The ACTION VAD Registry: A Collective Five-Year Experience.

Background: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) began in 2018 as a collaborative learning health system committed to improving outcomes in pediatric heart failure, including children and adults with congenital heart disease, supported with ventricular assist devices (VADs). This report describes patient and device characteristics, and outcomes through 1-year post-implant.

Methods: The ACTION VAD registry report was created from data submitted to the ACTION learning network from April 2018-June 2023.

Six-Month Outcomes in the Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children Study.

Importance: Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited.

Objective: To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms (z score ≥2.

2024 HRS perspective on advancing workflows for CIED remote monitoring.

Cardiac implantable electronic devices (CIEDs) generate substantial data, often stored in image or PDF formats. Remote monitoring, now an integral component of patient care, places considerable administrative burdens on clinicians and staff, in large part due to the challenge of integrating these data seamlessly into electronic health records. Since 2006, the Heart Rhythm Society, in collaboration with the CIED industry, has led an initiative to establish a unified standard nomenclature.

Implementation of a Checklist Tool to Improve Understanding of Rapid Response Team Protocols.

Rapid response teams (RRT) are multidisciplinary teams activated to assess patients outside of the Intensive Care Unit (ICU) and require a shared framework for approaching a deteriorating patient. In this project, we aimed to improve the understanding of RRT protocols through the development and implementation of the RRT Huddle Checklist. This quality improvement project took place from 2019 to 2022.

Outcomes of an Individualized Hybrid Model for Repair of Infants With Congenital Diaphragmatic Hernia Requiring Extracorporeal Life Support.

Introduction: Timing of repair for infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS) remains controversial. Approaches include early repair on ECLS, late repair on ECLS, or repair after ECLS decannulation; all have potential risks and benefits. To mitigate risk and maximize benefit, our group developed an individualized hybrid model in 2016 in which approach is based on prenatal risk stratification.

Outcomes of Growth-Friendly Surgical Treatment of Early Onset Scoliosis in Children With Prune Belly Syndrome: A Preliminary Report.

Background: Prune belly syndrome (PBS) is a rare condition characterized by absence of abdominal musculature, cryptorchidism, and obstructive uropathy. The most common orthopaedic problem is scoliosis, yet no reports on growth-friendly surgical treatment of early-onset scoliosis (EOS) exist. Our purpose was to evaluate outcomes of distraction-based implants in children with PBS.

Over 100 Fontan Patients on Systemic Ventricular Assist Device Support: An ACTION Update.

As the Fontan population grows, understanding successful strategies for ventricular assist device (VAD) support of the failing Fontan circulation is needed. We performed a retrospective analysis of patients with Fontan circulation and systemic VAD support in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. Competing outcomes and Kaplan-Meier estimated survival methods were used.

Regional Anesthesia and Surgical Volume in Children Undergoing Nuss Repair: A Multicenter Review.

Introduction: Regional anesthetic approach and surgical volume have been shown to outcomes in patients undergoing Nuss procedure for pectus excavatum. However, their independent relationship is not described. We investigated how regional anesthesia and surgical volume are associated with length of stay (LOS), postoperative opioid use, operating room utilization, and complications.

Best practice peri-extubation bundle reduces neonatal and infant extubation failure after cardiac surgery.

Introduction: Extubation failure after neonatal cardiac surgery is associated with increased intensive care unit length of stay, morbidity, and mortality. We performed a quality improvement project to create and implement a peri-extubation bundle, including extubation readiness testing, spontaneous breathing trial, and high-risk criteria identification, using best practices at high-performing centers to decrease neonatal and infant extubation failure by 20% from a baseline of 15.7% to 12.

Machine Learning-Based Prediction Model for ICU Mortality After Continuous Renal Replacement Therapy Initiation in Children.

Background: Continuous renal replacement therapy (CRRT) is the favored renal replacement therapy in critically ill patients. Predicting clinical outcomes for CRRT patients is difficult due to population heterogeneity, varying clinical practices, and limited sample sizes.

Objective: We aimed to predict survival to ICUs and hospital discharge in children and young adults receiving CRRT using machine learning (ML) techniques.

LIV selection in 'tweener' patients treated with magnetically controlled growing rods vs. posterior spinal fusion.

Purpose: The purpose of this study was to compare the LIV selection in 'tweener' patients treated with MCGR or PSF.

Methods: A multicenter pediatric spine database was queried for ambulatory patients ages 8-11 years treated by MCGR or PSF with at least 2-year follow-up. The relationship between the LIV and preoperative spinal height, curve magnitude, and implant type were assessed.

Outcomes Following Fecal Diversion for Intractable Hirschsprung Associated Enterocolitis: A Study From the Pediatric Colorectal and Pelvic Learning Consortium.

Background: Hirschsprung associated enterocolitis (HAEC) is a challenging problem in a subset of children with Hirschsprung disease (HD). In refractory cases, fecal diversion may be required. The aim of this study was to characterize patients who require fecal diversion for HAEC management and examine their long-term outcomes.

