A text messaging intervention and quality of life in adolescents with solid organ transplants.

Background: Facilitating communication between adolescents and HCP outside of appointments may enhance patient experience and outcomes. The purpose of this study was to determine whether SMS enhances the healthcare experience, QoL, and medication adherence in adolescent SOT patients.

Methods: This was a prospective observational study of an SMS platform (WelTel Inc) for SOT patients aged 12-19 years.

Arrhythmic Mitral Valve Prolapse and Mitral Annulus Disjunction in Heritable Aortic Disease.

Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD.

Increasing age and atrial arrhythmias are associated with increased thromboembolic events in a young cohort of adults with repaired tetralogy of Fallot.

Background: Adults with repaired Tetralogy of Fallot (rTOF) comprise one of the largest cohorts among adults with congenital heart disease (ACHD). These patients have a higher burden of atrial arrhythmias (AA), leading to increased adverse events, including stroke and transient ischemic attack (TIA). However, the data on factors associated with stroke/TIA in rTOF are limited, and classic risk factors may not apply.

Atrial arrhythmias and thromboembolic complications in adults post Fontan surgery.

Objective: Patients with Fontan surgery experience late complications in adulthood. We studied the factors associated with the development and maintenance of atrial arrhythmias and thromboembolic complications in an adult population with univentricuar physiology post Fontan surgery.

Methods: Single centre retrospective cohort study of patients ≥18 years of age with Fontan circulation followed at our quaternary care centre for more than 1 year were included.

Clinical Importance of Fontan Circuit Thrombus in the Adult Population: Significant Association With Increased Risk of Cardiovascular Events.

Background: The impact of Fontan circuit thrombus is poorly understood. The objectives of this study were to determine (1) the incidence of Fontan circuit thrombus and proportion of silent thrombus; (2) any association between Fontan circuit thrombus and markers of Fontan circulatory dysfunction; and (3) the association of Fontan circuit thrombus with adverse cardiac outcomes.

Methods: We conducted a retrospective review of adult patients who underwent the Fontan procedure (aged > 18 years) followed at St.

Adults With Complex Congenital Heart Disease: Cerebrovascular Considerations for the Neurologist.

As infant and childhood mortality has decreased in congenital heart disease, this population is increasingly reaching adulthood. Adults with congenital heart disease (ACHD) represent a group with increased risk of stroke, silent brain infarcts, and vascular cognitive impairment. Cyanotic and other complex cardiac lesions confer the greatest risk of these cerebrovascular insults.

Outcomes related to respiratory syncytial virus with an abbreviated palivizumab regimen in children with congenital heart disease: a descriptive analysis.

Background: It has been hypothesized that 4 doses of palivizumab, a neutralizing monoclonal antibody against respiratory syncytial virus (RSV), administered during a fixed-date RSV season may reduce hospital admissions comparably to the standard 5-dose schedule. We report outcomes in children with congenital heart disease approved to receive this 4-dose palivizumab schedule in British Columbia.

Methods: We performed a population-based descriptive cohort analysis of all 406 approved palivizumab courses over 4 seasons (2012/13 to 2015/16) in 325 children with hemodynamically significant congenital heart disease enrolled in the British Columbia RSV Immunoprophylaxis Program.

Physiological Responses to Exercise in Pediatric Heart Transplant Recipients.

Introduction: Pediatric heart transplant (HTx) recipients have reduced exercise capacity typically two-thirds of predicted values, the mechanisms of which are not fully understood. We sought to assess the cardiorespiratory responses to progressive exercise in HTx relative to controls matched for age, sex, body size, and work rate.

Methods: Fourteen HTx recipients and matched controls underwent exercise stress echocardiography on a semisupine cycle ergometer.

Coronary artery intimal thickening and ventricular dynamics in pediatric heart transplant recipients.

Objective: Pediatric heart transplant recipients are at risk of posttransplant coronary artery disease known as cardiac allograft vasculopathy (CAV), and also may develop diastolic dysfunction. As CAV begins with a process of progressive intimal thickening, these occult diffuse changes may be detected using optical coherence tomography (OCT). We hypothesized that the development of CAV, as identified via OCT, may be a mechanism of declining ventricular function.

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.

Aspirin Dose and Prevention of Coronary Abnormalities in Kawasaki Disease.

Background: Acetylsalicylic acid (ASA) is part of the recommended treatment of Kawasaki disease (KD). Controversies remain regarding the optimal dose of ASA to be used. We aimed to evaluate the noninferiority of ASA at an antiplatelet dose in acute KD in preventing coronary artery (CA) abnormalities.

