Incidence and outcome of arrhythmias and electrical disease in patients with Trisomy 18.

Patients with Trisomy 18 have a high incidence of cardiac anomalies and are associated with early death. Because of early mortality, electrical system disease and arrhythmia has been difficult to delineate and the incidence remain unknown. We sought to describe the association and clinical outcomes of electrical system disease and cardiac tachy-arrhythmias in patients with Trisomy 18.

Regionalization or Access to Care? A Joint Pediatric Heart Care Program That Achieves Both: One Program-Two Sites.

Regionalization of care for children with congenital heart disease has been proposed as a method to improve outcomes. This has raised concerns about limiting access to care. We present the details of a joint pediatric heart care program (JPHCP) which utilized regionalization and actually improved access to care.

Efficacy, durability, and safety of intravitreal faricimab with extended dosing up to every 16 weeks in patients with diabetic macular oedema (YOSEMITE and RHINE): two randomised, double-masked, phase 3 trials.

Background: To reduce treatment burden and optimise patient outcomes in diabetic macular oedema, we present 1-year results from two phase 3 trials of faricimab, a novel angiopoietin-2 and vascular endothelial growth factor-A bispecific antibody.

Methods: YOSEMITE and RHINE were randomised, double-masked, non-inferiority trials across 353 sites worldwide. Adults with vision loss due to centre-involving diabetic macular oedema were randomly assigned (1:1:1) to intravitreal faricimab 6·0 mg every 8 weeks, faricimab 6·0 mg per personalised treatment interval (PTI), or aflibercept 2·0 mg every 8 weeks up to week 100.

Outcomes of Pediatric Patients With Defibrillators Following Initial Presentation With Sudden Cardiac Arrest.

Background: Implantable cardioverter defibrillators (ICD) are recommended for secondary prevention after sudden cardiac arrest (SCA). The outcomes of pediatric patients receiving an ICD after SCA remain unclear. The objective of this study is to evaluate outcomes, future risk for appropriate shocks, and identify characteristics associated with appropriate ICD therapy during follow-up.

A Phase 1b Study of Vismodegib with Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis.

Introduction: Components of the hedgehog signaling pathway are upregulated in patients with idiopathic pulmonary fibrosis (IPF). Vismodegib, a small-molecule inhibitor of hedgehog signaling, when used in combination with currently available antifibrotic therapy, may be more efficacious than antifibrotics alone. The objective of this study was to evaluate the safety and tolerability of vismodegib plus pirfenidone in patients with IPF.

Management of asymptomatic ventricular preexcitation.

An incidental discovery of Wolff Parkinson White (WPW) pattern on the electrocardiogram (ECG) is not an infrequent finding facing the physician. Most patients discovered incidentally are asymptomatic and it is hard to justify further management of such patients given the time-honored adage to "first do no harm." However, this finding does have implications.

Analysis of adults with congenital heart disease presenting to pediatric emergency departments with arrhythmias.

Objective: As survivors of congenital heart disease (CHD) continue to age, healthcare utilization by this population has increased. It is unknown how often these patients utilize the emergency department (ED) at children's hospitals and how arrhythmias play a role in their utilization of care.

Design: Using a retrospective cohort design, the Pediatric Hospital Information System (PHIS) database was investigated and we studied adults (≥18 years) with CHD (ACHD) who presented to pediatric EDs from 2004 to 2014.

Implementation of a Clinical Pathway for Chest Pain in a Pediatric Emergency Department.

Objective: To evaluate the impact of a pediatric emergency department (ED) chest pain clinical pathway on resource utilization.

Methods: Motivated by perceived overuse of cardiology consultation for non-cardiac chest pain in the ED, clinicians from the Divisions of Cardiology and Emergency Medicine collaboratively developed a chest pain clinical pathway, educated staff, and implemented the pathway on March 1, 2014. We reviewed records of children aged 3 to 18 years without prior diagnoses of heart disease who presented to the ED with chest pain between March 1, 2013, and April 22, 2015.

Pulmonary atresia with intact ventricular septum and coronary cameral fistulae: an unusual finding of a subsystemic right ventricle.

We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.

Embedding patient simulation in a pediatric cardiology rotation: a unique opportunity for improving resident education.

Objective: High-fidelity patient simulation (HFPS) has been used in medical education to bridge gaps in medical knowledge and clinical skills. Few studies have analyzed the impact of HFPS in subspecialty rotations for pediatric residents. We hypothesized that pediatric residents exposed to HFPS with a structured content curriculum would perform better on a case quiz than residents without exposure to HFPS.

Predictors of myocardial recovery in pediatric tachycardia-induced cardiomyopathy.

Background: Tachycardia-induced cardiomyopathy (TIC) carries significant risk of morbidity and mortality, although full recovery is possible. Little is known about the myocardial recovery pattern.

Objective: The purpose of this study was to determine the time course and predictors of myocardial recovery in pediatric TIC.

Late-onset en coup de sabre of the skull.

En coup de sabre is a rare subtype of linear scleroderma that characteristically affects the skin, underlying muscle, and bone of the frontoparietal region of the face and scalp. It typically presents in the first two decades of life, and may be associated with focal neurological deficits. We present a case of late-onset en coup de sabre of the frontal bone where the diagnosis was further complicated by a history of breast cancer, prior trauma to the region, and use of topical medication.

Coronary artery involvement in pediatric Takayasu's arteritis: Case report and literature review.

Takayasu's arteritis (TA) is a form of chronic vasculitis that typically occurs in young adult Asian females, but it can also present in younger patients not fitting this classic profile. In these cases, the sequelae are generally similar to those found in adults. The disease predominantly affects the aorta and its primary branches.

Physician behaviour for antimicrobial prescribing for paediatric upper respiratory tract infections: a survey in general practice in Trinidad, West Indies.

Background: Upper respiratory tract infections (URTIs) are among the most frequent reasons for physician office visits in paediatrics. Despite their predominant viral aetiology, URTIs continue to be treated with antimicrobials. We explored general practitioners' (GPs) prescribing behaviour for antimicrobials in children (< or = 16 years) with URTIs in Trinidad, using the guidelines from the Centers for Disease Control and Prevention (CDC) as a reference.