Doppler Echocardiography Assessment of Aortic Stiffness in Female Adolescents with Anorexia Nervosa.

Background: Anorexia nervosa (AN) is associated with abnormalities in biomarkers of cardiovascular risk. Arterial stiffness, as measured by pulse-wave velocity (PWV), is also a risk factor for cardiovascular disease. The aims of this study were to determine the stiffness of the aorta in female adolescents with AN and to determine if either the severity or the type of AN was associated with PWV.

Intimal thickening at coronary bifurcations in pediatric heart transplant recipients.

Heart transplant recipients are at increased risk for atherosclerosis and cardiac allograft vasculopathy, both initially presenting as intimal thickening. We aimed to determine the presence, extent, and anatomical characteristics of intimal thickness at coronary bifurcations in children using OCT. We measured the intimal thickness of coronary arteries in pediatric transplant recipients using OCT during routine cardiac catheterization.

The accessibility and utilization of genetic testing for inherited heart rhythm disorders: a Canadian cross-sectional survey study.

The genetic basis of many sudden death-related conditions has been elucidated. These include inherited arrhythmias and arrhythmogenic cardiomyopathies, termed inherited heart rhythm disorders (IHRD). Advising on and interpreting genetic testing is challenging for the general cardiologist.

Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study.

Objectives: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS).

Methods: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations.

The Safety and Effectiveness of Flecainide in Children in the Current Era.

This retrospective study sought to determine the safety and effectiveness of flecainide in children with normal hearts and those with congenital heart disease (CHD) or cardiomyopathy (CMO). Baseline and follow-up data at two pediatric cardiology sites were queried (2000-2015); a total of 175 patients (20 with CHD and two with CMO) receiving flecainide were assessed. When comparing patients with CHD to those with normal hearts, patients with CHD were younger at diagnosis (median age 19 days; IQR 3-157.

A novel treadmill protocol for exercise testing in children: the British Columbia Children's Hospital protocol.

Background: Exercise testing in children is widely recommended for a number of clinical and prescriptive reasons. Many institutions continue to use the Bruce protocol for treadmill testing; however, with its incremental changes in speed and grade, it has challenges for practical application in children. We have developed a novel institutional protocol (British Columbia Children's Hospital (BCCH)), which may have better utility in paediatric populations.

A Systematic Review of Infective Endocarditis in Patients With Bovine Jugular Vein Valves Compared With Other Valve Types.

Objectives: The aim of this study was to systematically evaluate the incidence of infective endocarditis (IE) in right ventricle-to-pulmonary artery conduits and valves, comparing bovine jugular vein (BJV) valves with all others.

Background: Recent evidence suggests that the incidence of IE is higher in patients with congenital heart disease who have undergone implantation of BJV valves in the pulmonary position compared with other valves.

Methods: Systematic searches of published research were conducted using electronic databases (MEDLINE, Embase, and CINAHL) and citations cross-referenced current to April 2016.

Left-Sided Reoperations After Arterial Switch Operation: A European Multicenter Study.

Background: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type.

Methods: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.

Congenital arch vessel anomalies in CHARGE syndrome: A frequent feature with risk for co-morbidity.

Background: CHARGE syndrome is a complex multiple congenital malformation disorder with variable expression that is caused by mutations in the gene. Variable heart defects occur in 74% of patients with a mutation, with an overrepresentation of atrioventricular septal defects and conotruncal defects - including arch vessel anomalies.

Methods And Results: We report an index patient with an arch vessel anomaly underlying serious feeding problems that resolved after arch vessel surgery.

A Doppler Echocardiographic Study of the Myocardial Inotropic Response to Peak Semisupine Exercise in Healthy Children: Development of a Simplified Index of Myocardial Reserve.

Background: Stress echocardiography has been advocated for the detection of abnormal myocardial function and unmasking diminished myocardial reserve in pediatric patients. The aim of this study was to create a simplified index of myocardial reserve, derived from the myocardial inotropic response to peak semisupine exercise in healthy children, and illustrate its applicability in a sample of pediatric oncology patients.

Methods: In this prospective analysis, children (7-18 years of age) with normal cardiac structure and function performed semisupine stress echocardiography to volitional fatigue.

Neonates with critical congenital heart defects: Impact of fetal diagnosis on immediate and short-term outcomes.

Background: Fetal echocardiography is being increasingly used for prenatal diagnosis of congenital cardiac malformations, but its impact on the neonatal outcomes in low- and middle-income countries is still unknown.

Aims: The objective of this study is to determine the impact of fetal echocardiography on immediate postnatal and short-term outcome in a tertiary pediatric cardiac center.

Study Design: This is a prospective study.

Beyond the Electrocardiogram: Mutations in Cardiac Ion Channel Genes Underlie Nonarrhythmic Phenotypes.

