Adolescents with solid organ transplant: Using the BRIEF2 parent-report and self-report to measure parent-child agreement and everyday executive function.

Agreement between parent and adolescent ratings of executive function (EF) is not known in adolescents with solid organ transplant (SOT), even though pressing concerns about EF deficits are being raised in this population. The current study investigated EF in adolescents with SOT using parent and self report. Twenty-five adolescents ( = 15.

Utility of 3D printed cardiac models in congenital heart disease: a scoping review.

Objective: Three-dimensional printing (3DP) is a novel technology with applications in healthcare, particularly for congenital heart disease (CHD). We sought to explore the spectrum of use of 3D printed CHD models (3D-CM) and identify knowledge gaps within the published body of literature to guide future research.

Methods: We conducted a scoping review targeting published literature on the use of 3D-CMs.

Utility and Access to 3-Dimensional Printing in the Context of Congenital Heart Disease: An International Physician Survey Study.

Background: Three-dimensional (3D) printing is a new technology capable of producing patient-specific 3D cardiac models.

Methods: A cross-sectional survey of pediatric cardiologists was conducted. Members of the Canadian Pediatric Cardiology Association and Congenital Cardiac Interventional Study Consortium were invited to participate.

An International Multicenter Evaluation of Inheritance Patterns, Arrhythmic Risks, and Underlying Mechanisms of -Catecholaminergic Polymorphic Ventricular Tachycardia.

Background: Genetic variants in calsequestrin-2 () cause an autosomal recessive form of catecholaminergic polymorphic ventricular tachycardia (CPVT), although isolated reports have identified arrhythmic phenotypes among heterozygotes. Improved insight into the inheritance patterns, arrhythmic risks, and molecular mechanisms of -CPVT was sought through an international multicenter collaboration.

Methods: Genotype-phenotype segregation in -CPVT families was assessed, and the impact of genotype on arrhythmic risk was evaluated using Cox regression models.

Two-stage repair of interrupted aortic arch type A: Arch reconstruction and pulmonary artery banding without CPB through a left thoracotomy.

Treatment of interrupted aortic arch (IAA) is inherently challenging. Single-stage complete repair has become the norm in contemporary practice.  While palliation, in general, has fallen out of favor, a 2-stage approach to correcting selected type A IAA appears to be a productive, safe, and effective option, considering the neonatal age at repair.

Surgical Melody® valve implantation to replace the mitral valve in children.

In this video tutorial we present our technique for hybrid surgical Melody® valve implantation in the left atrio-ventricular valve (henceforth referred to as mitral valve) position in children. The key steps, including valve preparation, implantation, and balloon dilatation, are depicted. We discuss the short-term outcome, we red-flag potential complications, and we hypothesize medium-term outcomes, including late balloon dilatation.

Dysphagia and an aberrant subclavian artery: more than just a coincidence.

Aberrant subclavian artery (arteria lusoria) is the most common congenital malformation of the aortic arch in the general population. As patients are more often asymptomatic, indications for correcting this abnormality, even in those having dysphagia, are very vague. While presenting a case of a 10-year-old girl who suffered from dysphagia lusoria for 2 years before being cured by a simple surgical translocation, we review the literature and debate various aspects of its management from diagnosis to surgery and cosmesis.

Eulogy: Professor Milan Šamánek, DrSc, FESC (*09.05.1931 †29.04.2020) - legend from the East.

This article is to commemorate and celebrate achievements of Professor Milan Šamánek who passed away on 29 April, 2020. Milan was an excellent and visionary paediatric cardiologist who helped to establish paediatric cardiology as a speciality in Czechoslovakia and several other east European countries in the late 1970s. Milan was also paramount for connecting the East and West, helping in no small way to establish the Association for European Paediatric Cardiology (AEPC) as the leading learned society in Europe.

Loss of ventricular preexcitation during noninvasive testing does not exclude high-risk accessory pathways: A multicenter study of WPW in children.

Background: Abrupt loss of ventricular preexcitation on noninvasive evaluation, or nonpersistent preexcitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events.

Objective: The purpose of this study was to compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted preexcited atrial fibrillation (RC-AF) in patients with nonpersistent and persistent preexcitation.

Methods: Patients 21 years or younger with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases.

Efficacy of radiofrequency catheter ablation for premature ventricular contractions in children.

Purpose: This study evaluated the efficacy and safety of transcatheter radiofrequency ablation (RFCA) in treating ventricular premature contractions (PVCs) in children, summarized the countermeasures during intraoperative ventricular fibrillation (VF), and improved the safety of ventricular premature treatment.

Methods: A retrospective analysis was conducted on 75 children with PVCs who received RFCA in the Second Affiliated Hospital of Wenzhou Medical University from January 2010 to April 2019. Data including age, sex, body weight, ejection fraction, left ventricular end diastolic diameter, burden and number of PVCs/24 h, origin of PVCs, and its complications were collected.