Canadian Cardiovascular Society/Canadian Pediatric Cardiology Association Position Statement on Pulse Oximetry Screening in Newborns to Enhance Detection of Critical Congenital Heart Disease.

Congenital heart disease is the most common congenital malformation and approximately 3 in 1000 newborns have critical congenital heart disease (CCHD). Timely diagnosis affects morbidity, mortality, and disability, and newborn pulse oximetry screening has been studied to enhance detection of CCHD. In this position statement we present an evaluation of the literature for pulse oximetry screening.

Assessment of Liver Stiffness in Pediatric Fontan Patients Using Transient Elastography.

. Hepatic fibrosis is a potential complication following Fontan surgery and heralds long-term risk for cirrhosis. Transient elastography (TE) is a rapid, noninvasive method to assess liver fibrosis by measuring liver stiffness.

Fatal congenital hypertrophic cardiomyopathy and a pancreatic nodule morphologically identical to focal lesion of congenital hyperinsulinism in an infant with costello syndrome: case report and review of the literature.

Costello syndrome is characterized by constitutional mutations in the proto-oncogene HRAS, causing dysmorphic features, multiple cardiac problems, intellectual disability, and an increased risk of neoplasia. We report a male infant with dysmorphic features, born prematurely at 32 weeks, who, during his 3-month life span, had an unusually severe and ultimately fatal manifestation of hypertrophic cardiomyopathy and hyperinsulinemic hypoglycemia. Molecular studies in this patient demonstrated the uncommon Q22K mutation in the HRAS gene, diagnostic of Costello syndrome.

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.

Infant repair of massive aortic aneurysm with prosthetic valved conduit.

A 4-month-old child with severe infantile Marfan syndrome underwent successful repair of an extremely dilated aortic root and severe aortic valve insufficiency using a prosthetic valved conduit.

Epidemiology of cardiac surgery-associated acute kidney injury in neonates: a retrospective study.

Background: Cardiac surgery is a known risk factor for acute kidney injury (AKI) in children. However, cardiac surgery-associated AKI (CS-AKI) in neonates has not been well studied. The objectives of this study were: (1) to describe the epidemiology of CS-AKI in neonates utilizing the Acute Kidney Injury Network (AKIN) definition, (2) to identify risk factors for neonatal CS-AKI, and (3) to determine if neonatal CS-AKI is associated with increased morbidity and mortality.

Management of adults with congenital heart disease using videoconferencing across Western Canada: a 3-year experience.

Background: Management of adults with congenital heart disease (ACHD) requires quaternary centres with cardiologists and cardiac surgeons who have expertise in the diagnosis and management of this patient population. We report on the feasibility of the management of ACHD patients using videoconferencing and streaming through 1 regional referral centre covering 4 western Canadian provinces, roughly 30% of Canada's land and population.

Methods: Videoconferencing sessions from January 2008 to December 2010 were systematically reviewed.

Evaluation of silent thrombus after the Fontan operation.

Background: Thromboembolic complications have been noted after the Fontan operation. However, the prevalence of silent events among an adult contemporary population is not known. Noninvasive screening by any method including computed tomography (CT) has been technically limited to date.

The Contegra conduit: Late outcomes in right ventricular outflow tract reconstruction.

Objectives: To report the clinical outcomes (early death, late death, and rate of reintervention) and performance of the Contegra conduit as a right ventricle outflow tract implant and to determine the risk factors for early reintervention.

Methods: Forty-nine Contegra conduits were implanted between January 2002 and June 2009. Data collection was retrospective.

Kingella kingae endocarditis: A rare case of mitral valve perforation.

Kingella kingae, a HACEK (Haemophilus parainfluenzae, Aggregatibacter actinomycetemcomitans, Aggregatibacter aphrophilus, Cardiobacterium hominis, Eikenella corrodens, Kingella kingae) organism, is a common resident of the upper airway in children; it has been associated with endocarditis in children with pre-existing heart conditions. This case report describes K. kingae endocarditis leading to valvular damage in a previously healthy 18-month-old child.

Left ventricle pseudoaneurysm after aortic valvuloplasty.

Acquired left ventricular aneurysm is extremely rare in children. This report describes an infant with acquired left ventricular aneurysm after percutaneous aortic balloon valvuloplasty for critical aortic stenosis. The potential risk factors for myocardial injury during cardiac catheterization and potential complications are discussed.