Indications and management of implantable cardioverter-defibrillator therapy in childhood hypertrophic cardiomyopathy.

Sudden cardiac death is the most common mode of death during childhood and adolescence in hypertrophic cardiomyopathy, and identifying those individuals at highest risk is a major aspect of clinical care. The mainstay of preventative therapy is the implantable cardioverter-defibrillator, which has been shown to be effective at terminating malignant ventricular arrhythmias in children with hypertrophic cardiomyopathy but can be associated with substantial morbidity. Accurate identification of those children at highest risk who would benefit most from implantable cardioverter-defibrillator implantation while minimising the risk of complications is, therefore, essential.

Multicenter cohort study on duration of antiarrhythmic medication for supraventricular tachycardia in infants.

Antiarrhythmic medication (AM) is commonly used to prevent supraventricular tachycardia (SVT) recurrence in infants. Our aim was to determine whether a shorter duration of AM is sufficient to prevent atrioventricular reentrant tachycardia (AVRT) recurrence and evaluate risk factors for recurrence of SVT after discontinued AM.This multicenter cohort study included all infants diagnosed with SVT in the five university hospitals in Finland between 2005 and 2017.

Institutional transition from invasive to non-invasive imaging in children with univentricular heart defects: safety and cost savings.

Objectives: Patients with univentricular heart defects require lifelong imaging surveillance. Recent advances in non-invasive imaging have enabled replacing these patients' routine catheterisation. Our objective was to describe the safety and cost savings of transition of a tertiary care children's hospital from routine invasive to routine non-invasive imaging of low-risk patients with univentricular heart defects.

Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study.

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns.

Methods: An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands.

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.

Long-term outcome after treatment of pulmonary atresia with ventricular septal defect: nationwide study of 109 patients born in 1970-2007.

Objectives: Treatment of pulmonary atresia with ventricular septal defect (PA + VSD) has evolved during recent decades, but it still remains challenging. This study evaluated 41-year experience of outcome, survival and treatment of PA + VSD patients.

Methods: Patient records and angiograms of 109 patients with PA + VSD born in Finland between 1970 and 2007, and treated at the Children's Hospital, Helsinki University Central Hospital, were retrospectively analysed in this nationwide study.

Follow-up of 316 molecularly defined pediatric long-QT syndrome patients: clinical course, treatments, and side effects.

Background: Inherited long-QT syndrome (LQTS) is associated with risk of sudden death. We assessed the clinical course and the fulfillment of current treatment strategies in molecularly defined pediatric LQTS type 1 and (LQT1) and type 2 (LQT2) patients.

Methods And Results: Follow-up data covering a mean of 12 years were collected for 316 genotyped LQT1 and LQT2 patients aged 0 to 18 years.

Population-based single-center outcome for pediatric catheter ablation of common supraventricular tachycardias.

Background: Catheter ablation has become the preferred treatment for common supraventricular tachycardia (SVT) in children and adolescents, but long-term follow-up data on pediatric patients remain limited.

Methods: To provide follow-up data, we created a population-based prospective pediatric catheter ablation registry in 1997. All patients underwent clinical follow-up at 1 month and 1 year after the procedure.

[Myocarditis in children--a diagnostic and therapeutic challenge].

Viral infections are the most common causes of myocarditis in children. Chronic myocardial injury may develop following an immune or autoimmune reaction triggered or maintained by an infection, or can be part of a systemic autoimmune disease. Although many of the children having developed myocarditis are symptomless, initial symptoms may include cardiac insufficiency, arrhythmias and sudden death.

Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement.

In children with structurally normal hearts, the mechanisms of arrhythmias are usually the same as in the adult patient. Some arrhythmias are particularly associated with young age and very rarely seen in adult patients. Arrhythmias in structural heart disease may be associated either with the underlying abnormality or result from surgical intervention.

[Cryoablation in the treatment of cardiac arrhythmias].

Catheter ablation is currently the preferred treatment for various arrhythmias. Radiofrequency ablation has been shown to be efficacious and safe. Cryoablation provides better ablation catheter stability and reduces the risk of an inadvertent atrioventricular (AV) block when treating arrhythmia substrates near the normal conduction system.

Long-term performance of beat-to-beat automatic ventricular threshold adjustment in infants with congenital atrioventricular block.

