Rationale and design of the Canadian Outcomes Registry Late After Tetralogy of Fallot Repair: the CORRELATE study.

Background: Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined.

Low-weight infants are at increased mortality risk after palliative or corrective cardiac surgery.

Background: Low weight is an established risk factor for mortality after congenital cardiac surgery. Given the advances in the care of neonates and infants after surgery, we sought to examine the effect of low weight on outcomes in the current era.

Methods: From 2002 to 2012, 2051 infants aged 90 days or less underwent cardiac surgery including 534 (26.

Adolescents with severe obesity: outcomes of participation in an intensive obesity management programme.

Background: Most interventions for childhood obesity are randomized controlled studies. Less is known about the effectiveness of clinical obesity programmes.

Objective: To assess outcomes in adolescents participating in the SickKids Team Obesity Management Program (STOMP) vs.

Results of palliation with an initial pulmonary artery band in patients with single ventricle associated with unrestricted pulmonary blood flow.

Objectives: Pulmonary artery banding is the initial palliative surgery in patients with single ventricle cardiac anomalies presenting with unrestricted pulmonary blood flow. Reported mortality in those receiving pulmonary artery banding is high, and its application in patients with single ventricle anomalies and arch obstruction is controversial. We report current-era results after pulmonary artery banding in patients with single ventricle anomalies, including those with arch obstruction.

Prognostic implications of the systolic to diastolic duration ratio in children with idiopathic or familial dilated cardiomyopathy.

Background: Childhood dilated cardiomyopathy (DCM) carries high morbidity and mortality. The echocardiographic systolic to diastolic (S:D) duration ratio, an indicator of global cardiac performance, is elevated in DCM; however, its prognostic implications have not been investigated in this population.

Methods And Results: We investigated systolic and diastolic durations and the resultant S:D ratio using pulsed tissue Doppler imaging in children with idiopathic or familial DCM.

Left ventricular myocardial response to exercise in children after heart transplant.

Background: Data on myocardial response to exercise after pediatric heart transplant (HTx) are limited. In this study we used semi-supine bicycle ergometry (SSCE) stress echocardiography to evaluate left ventricular (LV) systolic and diastolic reserve in pediatric HTx recipients.

Methods: Forty-three HTx patients and 23 controls underwent stepwise SSCE stress echocardiography.

Remote ischemic preconditioning in children undergoing cardiac surgery with cardiopulmonary bypass: a single-center double-blinded randomized trial.

Background: Remote ischemic preconditioning (RIPC) harnesses an innate defensive mechanism that protects against inflammatory activation and ischemia-reperfusion injury, known sequelae of cardiac surgery with cardiopulmonary bypass. We sought to determine the impact of RIPC on clinical outcomes and physiological markers related to ischemia-reperfusion injury and inflammatory activation after cardiac surgery in children.

Methods And Results: Overall, 299 children (aged neonate to 17 years) were randomized to receive an RIPC stimulus (inflation of a blood pressure cuff on the left thigh to 15 mm Hg above systolic for four 5-minute intervals) versus a blinded sham stimulus during induction with a standardized anesthesia protocol.

Cohort Profile: The Applied Research Group for Kids (TARGet Kids!).

The Applied Research Group for Kids (TARGet Kids!) is an ongoing open longitudinal cohort study enrolling healthy children (from birth to 5 years of age) and following them into adolescence. The aim of the TARGet Kids! cohort is to link early life exposures to health problems including obesity, micronutrient deficiencies and developmental problems. The overarching goal is to improve the health of Canadians by optimizing growth and developmental trajectories through preventive interventions in early childhood.

Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort.

Objective: Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow-up data in a previously well-characterized cohort.

Design: Baseline data from the Fontan Cross-Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.

Factors associated with in utero demise of fetuses that have underlying cardiac pathologies.

Factors associated with in utero fetal demise (IUFD) of fetuses that have underlying cardiac pathologies are largely unknown. This case-control study aimed to define the prevalence of IUFD in fetuses with a diagnosis of cardiac pathologies and to identify prenatal predictors of IUFD. Between January 2004 and December 2010, 74 IUFD cases [4.

Healthcare transition for youth with heart disease: a clinical trial.

Objectives: Adolescents with heart disease have complex health needs and require lifelong cardiology follow-up. Interventions to facilitate paediatric to adult healthcare transition are recommended, although outcomes are unknown. We sought to determine the impact of a transition intervention on improving knowledge and self-management skills among this population.

The impact of not having a ductus arteriosus on clinical outcomes in foetuses diagnosed with tetralogy of Fallot.

Background: Foetuses with simple tetralogy of Fallot almost universally have a patent ductus arteriosus. Two recently identified cases had an absent patent ductus arteriosus, requiring emergent intervention at birth. The objective of this study was to determine whether foetuses diagnosed with tetralogy of Fallot and no patent ductus arteriosus have poorer outcomes compared with those with tetralogy of Fallot+patent ductus arteriosus.

Perioperative factors associated with in-hospital mortality or retransplantation in pediatric heart transplant recipients.

Objective: Despite improved long-term survival after pediatric heart transplantation, perioperative mortality has remained high. We sought to understand the factors associated with perioperative graft loss after pediatric heart transplantation.

