0.74 [95% CI 0.71–0.78]) than the non-CHD group. There was also a lower fertility rate in the CHD group (IRR 0.81 [95% CI 0.78–0.84]) than the non-CHD group. Abortion rates between the two groups were similar.Conclusion: Congenital structural heart disease compromises female fertility, even among patients with simple forms of CHDs. It is suggested that pregnant patients with CHDs are early appeared to and advised personally with multidisciplinary care to improve their outcomes.