Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations.

Background: Lymphatic disorders such as protein-losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers.

Clinical Examples of the Additive Value of Absolute Quantification of Cell-Free DNA After Heart Transplantation.

Objective: Cell-free DNA (cfDNA) is used as a biomarker after transplantation to detect graft injury, relying on the donor fraction (DF). We have established a PCR-based approach allowing us to separately quantify absolute values of dd-cfDNA and recipient-derived cfDNA (rd-cfDNA). We aimed to present typical clinical scenarios after heart transplantation (HTx) to illustrate the advantages of absolute cfDNA values over DF.

Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study.

Background: Long-term survival after single-ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported.

Methods And Results: This nationwide cohort study included all children undergoing operation with single-ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations.

Organisation of paediatric echocardiography laboratories and governance of echocardiography services and training in Europe: current status, disparities, and potential solutions. A survey from the Association for European Paediatric and Congenital Cardiology (AEPC) imaging working group.

Background: There is limited data on the organisation of paediatric echocardiography laboratories in Europe.

Methods: A structured and approved questionnaire was circulated across all 95 Association for European Paediatric and Congenital Cardiology affiliated centres. The aims were to evaluate: (1) facilities in paediatric echocardiography laboratories across Europe, (2) accredited laboratories, (3) medical/paramedical staff employed, (4) time for echocardiographic studies and reporting, and (5) training, teaching, quality improvement, and research programs.

Reducing windmill artifacts in clinical spiral CT using a deep learning-based projection raw data upsampling: Method and robustness evaluation.

Background: Multislice spiral computed tomography (MSCT) requires an interpolation between adjacent detector rows during backprojection. Not satisfying the Nyquist sampling condition along the z-axis results in aliasing effects, also known as windmill artifacts. These image distortions are characterized by bright streaks diverging from high contrast structures.

Absolute Quantification of Donor-Derived Cell-Free DNA in Pediatric and Adult Patients After Heart Transplantation: A Prospective Study.

In this prospective study we investigated a cohort after heart transplantation with a novel PCR-based approach with focus on treated rejection. Blood samples were collected coincidentally to biopsies, and both absolute levels of dd-cfDNA and donor fraction were reported using digital PCR. 52 patients (11 children and 41 adults) were enrolled (NCT03477383, clinicaltrials.

Outcomes in neonatal critical and non-critical aortic stenosis: a retrospective cohort study.

Objective: To compare long-term survival, reinterventions and risk factors using strict definitions of neonatal critical and non-critical valvular aortic stenosis (VAS).

Design: A nationwide retrospective study using data from patient files, echocardiograms and the Swedish National Population Registry.

Setting And Patients: All neonates in Sweden treated for isolated VAS 1994-2018.

Treatment decision in a 4-year-old-boy with left ventricular outpouching after advanced hemodynamical flow evaluation with 4Dflow CMR: A case report.

Background: The present study presents a diagnostic course for the characterization of a congenital left ventricular outpouching (LVO) in a 4-year-old boy with severe neonatal heart failure, evaluating the added value of cardiac magnetic resonance (CMR) 4Dflow.

Case Presentation: A boy, born at full term, presented with heart failure immediately after birth. Echocardiography showed dilated left ventricle with poor function and LVO was initially interpreted as an aneurysm.

Congenital heart disease does not entail an increased risk for severe COVID-19.

Aim: To analyse the risk of developing serious disease or death due to COVID-19 among patients who underwent heart surgery during childhood.

Methods: A retrospective combined register and patient file study. We identified all individuals who had undergone surgery for congenital heart disease in childhood between 1994 and 2019 in our Local Surgical Register that covers half of the Swedish population.

Life satisfaction, health-related quality of life and physical activity after treatment for valvular aortic stenosis.

Objective: To investigate health-related quality of life and life satisfaction in children and adolescents treated for isolated congenital valvular aortic stenosis compared to healthy peers. Our second aim was to investigate the relationship between objectively measured physical activity, health-related quality of life and life satisfaction in the same group.

Methods: Forty-eight patients, 8-18 years of age, were recruited, as well as 43 healthy peers matched for age, gender and residential area.

Children and Adolescents Treated for Valvular Aortic Stenosis Have Different Physical Activity Patterns Compared to Healthy Controls: A Methodological Study in a National Cohort.

Previous research in children and adolescents with congenital heart defects presents contradictory findings concerning their physical activity (PA) level, due to methodological limitations in the PA assessment. The aim of the present cross-sectional study was to compare PA in children and adolescents treated for valvular aortic stenosis with healthy controls using an improved accelerometer method. Seven-day accelerometer data were collected from the hip in a national Swedish sample of 46 patients 6-18 years old treated for valvular aortic stenosis and 44 healthy controls matched for age, gender, geography, and measurement period.

Outcome after surgery for pulmonary atresia with ventricular septal defect, a long-term follow-up study.

Aim: To study the long-term outcome after surgery for pulmonary atresia and ventricular septal defect (PA-VSD), and to determine association between the contribution of major aorto-pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death.

