Pulmonary Atresia With Intact Ventricular Septum With Borderline Tricuspid Valve: How Small Is Too Small.

PA/IVS is a rare, heterogenous congenital heart defect anatomically defined by complete obstruction to the right ventricular outflow tract with varying degrees of hypoplasia of the right ventricle and tricuspid valve. This lesion can have associated coronary artery anomalies and, in some cases, right ventricular-dependent coronary circulation. Due to the wide spectrum of presenting anatomic and clinical features, the treatment options are often dictated by the degree of development of the tricuspid valve and right ventricle.

The Bayley-III scale may underestimate neurodevelopmental disability after cardiac surgery in infants.

Objectives: Neurodevelopmental disability is the most common complication among congenital heart surgery survivors. The Bayley scales are standardized instruments to assess neurodevelopment. The most recent edition (Bayley Scales of Infant and Toddler Development 3rd Edition, Bayley-III) yields better-than-expected scores in typically developing and high-risk infants than the second edition (Bayley Scales of Infant Development 2nd Edition, BSID-II).

Relationship Between Time to Left Atrial Decompression and Outcomes in Patients Receiving Venoarterial Extracorporeal Membrane Oxygenation Support: A Multicenter Pediatric Interventional Cardiology Early-Career Society Study.

Objectives: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort.

Design: Multicenter retrospective study.

Setting: Eleven pediatric hospitals within the United States.

Characteristics and operative outcomes for children undergoing repair of truncus arteriosus: A contemporary multicenter analysis.

Objective: We sought to describe characteristics and operative outcomes of children who underwent repair of truncus arteriosus and identify risk factors for the occurrence of major adverse cardiac events (MACE) in the immediate postoperative period in a contemporary multicenter cohort.

Methods: We conducted a retrospective review of children who underwent repair of truncus arteriosus between 2009 and 2016 at 15 centers within the United States. Patients with associated interrupted or obstructed aortic arch were excluded.

Adolescent Substance Abuse Risk: A Comparison of Pediatric Emergency Care Applied Research Network Emergency Department Patients and 2 Nationally Representative Samples.

Objectives: The aim of this study was to understand the prevalence of alcohol and other substance use among teenagers in generalized samples.

Methods: This study compared the alcohol and other substance use of adolescents enrolled in a screening study across 16 Pediatric Emergency Care Applied Research Network emergency departments (EDs) (ASSESS) with those sampled in 2 nationally representative surveys, the Youth Risk Behavior Surveillance System (YRBSS) and the National Survey of Drug Use and Health (NSDUH). The analysis includes 3362 ASSESS participants and 11,142 YRBSS and 12,086 NSDUH respondents.

Use of serum citrulline concentrations from routine newborn screen as a biomarker for necrotizing enterocolitis.

Purpose: Necrotizing enterocolitis (NEC), a leading cause of mortality and morbidity in preterm neonates, lacks a reliable biomarker. Citrulline is primarily produced by enterocytes and correlates with intestinal function. Serum citrulline concentration (CIT) is routinely measured in routine newborn screening (NBS).

Gross Motor Function Classification System Specific Growth Charts-Utility as a Risk Stratification Tool for Surgical Site Infection Following Spine Surgery.

Background: There is currently minimal evidence that preoperative malnutrition increases surgical site infection (SSI) risk in children with cerebral palsy (CP) undergoing spinal deformity surgery. Growth charts specifically for patients with CP have been created to aid in the clinical interpretation of body mass index (BMI) as a marker of nutritional status, but to our knowledge these charts have never been used to risk stratify patients before orthopaedic surgery. We hypothesize that patients with CP who have BMI-for-age below the 10th percentile (BMI≤10) on CP-specific growth charts are at increased risk of surgical site infection following spinal deformity surgery compared with patients with BMI-for-age above the 10th percentile (BMI>10).

QT Interval Prolongation in the Pediatric Oncologic Population on Methadone.

Studies have been conducted on adults prescribed with methadone to determine the necessary frequency of QTc monitoring but no consensus has been reached and no similar research has been conducted in the pediatric population. The objective of this retrospective study was to determine the occurrence rate of QTc interval prolongation associated with methadone use in a pediatric oncologic population. In total, 18% of patients developed QTc interval prolongation.

Non-operative management of solid organ injuries in children: An American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee systematic review.

Purpose: The American Pediatric Surgical Association (APSA) guidelines for the treatment of isolated solid organ injury (SOI) in children were published in 2000 and have been widely adopted. The aim of this systematic review by the APSA Outcomes and Evidence Based Practice Committee was to evaluate the published evidence regarding treatment of solid organ injuries in children.

Methods: A comprehensive search strategy was crafted and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were utilized to identify, review, and report salient articles.

Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations.

Background Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences during early childhood after staged surgical palliations using echocardiography. Methods We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt.

The Impact of Transphyseal Anterior Cruciate Ligament Reconstruction on Lower Extremity Growth and Alignment.

Purpose: To evaluate the effect of transphyseal anterior cruciate ligament (ACL) reconstruction on lower extremity radiographic growth and alignment.

