Neonatal sepsis and cardiovascular dysfunction I: mechanisms and pathophysiology.

The highest incidence of sepsis across all age groups occurs in neonates leading to substantial mortality and morbidity. Cardiovascular dysfunction frequently complicates neonatal sepsis including biventricular systolic and/or diastolic dysfunction, vasoregulatory failure, and pulmonary arterial hypertension. The haemodynamic response in neonatal sepsis can be hyperdynamic or hypodynamic and the underlying pathophysiological mechanisms are heterogeneous.

Multistakeholder Recommendations for Supporting Patients and Families Transitioning From Paediatric to Adult Congenital Heart Disease Care.

Background: Transitioning from paediatric to adult congenital heart disease (CHD) care is a high-risk time for being lost to follow-up. Existing CHD transition programmes have not included patients, caregivers, and health care providers as partners in their development. This study aimed to develop recommendations for a CHD transition programme driven by lived and clinical experiences.

20-Year follow-up and comparison of valved conduits used for right ventricular outflow tract reconstruction: single-centre, propensity score match analysis.

Objectives: Surgical repair of complex congenital heart defects with hypoplasia or atresia of the right ventricular outflow tract (RVOT) may require pulmonary valve implantation or replacement during the primary repair or reoperation. The purpose of this study is to evaluate the outcomes of cryopreserved homografts, bovine jugular vein conduits and decellularized Matrix P Plus N conduits in patients undergoing RVOT reconstruction at a single centre.

Methods: Retrospective, single-centre review of 173 patients with 199 conduits undergoing right ventricle-to-pulmonary artery reconstruction with valved conduit from 2002 to 2022.

Flecainide Is Associated With a Lower Incidence of Arrhythmic Events in a Large Cohort of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia.

Background: In severely affected patients with catecholaminergic polymorphic ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this study was to evaluate whether flecainide is associated with a lower incidence of arrhythmic events (AEs) when added to beta-blockers in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia.

Methods: From 2 international registries, this multicenter case cross-over study included patients with a clinical or genetic diagnosis of catecholaminergic polymorphic ventricular tachycardia in whom flecainide was added to beta-blocker therapy.

Establishing a nationwide pediatric heart transplantation program with mid-term results comparable to worldwide data - The Czech experience.

Background: Heart transplantation (HTx) is an established therapeutic option for children with end-stage heart failure. Comprehensive pediatric nationwide HTx program was introduced in 2014 in the Czech Republic. The aim of this study was to evaluate its mid-term characteristics and outcomes and to compare them with international data.

Pregnancy Termination and Postnatal Major Congenital Heart Defect Prevalence After Introduction of Prenatal Cardiac Screening.

Importance: Prenatal cardiac screening of the first and second trimesters has had a major impact on postnatal prevalence of congenital heart defects (CHDs), rates of termination of pregnancy (TOP), and outcomes among children born alive with CHDs.

Objective: To examine the prenatal and postnatal incidence of major CHDs (ie, necessitating intervention within the first year of life), detection rate trends, rates of TOP, and the association of cardiac screening with postnatal outcomes.

Design, Settings, And Participants: In this cross-sectional study, 3827 fetuses with antenatally diagnosed major CHDs in the Czech Republic (population 10.

Advancing the science of management of arrhythmic disease in children and adult congenital heart disease patients within the last 25 years.

This review article reflects how publications in EP Europace have contributed to advancing the science of management of arrhythmic disease in children and adult patients with congenital heart disease within the last 25 years. A special focus is directed to congenital atrioventricular (AV) block, the use of pacemakers, cardiac resynchronization therapy devices, and implantable cardioverter defibrillators in the young with and without congenital heart disease, Wolff-Parkinson-White syndrome, mapping and ablation technology, and understanding of cardiac genomics to untangle arrhythmic sudden death in the young.

Sudden cardiac arrest in a child with Gitelman syndrome: a case report and literature review.

Salt-losing tubulopathies are well-recognised diseases predisposing to metabolic disturbances in affected patients. One of the most severe complications can be life-threatening arrhythmias causing sudden cardiac arrest. We present here the first case of a pediatric patient with Gitelman syndrome associated sudden cardiac arrest without precipitating event.

Long-Term Results of Congenital Aortic Stenosis Treatment in the Era of Percutaneous Balloon Valvuloplasty: Up to 33 Years Follow-Up.

