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.

Multimodality 3D image fusion with live fluoroscopy reduces radiation dose during catheterization of congenital heart defects.

Introduction: Imaging fusion technology is promising as it is radiation and contrast sparing. Herein, we compare conventional biplane angiography to multimodality image fusion with live fluoroscopy using two-dimensional (2D)-three-dimensional (3D) registration (MMIF) and assess MMIF impact on radiation exposure and contrast volume during cardiac catheterization of patients with congenital heart disease (CHD).

Methods: We matched institutional MMIF procedures and controls according to patient characteristics (body mass index, age, and gender) and the seven procedure-type subgroups.

Evaluation of a nurse-led multi-component transition program for adolescents with congenital heart disease.

Objective: To evaluate the effectiveness of the transition program for adolescents with congenital heart disease (CHD) 'Transition With a Heart' (TWAH) on disease-related knowledge, quality of life (QoL), transition experiences, and gaps in follow-up.

Methods: A study with pre-posttest and control group (post-test) using consecutive sampling, including adolescents with moderate to severely complex CHD, without intellectual disability, aged≥ 12 y, and parents. After weighting, t-tests were performed.

Evaluation of late cardiac effects after multisystem inflammatory syndrome in children.

Introduction: Multisystem inflammatory syndrome in children (MIS-C) is associated with important cardiovascular morbidity during the acute phase. Follow-up shows a swift recovery of cardiac abnormalities in most patients. However, a small portion of patients has persistent cardiac sequelae at mid-term.

Long-term follow-up of atrioventricular valve function in Fontan patients: effect of atrioventricular valve surgery.

Objectives: The aim of this study was to evaluate the relationship between atrioventricular valve and ventricular function in Fontan survivors, including the effect of atrioventricular valve surgery.

Methods: Analysis focused on transplant-free survival and the need for atrioventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed.

Potential of Urine Biomarkers CHI3L1, NGAL, TIMP-2, IGFBP7, and Combinations as Complementary Diagnostic Tools for Acute Kidney Injury after Pediatric Cardiac Surgery: A Prospective Cohort Study.

Acute kidney injury (AKI) is common after pediatric cardiac surgery (CS). Several urine biomarkers have been validated to detect AKI earlier. The objective of this study was to evaluate urine CHI3L1, NGAL, TIMP-2, IGFBP7, and NephroCheck as predictors for AKI ≥ 1 in pediatric CS after 48 h and AKI ≥ 2 after 12 h.

Hypertension after coarctation repair-a systematic review.

Background: Coarctation of the Aorta (CoA) leads to increased morbidity and mortality later in life despite early surgical or percutaneous treatment. Many long-term complications are related to hypertension (HT) which is a common finding late after coarctation repair.

Methods: A systematic Review was performed including articles published between February 2012 to December 2020.

Paediatric and adult congenital cardiology education and training in Europe.

Background: Limited data exist on training of European paediatric and adult congenital cardiologists.

Methods: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.

Results: Delegates from 30 countries (91%) responded.

A Reassessment of Copy Number Variations in Congenital Heart Defects: Picturing the Whole Genome.

Copy number variations (CNVs) can modulate phenotypes by affecting protein-coding sequences directly or through interference of gene expression. Recent studies in cancer and limb defects pinpointed the relevance of non-coding gene regulatory elements such as long non-coding RNAs (lncRNAs) and topologically associated domain (TAD)-related gene-enhancer interactions. The contribution of such non-coding elements is largely unexplored in congenital heart defects (CHD).

Evaluation of a no-reference image quality metric for projection X-ray imaging using a 3D printed patient-specific phantom.

Purpose: Feasability of a no-reference image quality metric was assessed on patient-like images using a patient-specific phantom simulating a frame of a coronary angiogram.

Methods: One background and one contrast-filled frame of a coronary angiogram, acquired using a clinical imaging protocol, were selected from a Philips Integris Allura FD (Philips Healthcare, Best, The Netherlands). The background frame's pixels were extruded to a thickness proportional to their grey value.

Non-invasive assessment of liver abnormalities in pediatric Fontan patients.

Liver abnormalities are well known among long-term survivors of Fontan palliation, which remains the definite surgery for complex congenital heart disease and single ventricle physiology. Pediatric data however are scarce. We assessed the prevalence and degree of liver abnormalities in pediatric Fontan patients through non-invasive investigations suitable for longitudinal follow-up.

Paediatric subaortic stenosis: long-term outcome and risk factors for reoperation.