Background: AutoCapture™ (AC) of St. Jude Medical (SJM; St. Paul, MN, USA) pacemakers provides beat-to-beat ventricular capture verification and allows low-amplitude pacing.

Intravenous epinephrine infusion test in diagnosis of catecholaminergic polymorphic ventricular tachycardia.

Introduction: A test involving intravenous infusion of epinephrine has been proposed as a method alternative to exercise stress test in diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT). We aimed at estimating the predictive value of intravenous epinephrine administration in CPVT patients with frequent exercise-induced ventricular ectopy.

Methods And Results: We recruited 81 subjects, including 25 CPVT-linked ryanodine receptor 2 (RYR2) mutation carriers, 11 genetically undefined CPVT patients, and 45 unaffected family members.

Junctional ectopic tachycardia after surgery for congenital heart disease: incidence, risk factors and outcome.

Objectives: Junctional ectopic tachycardia (JET) is a serious, haemodynamically compromising tachyarrhythmia associated with paediatric cardiac surgery, with a reported mortality up to 14%. The incidence, risk factors and outcome of this tachyarrhythmia were evaluated in this population-based, case-control patient cohort.

Methods: A total of 1001 children, who underwent open-heart surgery during a 5-year period, were retrospectively analysed.

Normal growth and intravascular volume status with good metabolic control during peritoneal dialysis in infancy.

The most demanding patient population on peritoneal dialysis (PD) consists of children under 2 years of age. Their growth is inferior to that of older children and maintaining euvolemia is difficult, especially in anuric patients. In this prospective study reported here, we enrolled 21 patients <2 years of age (mean 0.

Automatic atrial threshold measurement and adjustment in pediatric patients.

Background: Automatic threshold measurement and output adjustment are used as default settings in modern pacemakers. The purpose of the study was to assess Atrial Capture Management (ACM) of Medtronic pacemakers in pediatric patients.

Methods: Forty children were enrolled in two centers.

Excellent functional result in children after correction of anomalous origin of left coronary artery from the pulmonary artery--a population-based complete follow-up study.

Surgical strategy to construct a two-coronary system for a patient with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) has evolved with time. Limited long-term follow-up data are available on these children. We report population-based follow-up in children operated on for ALCAPA.

Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates.

Background: Cardiac resynchronisation therapy (CRT) is increasingly used in children in a variety of anatomical and pathophysiological conditions, but published data are scarce.

Objective: To record current practice and results of CRT in paediatric and congenital heart disease.

Design: Retrospective multicentre European survey.

Atresia of proximal coronary arteries in pulmonary atresia with intact ventricular septum - fetal and neonatal findings.

Pulmonary atresia with intact ventricular septum (PA+IVS) is a rare congenital cardiac malformation which is associated with ventriculocoronary arterial communications from the right ventricle. We present a case of PA+IVS with a bilateral atresia of the coronary ostia, and thus, a completely right ventricular-dependent coronary circulation followed up by fetal echocardiography. Eventually the infant died of myocardial infarction at 2 days of age.

Automatic ventricular threshold measurement in children with epicardial pacing leads.

Background: The aim of the study was to evaluate the safety and reliability of automatic ventricular pacing threshold measurement, the Medtronic Capture Management (CM), in children with epicardial pacing leads. CM has not been recommended for use with epicardial leads due to lack of pertinent data.

Methods: During a 2-year study period, 34 children (mean age 6.

Increased QT dispersion and loss of sinus rhythm as risk factors for late sudden death after Mustard or Senning procedures for transposition of the great arteries.

Increased QT dispersion and loss of sinus rhythm were both associated with sudden cardiac death in patients with transposition of the great arteries after Mustard and Senning operations at the early and late postoperative periods. A combination of increased QT dispersion with loss of sinus rhythm increases the positive predictive value for sudden cardiac death in patients with transposition of the great arteries after Mustard and Senning procedures.

Hypertension, cardiac state, and the role of volume overload during peritoneal dialysis.

The cardiac state and the prevalence of high blood pressure (BP) were analyzed in 21 pediatric patients (mean age 5.3 +/- 5.3 years) on chronic peritoneal dialysis (CPD), the aim being to specify the impact of hypervolemia in the etiology of hypertension.