Background The goal of this study was to evaluate long-term results of percutaneous balloon valvuloplasty (BVPL) used exclusively for initial management of congenital aortic stenosis in children. Methods and Results A total of 409 consecutive pediatric patients (134 newborns, 275 older patients) who underwent BVPL as initial treatment of aortic stenosis in a single nationwide pediatric center were subjected to a retrospective follow-up study. The resulting follow-up time reached a median of 18.

Post-mortem genetic testing in sudden cardiac death and genetic screening of relatives at risk: lessons learned from a Czech pilot multidisciplinary study.

Sudden cardiac death (SCD) might have an inherited cardiac condition background. Genetic testing supports post-mortem diagnosis and screening of relatives at risk. Our aim is to determine the feasibility of a Czech national collaboration group and to establish the clinical importance of molecular autopsy and family screening.

Management of Inherited Arrhythmia Syndromes: A HiRO Consensus Handbook on Process of Care.

Inherited arrhythmia syndromes are rare genetic conditions that predispose seemingly healthy individuals to sudden cardiac arrest and death. The Hearts in Rhythm Organization is a multidisciplinary Canadian network of clinicians, researchers, patients, and families that aims to improve care for patients and families with inherited cardiac conditions, focused on those that confer predisposition to arrhythmia and sudden cardiac arrest and/or death. The field is rapidly evolving as research discoveries increase.

Natural History of Nonsurgical Complete Atrioventricular Block in Children and Predictors of Pacemaker Implantation.

Background: Data on the natural history of complete atrioventricular block (CAVB) in children are scarce, and criteria for pacemaker (PM) implantation are based on low levels of evidence.

Objectives: This study aimed to evaluate the natural course and predictors of PM implantation in a nationwide cohort of pediatric patients with nonsurgical CAVB.

Methods: All children with CAVB in the absence of structural heart disease presenting from 1977 to 2016 were retrospectively identified, yielding 95 subjects with a mean age of 4.

Clinical experience of reoperative right ventricular outflow tract reconstruction with valved conduits: risk factors for conduit failure in long-term follow-up.

Reconstruction of right ventricular outflow tract in patients with congenital heart disease in various age groups remains a controversial issue. Currently, a little is known about the fate of secondary and subsequent conduit. The aim of the study was to determine risk factors of conduit failure, evaluate long-term conduit survival, find out which type of conduit should be preferred in case of reoperations.

Effectiveness of the STEPSTONES Transition Program for Adolescents With Congenital Heart Disease-A Randomized Controlled Trial.

Purpose: Adolescents with congenital heart disease transition from childhood to adulthood and transfer from pediatric-oriented to adult-oriented care. High-level empirical evidence on the effectiveness of transitional care is scarce. This study investigated the empowering effect (primary outcome) of a structured person-centered transition program for adolescents with congenital heart disease and studied its effectiveness on transition readiness, patient-reported health, quality of life, health behaviors, disease-related knowledge, and parental outcomes e.

Exceptional case of aortic atresia associated with atypical double aortic arch.

We present the unique case of atretic aortic valve associated with hypoplastic ascending aorta and double aortic arch of unusual configuration supplying retrograde blood flow to the coronary arteries.

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.

Management of Heart Failure With Arrhythmia in Adults With Congenital Heart Disease: JACC State-of-the-Art Review.

Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, therapeutic, and prognostic overlap between them demands increased recognition. In fact, given the near ubiquity of heart failure and arrhythmia among ACHDs, there is perhaps no other arena within cardiology where this critical intersection is more frequently observed.

Safety issues with an inter-hospital transport of a patient with a Berlin Heart Excor biventircular assist device.

Background: Interhospital transfers of pediatric patients on the Berlin Heart Excor have been published on an occasional basis.

Methods: Nowadays medicine evolves away from just feasibility towards quality and safety issues. Management tools like risk analysis have found their way into clinical practice.

Surgical correction of partial anomalous left superior pulmonary venous connection to the innominate vein.

Partial anomalous pulmonary venous connection is reported to occur in 0.4 to 0.7% of children.

Screening of Coronary Artery Origin by Echocardiography: Definition of Normal (and Abnormal) Take-Off by Standard Echocardiographic Views in a Healthy Pediatric Population.

Background: Echocardiographic screening of anomalous coronary artery origin is of increasing interest for children participating in sport activities. However, criteria to define normal coronary artery origins in children are poorly defined. Thus, the aim of the present investigation is to define the normal origin and angle of emergence of coronary arteries by echocardiography in healthy children.