Methods: We retrospectively reviewed patients who underwent transphyseal ACL reconstruction and were followed to skeletal maturity or at least 2 years, with the nonoperative limb used as an internal control. Changes in coronal plane alignments and tibial slope of the operative limb were compared with a Wilcoxon test.

Multicenter Analysis of Early Childhood Outcomes After Repair of Truncus Arteriosus.

Background: Literature describing morbidity and mortality after truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population.

Methods: We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016.

Mid-Term Valve-Related Outcomes After Transcatheter Tricuspid Valve-in-Valve or Valve-in-Ring Replacement.

Background: Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis.

Objectives: The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes.

Cerebral Vasospasm after Open Fenestration of an Arachnoid Cyst in a 4-Year-Old Boy: Case Report and Review of the Literature.

Cerebral vasospasm is associated with significant morbidity, and most commonly occurs following subarachnoid hemorrhage. Rarely, vasospasm can follow tumor resection and traumatic brain injury. We present the first reported case of a young child who developed diffuse vasospasm following open fenestration of an arachnoid cyst and was promptly treated, with full recovery of neurologic function.

Use of Capnography and Cardiopulmonary Resuscitation Feedback Devices Among Prehospital Advanced Life Support Providers.

Background: Capnography and cardiopulmonary resuscitation (CPR) feedback devices have been shown to improve resuscitation outcomes, with the American Heart Association recommending their use during advanced life support (ALS). Little is known about the availability of these devices, their protocoled use, and the attitudes toward them in the prehospital setting.

Objectives: The objectives of this study were to assess the availability of capnography and CPR feedback devices among prehospital ALS agencies in New York State (NYS), to describe the protocoled use of these devices, and to evaluate the attitudes of providers toward the use of these devices.

Pressure ulcers in paediatric patients on extracorporeal membrane oxygenation.

It has been shown that pressure ulcer formation in critically ill paediatric patients increases morbidity and mortality. We sought to identify factors associated with pressure ulcer formation in paediatric patients on extracorporeal membrane oxygenation (ECMO). From December 2014 to 2015, we identified patients at our institution who developed a pressure ulcer to create two cohorts: ulcer and no ulcer.

Pharmacological Management of Severe Neuropathic Pain in a Case of Eosinophilic Meningitis Related to Angiostrongylus cantonensis.

Angiostrongylus cantonensis, the rat lungworm, is the most common infectious cause of eosinophilic meningitis and can be fatal. The parasite can be found throughout Southeast Asia and Pacific Islands and the global distribution is expanding. We present the case of a fourteen-year-old female who had previously traveled to Hawaii and developed severe neuropathic pain related to A.

Influenza Myocarditis Treated With Antithymocyte Globulin.

Influenza is a cause of significant morbidity and mortality worldwide. Myocarditis is a rare complication of the virus and can vary widely in severity. The published cases of influenza B myocarditis in children tend to be severe with a high mortality rate.

Hemodynamic assessment of atrial septal defects.

Atrial septal defect (ASD) is one of the most common congenital cardiac anomalies. ASD can present as an isolated lesion in an otherwise normal heart or in association with other congenital heart conditions. Regardless of the type of ASD, the direction and degree of shunting across the communication is mainly determined by the difference in compliance between the right and left ventricle.

New primary immunodeficiency diseases: context and future.

Purpose Of Review: Primary immunodeficiency diseases (PIDs) are genetic disorders classically characterized by impaired host defense and an increased susceptibility to infections. It is now appreciated that these conditions broadly include variations in the genetic code that cause dysregulated immune function. This review highlights the newly defined PIDs in the 2017 International Union of Immunologic Societies (IUIS) report, current approaches to diagnosing PIDs, and the implications for the future management of PIDs.

Refining the phenotype associated with GNB1 mutations: Clinical data on 18 newly identified patients and review of the literature.

De novo germline mutations in GNB1 have been associated with a neurodevelopmental phenotype. To date, 28 patients with variants classified as pathogenic have been reported. We add 18 patients with de novo mutations to this cohort, including a patient with mosaicism for a GNB1 mutation who presented with a milder phenotype.

"Splint" Mitral Valve Repair for Destructive Endocarditis in Children.

Medical management of infective endocarditis in the pediatric population has an associated in-hospital mortality rate of up to 25%. In the past, infective endocarditis of the mitral valve was surgically managed with a valve replacement. Now, there is a shift toward repair.

Hydronephrosis After Pyeloplasty: "Will It Go Away?".

Objective: To identify attributes of pediatric patients with hydronephrosis due to ureteropelvic junction obstruction and of their surgical encounters which are predictive of resolution of dilatation in order to provide more effective counseling about expected outcomes. This study was inspired by the suggestion in recent literature that greater than 20% improvement in anteroposterior diameter (APD) of the renal pelvis after pyeloplasty is indicative of resolution of obstruction. The remaining dilatation, however, is often distressing to caregivers, and there are no data to guide clinicians in counseling about its likelihood to resolve.