Establishing consensus: determinants of high-risk and preventative strategies for neurological events in complex spinal deformity surgery.

Purpose: To establish expert consensus on various parameters that constitute elevated risk during spinal deformity surgery and potential preventative strategies that may minimize the risk of intraoperative neuromonitoring (IONM) events and postoperative neurological deficits.

Methods: Through a series of surveys and a final virtual consensus meeting, the Delphi method was utilized to establish consensus among a group of expert spinal deformity surgeons. During iterative rounds of voting, participants were asked to express their agreement (strongly agree, agree, disagree, strongly disagree) to include items in a final set of guidelines.

Inferring pediatric knee skeletal maturity from MRI using deep learning.

Purpose: Many children who undergo MR of the knee to evaluate traumatic injury may not undergo a separate dedicated evaluation of their skeletal maturity, and we wished to investigate how accurately skeletal maturity could be automatically inferred from knee MRI using deep learning to offer this additional information to clinicians.

Materials And Methods: Retrospective data from 894 studies from 783 patients were obtained (mean age 13.1 years, 47% female).

Relation of Norwood Shunt Type and Frequency of Arrhythmias at 6 Years (from the Single Ventricle Reconstruction Trial).

The Norwood procedure with a right ventricular to pulmonary artery shunt (RVPAS) decreases early mortality, but requires a ventriculotomy, possibly increasing risk of ventricular arrhythmias (VAs) compared with the modified Blalock-Taussig shunt (MBTS). The effect of shunt and Fontan type on arrhythmias by 6 years of age in the SVRII (Single Ventricle Reconstruction Extension Study) was assessed. SVRII data collected on 324 patients pre-/post-Fontan and annually at 2 to 6 years included antiarrhythmic medications, electrocardiography (ECG) at Fontan, and Holter/ECG at 6 years.

Right Ventricular Strain Is Associated With Increased Length of Stay After Tetralogy of Fallot Repair.

Background: Little is known regarding right ventricular (RV) remodeling immediately after Tetralogy of Fallot (TOF) repair. We sought to describe myocardial deformation by cardiac magnetic resonance imaging (CMR) after TOF repair and investigate associations between these parameters and early post-operative outcomes.

Methods: Fifteen infants underwent CMR without sedation as part of a prospective pilot study after undergoing complete TOF repair, prior to hospital discharge.

Diversity of Dystrophin Gene Mutations and Disease Progression in a Contemporary Cohort of Duchenne Muscular Dystrophy.

Abnormal dystrophin production due to mutations in the dystrophin gene causes Duchenne Muscular Dystrophy (DMD). Cases demonstrate considerable genetic and disease progression variability. It is unclear if specific gene mutations are prognostic of outcomes in this population.

Molecular characterization and investigation of the role of genetic variation in phenotypic variability and response to treatment in a large pediatric Marfan syndrome cohort.

Purpose: In a large cohort of 373 pediatric patients with Marfan syndrome (MFS) with a severe cardiovascular phenotype, we explored the proportion of patients with MFS with a pathogenic FBN1 variant and analyzed whether the type/location of FBN1 variants was associated with specific clinical characteristics and response to treatment. Patients were recruited on the basis of the following criteria: aortic root z-score > 3, age 6 months to 25 years, no prior or planned surgery, and aortic root diameter < 5 cm.

Methods: Targeted resequencing and deletion/duplication testing of FBN1 and related genes were performed.

The role of stereo-electroencephalography to localize the epileptogenic zone in children with nonlesional brain magnetic resonance imaging.

Objective: This study aimed to assess the clinical outcome and outcome predictive factors in pediatric epilepsy patients evaluated with stereo-electroencephalography (SEEG).

Methods: Thirty-eight patients who underwent SEEG implantation at the Pediatric Epilepsy Center in New York Presbyterian Hospital between June 2014 and December 2019 were enrolled for retrospective chart review. Postoperative seizure outcomes were evaluated in patients with at least 12-months follow up.

High-impact rare genetic variants in severe schizophrenia.

