Immunosuppressive Therapy of Antibody-Mediated aHUS and TTP.

The recent classification of pediatric thrombotic microangiopathies (TMA) takes into consideration mechanisms of disease for guidance to targeted therapies. We present our experience with seven patients with antibody mediated atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP). Five children had aHUS with antibodies against complement factor H (CFH-ab) and two with TTP with antibodies against metalloproteinase ADAMTS13.

Analysis of possible risk factors for the severity of paediatric obstructive sleep apnoea syndrome.

Purpose: This study aimed to determine the effect of body mass index (BMI) percentile, asthma, sex, and age on the paediatric obstructive sleep apnoea (OSA) severity. Furthermore, to determine the possible predictive role of the BMI percentile and age in severe OSA.

Methods: This retrospective study included 921 children aged 2-18 years diagnosed with OSA by polysomnography.

Get to the heart of pediatric kidney transplant recipients: Evaluation of left- and right ventricular mechanics by three-dimensional echocardiography.

Background: Kidney transplantation (KTX) markedly improves prognosis in pediatric patients with end-stage kidney failure. Still, these patients have an increased risk of developing cardiovascular disease due to multiple risk factors. Three-dimensional (3D) echocardiography allows detailed assessment of the heart and may unveil distinct functional and morphological changes in this patient population that would be undetectable by conventional methods.

Infective endocarditis in adults with congenital heart disease: Contemporary management and related outcomes in Central and South-Eastern European region.

Background: Infective endocarditis (IE) remains major cause of morbidity and mortality in adult congenital heart disease (ACHD). Limited data exists on ACHD with IE in Central and South-Eastern European (CESEE) countries. The aim of this study is to characterize contemporary management and assess outcomes of ACHD with IE in CESEE region.

Subclinical cardiac dysfunction in pediatric kidney transplant recipients identified by speckle-tracking echocardiography.

Background: Kidney transplantation (KTx) improves prognosis in children with kidney failure; still, these patients are prone to cardiovascular damage due to multiple risk factors. Our aim was to assess myocardial structure and function in pediatric KTx by conventional and speckle-tracking echocardiography (STE) in association with established cardiovascular risk factors.

Methods: Forty-two KTx and 39 healthy age- and gender-matched children were evaluated.

Feasibility and outcome of the Rotapro system in treating severely calcified coronary lesions: The Rotapro study.

Background: The Rotapro study was conducted to evaluate the safety and feasibility of the new Rotapro rotational atherectomy system (RAS) for lesion preparation in calcified coronary artery stenosis.

Methods: Between 2015 and 2019 consecutive patients undergoing rotational atherectomy (RA) with the new Rotapro system and the conventional rotablator (Rotablator) were included from the Bad Krozingen Rotablation Registry. The primary endpoint was the incidence of in-hospital major adverse cardiovascular and cerebral event (MACCE) rate.

Effectiveness of fetal ultrasound diagnostics in cardiac malformations and association with polyhydramnios and oligohydramnios.

Background: Examine the effectiveness of prenatal ultrasound diagnostics in the detection of cardiovascular malformations, and their association with polyhydramnios and oligohydramnios.

Methods: We examined the fetal ultrasonography and postnatal clinical/fetopathological data of 372 newborns/fetuses over a 7-year period in a tertiary centre. Fetal echocardiography was performed in cases of suspected US findings between 18-32 weeks.

Supraventricular tachycardias in neonates and infants: factors associated with fatal or near-fatal outcome.

Prognosis of supraventricular tachycardias in neonates and infants is thought to be excellent with rare fatal outcomes. Nevertheless, initial management can be challenging. The aim of this study was to perform a retrospective analysis in neonates/infants with non-pos-toperative supraventricular tachycardias regarding risk factors for clinical outcome and type of antiarrhythmic drug therapy.

ADHD symptomatology of children with congenital heart disease 10 years after cardiac surgery: the role of age at operation.

Background: The aim of the present study was to investigate the differences in ADHD symptomatology between healthy controls and children who underwent cardiac surgery at different ages.

Methods: Altogether, 133 children (54 patients with congenital heart disease undergoing first cardiac surgery under 3 years of age, 26 operated at the age of 3 or later, and 53 healthy controls) were examined. Patients completed the Youth Self Report (YSR), while their parents completed the Child Behaviour Checklist (CBCL) and the ADHD Rating Scale-IV.

Association of Prolonged Fluoroscopy Time with Procedural Success of Percutaneous Coronary Intervention for Stable Coronary Artery Disease with and without Chronic Total Occlusion.

Background: In percutaneous coronary interventions (PCI), the impact of prolonged fluoroscopy time (FT) on procedural outcomes is poorly studied.

Methods And Results: We analyzed the outcomes of 12,538 consecutive elective PCIs. The primary endpoint was procedure failure (PF), the composite of technical failure, and adverse in-hospital events including all-cause death, myocardial infarction, stroke, and target vessel revascularization (MACCE), as well as pericardial tamponade.

A Novel Restorative Pulmonary Valve Conduit: Early Outcomes of Two Clinical Trials.

We report the first use of a biorestorative valved conduit (Xeltis pulmonary valve-XPV) in children. Based on early follow-up data the valve design was modified; we report on the comparative performance of the two designs at 12 months post-implantation. Twelve children (six male) median age 5 (2 to 12) years and weight 17 (10 to 43) kg, had implantation of the first XPV valve design (XPV-1, group 1; 16 mm ( = 5), and 18 mm ( = 7).