Objectives: Surgical repair of subaortic stenosis (SAS) is associated with a substantial reoperation risk. We aimed to identify risk factors for reintervention in relation to discrete and tunnel-type SAS morphology.

Methods: Single-centre retrospective study of paediatric SAS diagnosed between 1992 and 2017.

Needs and Experiences of Adolescents with Congenital Heart Disease and Parents in the Transitional Process: A Qualitative Study.

Purpose: Most patients with congenital heart disease (CHD) need lifelong cardiac follow-up. Transitioning to adulthood and transferring to adult-focused care are often challenging. We explored the experiences and needs of adolescents with CHD and parents during the entire transitional process, including the post-transfer period.

Atrial flow regulator for drug-resistant pulmonary hypertension in a young child.

This case describes the successful implantation of an Occlutech Atrial Flow Regulator in a young child with idiopathic pulmonary hypertension and recurrent syncope despite targeted combination therapy, with subsequent relief of symptoms.

Determinants of Physical Fitness in Children with Repaired Congenital Heart Disease.

The aim of this study was to determine factors associated with physical fitness (PF) in children who underwent surgery for congenital heart disease (CHD). Sixty-six children (7-14 years) who underwent surgery for ventricular septal defect (n = 19), transposition of great arteries (n = 22), coarctation of aorta (n = 10), and tetralogy of Fallot (n = 15) were included. All children performed PF tests: cardiorespiratory fitness, upper- and lower-limb muscle strength, speed, balance, and flexibility.

Analysis of the recovery phase after maximal exercise in children with repaired tetralogy of Fallot and the relationship with ventricular function.

Background: Few studies demonstrate delayed recovery after exercise in children and adults with heart disease. We assess the recovery patterns of gas exchange parameters and heart rate (HR) in children with repaired Tetralogy of Fallot (rToF) compared to healthy peers and investigate the correlation with ventricular function and QRS duration.

Methods: 45 children after rToF and 45 controls performed a maximal incremental cardiopulmonary exercise test.

Varying Presentations of Multisystem Inflammatory Syndrome Temporarily Associated with COVID-19.

. A novel coronavirus identified in 2019 leads to a pandemic of severe acute respiratory distress syndrome with important morbidity and mortality. Initially, children seemed minimally affected, but there were reports of cases similar to (atypical) Kawasaki disease or toxic shock syndrome, and evidence emerges about a complication named paediatric inflammatory multisystem syndrome temporarily associated with SARS-CoV-2 (PIMS-TS) or multisystem inflammatory syndrome in children (MIS-C).

Myocardial disease and ventricular arrhythmia in Marfan syndrome: a prospective study.

Background: Aortic root dilatation and-dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.

Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.

Effect of aortic stiffness versus stenosis on ventriculo-arterial interaction in an experimental model of coarctation repair.

Objectives: The aim of this study was to investigate the effect of short- versus long-segment aortic stiffness and stenosis on ventriculo-arterial interaction in a porcine model of coarctation repair.

Methods: Short-long aortic stiffness was created by transection/suture [coarctation (CoA) suture, n = 6] and stenting (stent, n = 5) of the proximal descending aorta. Short-long aortic stenosis was achieved by wrapping a prosthetic graft around the aorta to 1/3-circumference reduction, over a segment length of 1 cm (CoA suture stenosis, n = 5) and 4.

The Pulmonary Autograft After the Ross Operation: Results of 25-Year Follow-Up in a Pediatric Cohort.

Background: Progressive autograft dilation and need for later reoperation remain major concerns of the Ross procedure. The study investigates the clinical outcome after the Ross operation, including a longitudinal analysis of autograft dimensions over 25 years.

Methods: From November 1991 to April 2019, 137 patients underwent a Ross procedure at the University Hospitals of UCL (Université catholique de Louvain)-Brussels and Ghent.

Endocarditis prophylaxis in daily practice of pediatricians and dentists in Flanders.

Endocarditis is a potentially life-threatening disease in children with congenital heart disease (CHD) and correct prophylaxis (EP) is of utmost importance. We conducted two surveys among pediatricians and dentists in Flanders about their knowledge of EP guidelines. The survey was completed by 910 dentists and 100 pediatricians.

A new dimension in patent foramen ovale size estimation.

Background: Detailed multidimensional assessment of patent foramen ovale (PFO) size with transesophageal echocardiography (TOE) may help to determine PFO pathogenicity in cryptogenic stroke patients. We explored the potential additive value of Live xPlane and three-dimensional (3D) TOE anatomical PFO sizing techniques.

Methods: Imaging data of 45 patients who underwent a 3D TOE-assisted percutaneous PFO closure were studied.