Extreme phenotype sequencing has led to the identification of high-impact rare genetic variants for many complex disorders but has not been applied to studies of severe schizophrenia. We sequenced 112 individuals with severe, extremely treatment-resistant schizophrenia, 218 individuals with typical schizophrenia, and 4,929 controls. We compared the burden of rare, damaging missense and loss-of-function variants between severe, extremely treatment-resistant schizophrenia, typical schizophrenia, and controls across mutation intolerant genes.

Distal adding-on in adolescent idiopathic scoliosis results in diminished health-related quality of life at 10 years following posterior spinal fusion.

Purpose: The purpose of this study was to investigate associations between the various definitions of adding-on identified in the literature and HRQoL at 10 years following posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) to identify a clinically relevant definition.

Methods: In this retrospective cohort study, patients with Lenke type 1 or 2 AIS with 10-year follow-up after PSF were identified in a multicenter registry. Adding-on was identified independently using 5 published definitions and expert spine surgeons' visual confirmation.

Predictors of Prolonged Fluoroscopy Exposure in Pediatric Endoscopic Retrograde Cholangiopancreatography: Results From the Large Pediatric Endoscopic Retrograde Cholangiopancreatography Database Initiative Multicenter Cohort.

Background And Aims: Ionizing radiation exposure during endoscopic retrograde cholangiopancreatography (ERCP) is an important quality issue especially in children. We aim to identify factors associated with extended fluoroscopy time (FT) in children undergoing ERCP.

Methods: ERCP on children <18 years from 15 centers were entered prospectively into a REDCap database from May 2014 until May 2018.

Fifth Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report.

Background: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) provides detailed information on pediatric patients supported with ventricular assist devices (VADs).

Methods: From September 19, 2012, to December 31, 2020, 1229 devices in 1011 patients were reported to the registry from 47 North American Hospitals in patients aged younger than 19 years.

Results: Cardiomyopathy was the most common underlying etiology (58%), followed by congenital heart disease (CHD; 25%) and myocarditis (10%).

Implementing an Evidence-Based Feeding Protocol: Impact on Nurses' Knowledge, Perceptions, and Feeding Culture in the NICU.

Background: Evidence-based feeding practices are often variable among neonatal providers due to lack of knowledge and neonatal intensive care unit (NICU) feeding culture norms.

Purpose: To evaluate changes in NICU nurses' knowledge, perceptions, feeding practices and culture following education about, and implementation of, an evidence-based Infant-Driven Feeding (IDF) protocol.

Methods: A pre-/postprospective comparative design was used to survey 120 registered nurses employed in a level 3 NICU about feeding practices, knowledge, and culture prior to IDF education and 1 to 2 months after IDF implementation.

Pulmonary Vein Stenosis in Children: A Programmatic Approach Employing Primary and Anatomic Therapy.

Pulmonary vein stenosis (PVS) is a difficult condition to treat due to recurrence and progression. In 2017, we developed a comprehensive PVS Program at our center to address the multidisciplinary needs of these patients. We discuss the components of our program and our approach to these patients, using a combination of primary (medical) therapy in addition to anatomic therapy to preserve vessel patency.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

Background: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria.

Objectives: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management.

Anodal stimulation in pediatric patients with permanent epicardial ventricular pacemakers.

Objectives: We aimed to measure the frequency and factors associated with anodal stimulation in a pediatric population with epicardial pacing leads.

Background: In bipolar pacemakers, capture of the myocardium typically occurs at the cathode. However, AS with capture at the anode has been described.

Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected.

Simulation as an Educational Tool in the Pediatric Cardiac Intensive Care Unit.

Purpose Of Review: This review highlights the use of simulation as an educational tool in the highly specialized pediatric cardiac intensive care unit (PCICU).

Recent Findings: Healthcare simulation is used in high acuity medical environments to test healthcare systems. Healthcare simulation can improve team training, patient safety, and improve medical decision-making.

Left Heart Decompression on Veno-Arterial Extracorporeal Membrane Oxygenation in Children With Dilated Cardiomyopathy and Myocarditis: An Extracorporeal Life Support Organization Registry Review.

Objectives: To describe the association between left heart decompression on veno-arterial extracorporeal membrane oxygenation and survival in patients with myocarditis and dilated cardiomyopathy. The secondary outcome is to study association of left heart decompression with survival in children with myocarditis compared with those with dilated cardiomyopathy.

Design: Retrospective study of a multicenter registry database.