The First 5 Years of the Newest Eurotransplant Member State: Hungarian Results of International Organ Exchange From 2014 to 2018.

Introduction: Hungary joined Eurotransplant International (ET) to improve the chance of transplantation for Hungarian patients and patient outcomes, including access and graft and patient survival. After 5 years of full membership, the evaluation of numbers and quality indicators is possible.

Method: A comparison was made between 5 years prior to a preliminary cooperation agreement (2007-2011) and 5 years after full ET membership (2014-2018).

Frequency of syncope as a presenting symptom in channelopathies diagnosed in childhood. Can the multivariable EGSYS score unmask these children?

Pediatric syncope raises cardiac etiology concern as it might be the first sign of life-threatening arrhythmia syndromes. Our aim was to study the incidence of syncope as the presenting symptom in children with arrhythmia syndromes, and if known, warning signs are helpful to reveal the arrhythmic origin. All data on children with channelopathy was followed by a tertiary pediatric cardiac center between 2000 and 2018 and data were reviewed retrospectively.

Simulation Training to Improve Informed Consent and Pharmacokinetic/Pharmacodynamic Sampling in Pediatric Trials.

: Pediatric trials to add missing data for evidence-based pharmacotherapy are still scarce. A tailored training concept appears to be a promising tool to cope with critical and complex situations before enrolling the very first patient and subsequently to ensure high-quality study conduct. The aim was to facilitate study success by optimizing the preparedness of the study staff shift.

Management of Microcatheter Fracture in Complex Percutaneous Coronary Intervention With Laser Atherectomy.

Background/objective: Microcatheters and dedicated guidewires for both chronic total occlusion (CTO) and non-CTO percutaneous coronary intervention (PCI) are increasingly being used to overcome complex coronary anatomy with severe tortuosity and calcification. In extremely resistant lesions, a potential over-rotation and device fatigue may result in entrapment or even fracture causing abrupt vessel closure and hinder successful revascularization.

Method/result: We demonstrate two consecutive cases complicated with dislodged microcatheter tip which was rescued uniquely with coronary laser atherectomy.

Recommendations from the Association for European Paediatric and Congenital Cardiology for training in diagnostic and interventional electrophysiology.

The field of electrophysiology (EP) in paediatric cardiology patients and adults with congenital heart disease is complex and rapidly growing. The current recommendations for diagnostic and invasive electrophysiology of the working group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Paediatric and Congenital Cardiology acknowledges the diveristy of European countries and centers. These training recommendations can be fulfilled in a manageable period of time, without compromising the quality of training required to become an expert in the field of paediatric and congenital EP and are for trainees undergoing or having completed accredited paediatric cardiologist fellowship.

Clinical characteristics and outcomes of patients with adult congenital heart disease listed for heart and heart‒lung transplantation in the Eurotransplant region.

Background: The therapeutic success in patients with congenital heart disease (CHD) leads to a growing number of adults with CHD (adult CHD [ACHD]) who develop end-stage heart failure. We aimed to determine patient characteristics and outcomes of ACHD listed for heart transplantation.

Methods: Using data from all the patients with ACHD in 20 transplant centers in the Eurotransplant region from 1999 to 2015, we analyzed patient characteristics, waiting list, and post-transplantation outcomes.

Distance measurement for pulse wave velocity estimation in pediatric age: Comparison with intra-arterial path length.

Background And Aims: Central pulse wave velocity (PWV) is a marker of arterial stiffness and is calculated by dividing the pulse wave travel distance by the transit time. However, there is no consensus as to the ideal distance measurement in children. The aim of our study was to identify the more reliable method to assess the distance measurement in the pediatric age.

Evaluation of a child with suspected nephrolithiasis.

Purpose Of Review: As the incidence of nephrolithiasis in children doubles every 10 years it is becoming a common disease associated with significant morbidity along with considerable economic burden worldwide. The aim of this review is to summarize current data on the epidemiology and causes of renal stones in children and to provide a frame for the first clinical evaluation of a child with suspected nephrolithiasis.

Recent Findings: Dietary and environmental factors are the driving force of changing epidemiology.

Prospective evaluation of iatrogenic atrial septal defect after cryoballoon or radiofrequency catheter ablation of atrial fibrillation-"EVITA" study.

Purpose: Iatrogenic atrial septal defect (IASD) after catheter ablation (CA) for atrial fibrillation (AF) due to transseptal puncture (TSP) can occur. The aim of this prospective study was to describe the incidence of IASD and to detect any cerebrovascular accident (CVA) after radiofrequency (RF) and cryoballoon (CB) CA.

Methods: Between July 2014 and September 2016, 94 patients (pts) (RF; 48, CB; 46, 30 (31.

Orodispersible minitablets of enalapril for use in children with heart failure (LENA): Rationale and protocol for a multicentre pharmacokinetic bridging study and follow-up safety study.

Introduction: Treatment of paediatric heart failure is based on paradigms extensively tested in the adult population assuming similar underlying pathophysiological mechanisms. Angiotensin converting enzyme inhibitors (ACEI) like enalapril are one of the cornerstones of treatment and commonly used off-label in children. Dose recommendations have been extrapolated from adult experience, but the relationship between dose and pharmacokinetics (PK) in (young) children is insufficiently studied.