Methods: Patients who had undergone surgery for PA-VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross-checked against the Swedish National Population Register.

A national study of the outcome after treatment of critical aortic stenosis in the neonate.

Objective: This study describes short-term and long-term outcome after treatment of critical valvular aortic stenosis in neonates in a national cohort, with surgical valvotomy as first choice intervention.

Methods: All neonates in Sweden treated for critical aortic stenosis between 1994 and 2016 were included. Patient files were analysed and cross-checked against the Swedish National Population Registry as of December 2017, giving complete survival data.

Outpatient volumes and medical staffing resources as predictors for continuity of follow-up care during transfer of adolescents with congenital heart disease.

Background: Providing continuous follow-up care to patients with congenital heart disease (CHD) remains a challenge in many settings. Previous studies highlight that patients with CHD experience discontinuation of follow-up care, but mainly describe a single-centre perspective, neglecting inter-institutional variations. Hospital-related factors above and beyond patient-related factors are believed to affect continuity of care.

Pacemaker treatment after Fontan surgery-A Swedish national study.

Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indications in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.

Survey of 23 Nordic university hospitals showed that 77% lacked written procedures for measuring and interpreting blood pressure in infants.

Aim: This study determined the use of standardised procedures for infant noninvasive blood pressure (NIBP) measurements in the Nordic countries and aimed to identify factors included in the standardisation and interpretation of NIBP measurements in infants.

Methods: A cross-sectional electronic questionnaire survey was sent to 84 physicians in all 23 university hospitals in Sweden, Norway, Denmark, Finland and Iceland and was completed from February to March 2017. The survey contained respondent characteristics, the presence and description of standardised procedures for NIBP measurements, daily practice of NIBP measurements and methodological considerations and interpretation of NIBP measurements in a healthy six-month-old child.

Treatment of valvular aortic stenosis in children: a 20-year experience in a single institution.

Objectives: This study presents short- and long-term follow-up after treatment for isolated valvular aortic stenosis in children with surgical valvotomy as the preferred 1st intervention.

Methods: All patients aged 0-18 years treated between 1994 and 2013 at our centre were reviewed regarding the mode of first treatment, mortality, reinterventions and the need for aortic valve replacement.

Results: A total of 113 patients were identified in local registries.

The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis.

Aim: The aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and noncompaction of the left ventricle (LVNC) in Swedish children.

Methods: We reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular noncompaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015.

Results: In total, 69 cases (61% males) were identified.

Do Self- and Proxy Reports of Cognitive Problems Reflect Intellectual Functioning in Children and Adolescents with Congenital Heart Defects?

Aim: Children with congenital heart defects (CHD) who suffer from cognitive impairments and school difficulties need to be identified as early as possible in order to set appropriate interventions in place that may enhance the school situation and quality of life for these children. Identifying children and adolescents at risk for cognitive difficulties requires specific screening tools. This study assessed such a tool - Pediatric Quality of Life Inventory Cardiac Module subscale: Cognitive Problems - to investigate whether proxy reported and self-reported cognitive problems were associated with measured intellectual functioning in children and adolescents with CHD treated with surgery or by catheter interventions.

Intellectual Functioning in Children with Congenital Heart Defects Treated with Surgery or by Catheter Interventions.

Background: Studies suggest that children with congenital heart defects (CHD) are at risk for adverse intellectual functioning. However, factors related to lower intellectual functioning in this group are largely unknown. This study describes intellectual functioning in children with CHD in relation to severity of the heart defect, the child's age, and the socioeconomic status of the family (SES).

Respiratory Tract Infection and Risk of Hospitalization in Children with Congenital Heart Defects During Season and Off-Season: A Swedish National Study.

Respiratory tract infections (RTI) are common among young children, and congenital heart defect (CHD) is a risk factor for severe illness and hospitalization. This study aims to assess the relative risk of hospitalization due to RTI in winter and summer seasons for different types of CHD. All children born in Sweden and under the age of two, in 2006-2011, were included.

Novel iterative reconstruction method with optimal dose usage for partially redundant CT-acquisition.

In CT imaging, a variety of applications exist which are strongly SNR limited. However, in some cases redundant data of the same body region provide additional quanta. Examples in dual energy CT, the spatial resolution has to be compromised to provide good SNR for material decomposition.

Altered expression of autoimmune regulator in infant down syndrome thymus, a possible contributor to an autoimmune phenotype.

Down syndrome (DS), caused by trisomy of chromosome 21, is associated with immunological dysfunctions such as increased frequency of infections and autoimmune diseases. Patients with DS share clinical features, such as autoimmune manifestations and specific autoantibodies, with patients affected by autoimmune polyendocrine syndrome type 1. Autoimmune polyendocrine syndrome type 1 is caused by mutations in the autoimmune regulator (AIRE) gene, located on chromosome 21, which regulates the expression of tissue-restricted Ags (TRAs) in thymic epithelial cells.