Hypertension Canada's 2020 Comprehensive Guidelines for the Prevention, Diagnosis, Risk Assessment, and Treatment of Hypertension in Adults and Children.

Hypertension Canada's 2020 guidelines for the prevention, diagnosis, risk assessment, and treatment of hypertension in adults and children provide comprehensive, evidence-based guidance for health care professionals and patients. Hypertension Canada develops the guidelines using rigourous methodology, carefully mitigating the risk of bias in our process. All draft recommendations undergo critical review by expert methodologists without conflict to ensure quality.

Is left bundle branch block pattern on the ECG caused by variable ventricular activation sequence?

Background: The presence and extent of ventricular dyssynchrony are currently assessed from the QRS complex morphology and width. However, similar electrocardiography (ECG) pattern may be caused by variable ventricular activation sequence. This may then contribute to interindividually different response to cardiac resynchronization therapy (CRT).

Recent Graduates Experience Difficulty in Finding Positions Despite Apparent Need for More Pediatric Cardiologists in Canada.

There have been reports that pediatric cardiology is an increasingly competitive field and that it has become difficult for recent graduates to find employment. In Canada, empirical observations are consistent with these findings, but this has not been formally evaluated. The aim of this study was to survey pediatric cardiologists (PCs) on their perception of the current workforce in Canada.

Heart Rate Recovery After Exercise Is Associated With Arrhythmic Events in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia.

Background: Risk stratification in catecholaminergic polymorphic ventricular tachycardia remains ill defined. Heart rate recovery (HRR) immediately after exercise is regulated by autonomic reflexes, particularly vagal tone, and may be associated with symptoms and ventricular arrhythmias in patients with catecholaminergic polymorphic ventricular tachycardia. Our objective was to evaluate whether HRR after maximal exercise on the exercise stress test (EST) is associated with symptoms and ventricular arrhythmias.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018.

Initially unexplained cardiac arrest in children and adolescents: A national experience from the Canadian Pediatric Heart Rhythm Network.

Background: Unexplained cardiac arrest (UCA) is rare in children. Despite investigations, the etiology in up to one-half of patients remains unknown.

Objective: The purpose of this study was to assess the management and outcomes of pediatric UCA survivors through the Canadian Pediatric Heart Rhythm Network.

Left ventricular apical pacing in children: feasibility and long-term effect on ventricular function.

Aims: Left ventricular apical pacing (LVAP) has been reported to preserve left ventricular (LV) function in chronically paced children with complete atrioventricular block (CAVB). We sought to evaluate long-term feasibility of LVAP and the effect on LV mechanics and exercise capacity as compared to normal controls.

Methods And Results: Thirty-six consecutive paediatric patients with CAVB and LVAP in the absence (N = 22) or presence of repaired structural heart disease (N = 14, systemic LV in all) and 25 age-matched normal controls were cross-sectionally studied after a median of 3.

Pubertal Hormonal Changes and the Autonomic Nervous System: Potential Role in Pediatric Orthostatic Intolerance.

Puberty is initiated by hormonal changes in the adolescent body that trigger physical and behavioral changes to reach adult maturation. As these changes occur, some adolescents experience concerning pubertal symptoms that are associated with dysfunction of the autonomic nervous system (ANS). Vasovagal syncope (VVS) and Postural Orthostatic Tachycardia Syndrome (POTS) are common disorders of the ANS associated with puberty that are related to orthostatic intolerance and share similar symptoms.

Unexpected ischemia during transcatheter patent arterial duct closure.

A child undergoing routine transcatheter patent arterial duct closure developed severe transient ischemic changes in the electrocardiogram (Pardee waves) while the aortic retention skirt of the Amplatzer™ Duct Occluder was pulled against the duct orifice. The occluder was then released, and the delivery system was pulled back to inferior caval vein which led to electrocardiogram normalisation. Aortic root angiography showed a single coronary artery originating from the right sinus of Valsalva with the left coronary stem wedged behind the posterior aspect of the right ventricular outflow tract.

Oxidised Low-Density Lipoprotein and Its Receptor-Mediated Endothelial Dysfunction Are Associated with Coronary Artery Lesions in Kawasaki Disease.

The study aimed to investigate the role of oxidised low-density lipoprotein (oxLDL)/lectin-like-oxLDL receptor-1 (LOX-1) in coronary artery lesions (CALs) in Kawasaki disease (KD) and of plasma oxLDL concentration in the early prediction of CALs in KD. This prospective study included 80 KD patients, 20 febrile and 20 healthy children. oxLDL, LOX-1 and other parameters were analysed in the acute phase.

Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids).

Importance: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk.

Objective: To develop and validate an SCD risk prediction model that provides individualized risk estimates.

Design, Setting, And Participants: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM.

Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study.

Background: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD).

Methods: An international multicenter prospective study of NOACs in ACHD was established.