Cardiac ion channelopathies are an important cause of sudden death in the young and include long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, idiopathic ventricular fibrillation, and short QT syndrome. Genes that encode ion channels have been implicated in all of these conditions, leading to the widespread implementation of genetic testing for suspected channelopathies. Over the past half-century, researchers have also identified systemic pathologies that extend beyond the arrhythmic phenotype in patients with ion channel gene mutations, including deafness, epilepsy, cardiomyopathy, periodic paralysis, and congenital heart disease.

Midterm results of the Ross procedure in children: an appraisal of the subannular implantation with interrupted sutures technique.

Objectives: The support of the pulmonary autograft root by the fibromuscular left ventricular outflow tract is emphasized to address the concern related to the dilatation of the pulmonary autograft structures in the paediatric population.

Methods: This retrospective study analyses the outcomes of 75 children who were operated between 1998 and 2012 with the subannular interrupted sutures technique at a median age of 10.2 years (range, 5.

Validity and reliability of the Physical Activity Questionnaire for Children (PAQ-C) and Adolescents (PAQ-A) in individuals with congenital heart disease.

Objective: To assess the criterion validity, internal consistency, reliability and cut-point for the Physical Activity Questionnaire for Children (PAQ-C) and Adolescents (PAQ-A) in children and adolescents with congenital heart disease-a special population at high cardiovascular risk in whom physical activity has not been extensively evaluated.

Methods: We included 84 participants (13.6±2.

Biomarkers for Early Acute Kidney Injury Diagnosis and Severity Prediction: A Pilot Multicenter Canadian Study of Children Admitted to the ICU.

Objective: Acute kidney injury occurs early in PICU admission and increases risks for poor outcomes. We evaluated the feasibility of a multicenter acute kidney injury biomarker urine collection protocol and measured diagnostic characteristics of urine neutrophil gelatinase-associated lipocalin, interleukin-18, and liver fatty acid binding protein to predict acute kidney injury and prolonged acute kidney injury.

Design: Prospective observational pilot cohort study.

Acute right ventricular resynchronization improves haemodynamics in children after surgical repair of tetralogy of Fallot.

Aims: Right bundle branch block is associated with right ventricular (RV) electromechanical dyssynchrony, which may contribute to acute haemodynamic impairment after repair of tetralogy of Fallot (ToF). We sought to evaluate the effects of RV resynchronization on haemodynamics and tissue oxygenation during the first 24 h after surgery.

Methods And Results: Arterial pressures, cardiac output, and tissue oxygenation were measured in 28 consecutive patients (median age 10.

Stress Echocardiographic Evaluation for D-Transposition of the Great Arteries after Atrial Redirection: Unmasking Early Signs of Myocardial Dysfunction and Baffle Stenosis.

Background: The authors used semisupine cycle ergometry stress echocardiography to assess cardiac function and unmask baffle stenosis in patients with d-transposition of the great arteries after atrial redirection surgery.

Methods: This was a retrospective review of semisupine cycle ergometry stress echocardiography performed in 53 patients (64% male; mean age, 24.0 years; 90% Mustard procedure) and 56 healthy control subjects.

The Current State and Future Potential of Pediatric and Congenital Electrophysiology.

Pediatric electrophysiologists specialize in the diagnosis and treatment of rhythm abnormalities in pediatric, congenital heart disease, and inherited arrhythmia syndrome patients. The field originated out of the unique knowledge base that rhythm management in young patients required. In the 1970s, pediatric electrophysiology was recognized as a distinct cardiac subspecialty and it has evolved rapidly since that time.

The clinical and genetic spectrum of catecholaminergic polymorphic ventricular tachycardia: findings from an international multicentre registry.

Aims: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an ion channelopathy characterized by ventricular arrhythmia during exertion or stress. Mutations in RYR2-coded Ryanodine Receptor-2 (RyR2) and CASQ2-coded Calsequestrin-2 (CASQ2) genes underlie CPVT1 and CPVT2, respectively. However, prognostic markers are scarce.

Exercise and the multidisciplinary holistic approach to adolescent dysautonomia.

Aim: To determine whether an eight-week strength training programme as part of a multidisciplinary approach would minimise symptoms and improve quality of life in patients with dysautonomia.

Methods: Adolescents referred to a tertiary-level cardiology service from May 2014-December 2015 with symptoms of dysautonomia were eligible. Participants completed an exercise test and a quality of life (QoL) questionnaire (PedsQL) prior to the intervention.

Morbidities in the ultra-athlete and marathoner.

The cardiovascular benefits of habitual exercise are well documented. In the current era, more of the population is exceeding the recommendations for physical activity as the popularity of endurance events increases. Recent data have proposed a U-shaped relationship between exercise intensity and cardiovascular outcomes.

Advances in the diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia.

Since the sentinel description of exercise-triggered ventricular arrhythmias in 21 children, our recognition and understanding of catecholaminergic polymorphic ventricular tachycardia has improved substantially. A variety of treatments are now available, but reaching a diagnosis before cardiac arrest remains a challenge. Most cases are related to variants in the gene encoding for ryanodine receptor-2 (RyR2), which mediates calcium-induced calcium release.