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.

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.

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.

Assessment of vocal fold mobility before and after cardiothoracic surgery in children.

Objectives: To assess the incidence of vocal fold immobility (VFI) after cardiothoracic surgery in children and to determine the factors potentially associated with this outcome.

Methods: Flexible laryngoscopy to assess vocal fold mobility was performed before surgery and within 72 hours after extubation in 100 pediatric patients who underwent cardiothoracic procedures. The 2 operating surgeons recorded the surgical technique and their impression of possible injury to the recurrent laryngeal nerve.

Economic evaluation of palivizumab in children with congenital heart disease: a Canadian perspective.

Background: Respiratory syncytial virus (RSV) is a common cause of bronchiolitis in infants. In children with congenital heart disease (CHD), it is associated with significant morbidity and mortality. Palivizumab is a monoclonal antibody that reduces the number of RSV-associated hospitalizations in children with CHD.

The registry and follow-up of complex pediatric therapies program of Western Canada: a mechanism for service, audit, and research after life-saving therapies for young children.

Newly emerging health technologies are being developed to care for children with complex cardiac defects. Neurodevelopmental and childhood school-related outcomes are of great interest to parents of children receiving this care, care providers, and healthcare administrators. Since the 1970s, neonatal follow-up clinics have provided service, audit, and research for preterm infants as care for these at-risk children evolved.

Serial measurements of exercise performance in pediatric heart transplant patients using stress echocardiography.

Heart transplantation is an increasingly acceptable therapeutic option for children with end-stage and complex congenital heart disease. With advances in surgery, immunosuppression, and follow-up care, functional outcomes need to be evaluated. We report the results of serial exercise testing performed using stress echocardiography in a cohort of pediatric HTP.

Living with complex congenital heart disease.

A child with complex congenital heart disease in 2008 is very likely to survive a series of surgical and medical interventions, and confront an array of medical and psychosocial stressors that are presently poorly understood. As approaches to medical problems change, careful assessment of those results is essential, and the initial work of the multicentre Pediatric Heart Network is a huge step in the right direction, setting the stage for proper controlled trials of therapies. Major complications, notably ventricular failure, rhythm problems and thromboembolism, will affect nearly one-quarter of survivors, necessitating further interventions.

A case of horseshoe lung and complex congenital heart disease in a term newborn.

We describe and illustrate a rare association of hypoplastic left heart syndrome, absent hilar left pulmonary artery, and an unusual bronchopulmonary malformation. This case highlights the utility of combination imaging of echocardiography and CT angiography in diagnosing a cyanotic newborn with a chest radiograph that is suspicious for lung hypoplasia.

The protean manifestations of blunt cardiac trauma in children.

We present a case series of pediatric blunt cardiac trauma with a variety of cardiac abnormalities, occurring immediately and after the initial insult. The range of complications and importance of serial evaluations are emphasized.