A pilot study of the WE BEAT Well-Being Education Programme to build resilience in adolescents with heart disease.

Objective(s): To examine feasibility, acceptability, and preliminary effectiveness of a novel group-based telemedicine psychoeducation programme aimed at supporting psychological well-being among adolescents with Fontan-palliated CHD.

Study Design: A 5-week telemedicine psychoeducation group-based programme (WE BEAT) was developed for adolescents ( = 20; 13-18 years) with Fontan-palliated CHD aimed at improving resiliency and psychological well-being. Outcome measures included surveys of resilience (Connor-Davidson Resilience Scale), benefit finding (Benefit/Burden Scale for Children), depression, anxiety, peer relationships, and life satisfaction (National Institutes of Health Patient-Reported Outcomes Measurement Information System scales).

Provider Perceptions of Outcomes in the Pediatric Cardiac Intensive Care Unit.

In the pediatric cardiac intensive care unit (PCICU), predicting the complications and long-term impact of extracorporeal membrane oxygenation (ECMO) and cardiopulmonary resuscitation (CPR) is influenced by the providers' perceptions. Little is understood about such perceptions as they relate to provider role and experience. A multi-disciplinary group in the PCICU of a single center was surveyed regarding two patient scenarios: (1) ECMO after cardiac surgery, and (2) cardiac arrest with need for CPR.

Perturbations of tryptophan catabolism via the kynurenine pathway are associated with stage 2 postoperative outcomes in single ventricle heart disease.

Preliminary evidence suggests perturbations of the kynurenine pathway (KP) of tryptophan metabolism in infants with single ventricle heart disease (SVHD). In 72 infants with SVHD undergoing stage 2 palliation (S2P) and 41 controls, we quantified serum KP metabolite concentrations via tandem mass spectroscopy pre-S2P and post-S2P at 2, 24, and 48 h and assessed metabolite relationships with post-S2P outcomes (length of stay, hypoxemia burden, and intubation duration). Pre-S2P, SVHD infants had lower tryptophan and serotonin levels and higher kynurenic acid, 3-hydroxykynurenine, and picolinic acid levels than controls.

Elevating diversity, inclusion, and health equity in Pediatric Heart Network Scholars grant funding: unique opportunities and lessons learned.

There is a growing awareness that diversity, health equity, and inclusion play a significant role in improving patient outcomes and advancing knowledge. The Pediatric Heart Network launched an initiative to incorporate diversity, health equity, and inclusion into its 2021 Scholar Award Funding Opportunity Announcement. This manuscript describes the process of incorporating diversity, health equity, and inclusion into the Pediatric Heart Network Scholar Award and the lessons learned.

Cracking the code of the slow code: A taxonomy of slow code practices and their clinical and ethical implications.

The ethical permissibility of the "slow code" sparks vigorous debate. However, definitions of the "slow code" that exist in the literature often leave room for interpretation. Thus, those assessing the ethical permissibility of the slow code may not be operating with shared definitions, and definitions may not align with clinicians' understanding and use of the term in clinical practice.

Management of patent ductus arteriosus - Evidence to practice.

Over the past last 50 years, patent ductus arteriosus (PDA) continues to be the leading hot topic debated worldwide in search of best treatment approach and the uncertainty around whether to treat or not treat a PDA. With the availability of bedside echocardiography and the increasing number of neonatologists acquiring this skill, on one hand there is better understanding of PDA physiology during transitional circulation and objectivity in management, but on the other hand clinicians are uncertain about benefits in health outcomes. Evidence from recent trials utilizing early selective treatment guided by bedside echocardiography should help in dispelling some myths if not providing the answer about how to manage the PDA.

Transition to Enteral Triazole Antifungal Therapy for Pediatric Invasive Candidiasis: Secondary Analysis of a Multicenter Cohort Study Conducted by the Pediatric Fungal Network.

Of 319 children with invasive candidiasis, 67 (21%) transitioned from intravenous to enteral antifungal therapy. Eight (12%) transitioned back to intravenous antifungal therapy, one due to perceived treatment failure defined by clinical progression or worsening. Global treatment response at study completion was successful in 66 participants who transitioned to enteral therapy.

Expanding the Molecular and Clinical Phenotype of Patients With De Novo Variants in KIF5C: A Six Patient Case Series.

Heterozygous de novo loss of function variants in the motor domain of KIF5C are associated with a neurodevelopmental disorder characterized by infantile-onset epilepsy, frontal cortical dysplasia, and developmental delays including motor and speech impairments. Previously, only three missense variants in KIF5C were known to be pathogenic. We identified an additional six patients with significant developmental delays with heterozygous de novo variants in the KIF5C gene (Glu237Val, Thr93Ile, Thr93Asn, Ser90del, Lys92Arg, and Glu237Lys), of which four variants have not been reported before.