Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study.

We sought to describe the clinical course and outcomes of patients who are diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) after infancy. We conducted a retrospective evaluation of patients who underwent ALCAPA surgery between January 2009 to March 2018 at 21 US centers. Clinical presentation, inpatient management, and postoperative outcomes of patients repaired ≥1 year of age were described.

A community divided: Post-transplant live vaccine practices among Society of Pediatric Liver Transplantation (SPLIT) centers.

Background: Historically, the IDSA and the AST have recommended that live vaccines not be administered post-transplant due to concern for induction of vaccine-strain disease in immunocompromised hosts. However, recent prospective studies and revised AST guidelines published in April 2019 suggest that in the current era of immunosuppression minimization, live vaccines may be safely administered to select transplant recipients with resulting immunoprotection. The goal of this study was to assess current post-transplant live vaccine practices at individual pediatric liver transplant centers following the updated AST guidelines.

Recruitment, retention, and adherence in a clinical trial: The Pediatric Heart Network's Marfan Trial experience.

Background/aims: The Pediatric Heart Network Marfan Trial was a randomized trial comparing atenolol versus losartan on aortic root dilation in 608 children and young adults with Marfan syndrome. Barriers to enrollment included a limited pool of eligible participants, restrictive entry criteria, and a diverse age range that required pediatric and adult expertise. Retention was complicated by a 3-year commitment to a complex study and medication regimen.

Management of transient central diabetes insipidus with intravenous desmopressin in a premature infant with gastroschisis and septo-optic dysplasia: A case report.

Central diabetes insipidus (CDI) may occur in the setting of intracranial abnormalities that affect the hypothalamus-pituitary system. It occurs rarely in neonates, especially in the premature population, and represents a challenging disease process to treat pharmacologically. Little is known regarding the treatment options in premature infants, including dose and route of administration of intravenous desmopressin (DDAVP).

Asthma among hospitalized patients with COVID-19 and related outcomes.

Background: Several underlying conditions have been associated with severe acute respiratory syndrome coronavirus 2 illness, but it remains unclear whether underlying asthma is associated with worse coronavirus disease 2019 (COVID-19) outcomes.

Objective: Given the high prevalence of asthma in the New York City area, our objective was to determine whether underlying asthma was associated with poor outcomes among hospitalized patients with severe COVID-19 compared with patients without asthma.

Methods: Electronic heath records were reviewed for 1298 sequential patients 65 years or younger without chronic obstructive pulmonary disease who were admitted to our hospital system with a confirmed positive severe acute respiratory syndrome coronavirus 2 test result.

COVID-19 reveals Brugada pattern in an adolescent patient.

A diagnosis of Brugada pattern in paediatric or adolescent patients is rare. COVID-19 is characterised by fevers and a pro-inflammatory state, which may serve as inciting factors for Brugada pattern. Recently described in two adult patients, we report the first case of Brugada pattern in an adolescent with COVID-19.

Variability in stable sagittal vertebra (SSV) during full-length biplanar xrays can affect the choice of fusion levels in patients with adolescent idiopathic scoliosis (AIS).

Purpose: Surgical planning for Adolescent Idiopathic Scoliosis (AIS) relies on the coronal and sagittal plane to determine the lowest instrumented vertebra (LIV). Failure to include the stable sagittal vertebra (SSV) within the construct can increase the incidence of postoperative distal junctional kyphosis (DJK). The purpose of this study is to assess the variability of SSV within patients and to identify positional parameters that may lead to its change.

Evaluation of the Patient-Practitioner Consultation on Surgical Treatment Options for Patients With Craniosynostosis.

Introduction: Endoscope-assisted craniectomy and spring-assisted cranioplasty with post-surgical helmet molding are minimally invasive alternatives to the traditional craniosynostosis treatment of open cranial vault remodeling. Families are often faced with deciding between techniques. This study aimed to understand providers' practice patterns in consulting families about surgical options.

Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus.

Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016.

Successful Increase of Outpatient Clinic Continuity in a Fellowship Quality Improvement Project.

Background: Patient-to-physician continuity is the result of coordinated and consistent care. Optimizing continuity can be a challenge in medical training without impacting work hours. We sought to use quality improvement science during graduate medical training to increase outpatient continuity.

Is rod diameter associated with the rate of rod fracture in patients treated with magnetically controlled growing rods?

Introduction: Few risk factors for fracture in magnetically controlled growing rods (MCGR) have been identified. We hypothesize an increased rate of rod fracture in small diameter rods compared to large diameter rods in patients with early-onset scoliosis (EOS). The purpose of this study was to determine the association between the diameter of MCGR constructs and the rate of rod fracture.

VEPTR Treatment of Early Onset Scoliosis in Children Without Rib Abnormalities: Long-term Results of a Prospective, Multicenter Study.

Background: In 2007, this prospective study on vertical expandable prosthetic titanium rib (VEPTR) treatment of early onset scoliosis in children without rib abnormalities was initiated. Two-year follow-up results have previously been reported. This study examines whether, at 5-year follow-up, VEPTR continues to control scoliosis and allow spinal growth.

Pulmonary Valve-Sparing Techniques for Tetralogy of Fallot: A Systematic Approach for Maximizing Success and Minimizing Risk.