Methods: The factors associated with primary heart transplant mortality and retransplantation before hospital discharge in 226 pediatric heart transplant recipients (1995-2010) at a single-center institution were analyzed using multivariable logistic regression models adjusted for age at surgery and year of surgery.

Linking the congenital heart surgery databases of the Society of Thoracic Surgeons and the Congenital Heart Surgeons' Society: part 2--lessons learned and implications.

Purpose: A link has been created between the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the Congenital Heart Surgeons' Society Database (CHSS-D). Five matrices have been created that facilitate the automated identification of patients who are potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices are now used to (1) estimate the denominator of patients eligible for CHSS studies and (2) compare "eligible and enrolled patients" to "potentially eligible and not enrolled patients" to assess the generalizability of CHSS studies.

Linking the congenital heart surgery databases of the Society of Thoracic Surgeons and the Congenital Heart Surgeons' Society: part 1--rationale and methodology.

Purpose: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) is the largest Registry in the world of patients who have undergone congenital and pediatric cardiac surgical operations. The Congenital Heart Surgeons' Society Database (CHSS-D) is an Academic Database designed for specialized detailed analyses of specific congenital cardiac malformations and related treatment strategies. The goal of this project was to create a link between the STS-CHSD and the CHSS-D in order to facilitate studies not possible using either individual database alone and to help identify patients who are potentially eligible for enrollment in CHSS studies.

Effectiveness of omega-3 polysaturated fatty acids (fish oil) supplementation for treating hypertriglyceridemia in children and adolescents.

Limited pharmacological options are available for management pediatric hypertriglyceridemia. We examined the effectiveness of dietary fish oil supplementation as a means to reduce triglyceride levels in pediatric patients. We reviewed 111 children aged 8 to 18 years with hypertriglyceridemia (≥1.

Education for lifestyle-based management of hyperlipidemia in children enhanced by a collaborative approach.

Background: Although therapeutic lifestyle changes are first-line measures in treating pediatric dyslipidemia, current didactic approaches for healthy lifestyle education are weakened by low adherence and poor sustainability. A collaborative education program including a clinician-led group education class with motivational counseling complemented by the addition of peer role models was implemented.

Objective: We sought to assess the effectiveness of motivational interviewing in collaboration with peers sharing their experience and its impact on serologic and lifestyle measures vs the conventional, didactic group education approach.

Cardiac energy metabolism is positively associated with skeletal muscle energy metabolism in physically active adolescents and young adults.

(31)Phosphorus Magnetic Resonance Spectroscopy ((31)P MRS) is a well-validated, noninvasive magnetic resonance imaging technique that has been used to determine cardiac and skeletal muscle energy metabolism in vivo. Few studies have documented cardiac energy metabolism in adolescents and young adult cohorts. This cross-sectional study sought to explore the association among cardiac energy metabolism, skeletal muscle energy metabolism, moderate-to-vigorous physical activity (MVPA), and age in adolescents and young adults.

Mortality and morbidity after retransplantation after primary heart transplant in childhood: an analysis from the registry of the International Society for Heart and Lung Transplantation.

Background: Retransplantation for graft failure in the pediatric population is rare with reports of decreased survival compared with primary transplantation. Little is known about the risk factors for retransplantation or death after retransplantation; in addition, there is a paucity of data in the literature on transplant-related morbidities in this patient population. The purpose of this analysis of the registry of the International Society of Heart and Lung Transplant (ISHLT) was to describe outcomes after retransplantation as compared with primary transplantation, including identifying risk factors leading to retransplantation and both transplant-related morbidities and mortality after retransplantation.

Does single ventricle physiology affect survival of children requiring extracorporeal membrane oxygenation support following cardiac surgery?

Background: Improved survival with postoperative extracorporeal membrane oxygenation (ECMO) has expanded its application to children with single ventricle (SV) anomalies. We examine current-era outcomes of postoperative ECMO with special focus on patients with SV.

Methods: Demographic, anatomic, surgical, and support details of 100 consecutive children requiring postoperative ECMO (2007-2012) were included into multivariable regression models to identify factors affecting survival.

Ectopic fat in youth: the contribution of hepatic and pancreatic fat to metabolic disturbances.

Objective: To study the relationships between parameters of glucose and insulin metabolism and visceral and abdominal ectopic fat in youth.

Methods: A cross sectional study of 50 children (24 females), 8-18 years old. Anthropometrics, body composition, blood-work and visceral and ectopic fat by magnetic resonance imaging were assessed.

Obesity, sex and pubertal status affect appetite hormone responses to a mixed glucose and whey protein drink in adolescents.

Background And Objectives: Little information is available on how food intake regulatory hormones may be altered during pubertal development and across the weight spectrum in adolescents. Therefore, the effect of obesity, sex and pubertal status on subjective appetite and appetite hormones in response to a mixed glucose and whey protein drink was determined in 8-18 year old adolescents.

Patients And Methods: A cross-sectional cohort study was conducted at the Hospital for Sick Children, Toronto.

The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure.

Despite hypothesized concerns about deterioration beginning in adolescence, longitudinal data and associated factors regarding standardized assessment of physical functioning are not available for Fontan patients. Parents who participated in the Fontan Cross-Sectional Study completed the Child Health Questionnaire at 2 time points for 245 subjects ages 6-18 years. Associations between change in Physical Functioning Summary Score and baseline patient, medical, and laboratory characteristics (mean age 9.