Valve-sparing techniques for the treatment of Tetralogy of Fallot with pulmonary stenosis have evolved over the past few decades. This article will discuss the rationale for a more aggressive approach to sparing the pulmonary valve, the various techniques available (including commissurotomy, leaflet thinning and debridement, balloon dilation, and finally modified monocusp repair), as well as a systematic approach for employing these techniques based on individual patient anatomy in a manner that takes full advantages of the benefits of the valve-sparing approach while mitigating the risks involved.

It is not just about the frontal plane: sagittal parameters impact curve progression in AIS patients undergoing brace treatment.

Study Design: Retrospective cohort study.

Objectives: The purpose of this study was to explore the association between pre-brace and in-brace sagittal parameters and curve progression. To date, there has been no published research focused on spinopelvic sagittal parameters and bracing outcomes in AIS.

Skip constructs in spinal muscular atrophy: outcomes of a novel approach for posterior spinal instrumentation and fusion.

Purpose: Many children with spinal muscular atrophy (SMA) develop progressive spinal deformity, worsening already compromised pulmonary function and global spinal balance. Early results demonstrate that intrathecal administration of nusinersen, a recent FDA-approved drug, improves motor function and ventilator-free survival, necessitating preservation of intrathecal access when considering PSIF. The purpose of this study is to assess medium-term outcomes of a specialized approach for posterior spinal instrumentation and fusion (PSIF) to preserve intrathecal access in patients with SMA.

Effects of donor cause of death, ischemia time, inotrope exposure, troponin values, cardiopulmonary resuscitation, electrocardiographic and echocardiographic data on recipient outcomes: A review of the literature.

Background: Heart transplantation has become standard of care for pediatric patients with either end-stage heart failure or inoperable congenital heart defects. Despite increasing surgical complexity and overall volume, however, annual transplant rates remain largely unchanged. Data demonstrating pediatric donor heart refusal rates of 50% suggest optimizing donor utilization is critical.

Caregiver Characteristics Associated With Quality of Cardiac Compressions on an Adult Mannequin With Real-Time Visual Feedback: A Simulation-Based Multicenter Study.

Introduction: Chest compression (CC) quality directly impacts cardiac arrest outcomes. Provider body type can influence the quality of cardiopulmonary resuscitation (CPR); however, the magnitude of this impact while using visual feedback is not well described. The aim of the study was to determine the association between provider anthropometric variables on fatigue and CC adherence to 2015 American Heart Association CPR while receiving visual feedback.

Bigger is better: larger thoracic height is associated with increased health related quality of life at skeletal maturity.

Study Design: Cross sectional OBJECTIVES: The purpose of this study is to evaluate the association between thoracic height and health-related quality of life (HRQoL) at skeletal maturity in patients with EOS. Current literature suggests a minimum thoracic height of 18 cm to 22 cm to avoid poor pulmonary function and related health outcomes.

Methods: Patients with EOS who reached skeletal maturity from 2005 to 2018 were identified in two registries including 32 centers.

Implantable Cardioverter Defibrillator Use in Males with Duchenne Muscular Dystrophy and Severe Left Ventricular Dysfunction.

Duchenne muscular dystrophy (DMD) is characterized by myocardial fibrosis and left ventricular (LV) dysfunction. Implantable cardioverter defibrillator (ICD) use has not been characterized in this population but is considered for symptomatic patients with severe LV dysfunction (SLVD) receiving guideline-directed medical therapy (GDMT). We evaluated ICD utilization and efficacy in patients with DMD.

The landscape of cardiovascular care in pediatric cancer patients and survivors: a survey by the ACC Pediatric Cardio-Oncology Work Group.

Objective: To enhance the understanding of cardiovascular care delivery in childhood cancer patients and survivors.

Study Design: A 20-question survey was created by the Pediatric Cardio-oncology Work Group of the American College of Cardiology (ACC) Cardio-oncology Section to assess the care, management, and surveillance tools utilized to manage pediatric/young adult cardio-oncology patients. The survey distribution was a collaborative effort between Cardio-oncology Section and membership of the Adult Congenital and Pediatric Cardiology Section (ACPC) of the ACC.

Acupuncture for pediatric sickle cell pain management: A promising non-opioid therapy.

Objective: To describe the use of acupuncture for pain management in children with sickle cell disease.

Design: A retrospective chart review of a single-institution experience on the use of acupuncture in pediatric patients with sickle cell disease was evaluated between 2012-2019. Demographic characteristics, presenting pain location, pain scores pre- and post-acupuncture, and adverse events were collected.

Percutaneous Impella RP use for refractory right heart failure in adolescents and young adults-A multicenter U.S. experience.

Objective: To assess the outcomes of the use of the percutaneous Impella RP device (Abiomed, Danvers, MA) in adolescents and young adults.

Background: Results of the Impella RP device have been reported in adults, but a multicenter experience in adolescents and young adults has yet to be reported.

Methods: Patients ≤21 years of age who underwent implantation of an Impella RP device for refractory right heart failure from June, 2016 to April, 2018 at nine U.

Functional connectivity of the anterior insula associated with intolerance of uncertainty in youth.

Intolerance of uncertainty (IU) is a trait characteristic marked by distress in the face of insufficient information. Elevated IU has been implicated in the development and maintenance of anxiety disorders, particularly during adolescence, which is characterized by dramatic neural maturation and the onset of anxiety disorders. Previous task-based work implicates the bilateral anterior insula in IU.

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

Objective: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.

Methods: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%.