ACTION-ARC Pediatric and Adult Congenital Heart Disease Ventricular Assist Device Adverse Event Definitions-2023.

Adverse events (AEs) experienced by children and adults with congenital heart disease (CHD) on ventricular assist devices (VADs) are sometimes unique to these populations. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and the Academic Research Consortium (ARC) aimed to harmonize definitions of pediatric and CHD AEs for use in clinical trials, registries, and regulatory evaluation. Data from the ACTION registry and adjudication committee were used to adapt general mechanical circulatory support ARC definitions.

A 1-Day-Old Girl With Infantile Hemangioma and Sternal Cleft.

A newborn girl presented to the hospital on the first day of life because of respiratory failure. She was born at home at 37 weeks' gestation with minimal prenatal care and was found to be small for gestational age. The patient was found to have partial sternal agenesis and sternal cleft, cutis aplasia, left facial hemangioma, micrognathia, wide-spaced nipples, and low-set ears.

A prospective multicenter feasibility study of a miniaturized implantable continuous flow ventricular assist device in smaller children with heart failure.

Background: There is no FDA-approved left ventricular assist device (LVAD) for smaller children permitting routine hospital discharge. Smaller children supported with LVADs typically remain hospitalized for months awaiting heart transplant-a major burden for families and a challenge for hospitals. We describe the initial outcomes of the Jarvik 2015, a miniaturized implantable continuous flow LVAD, in the NHLBI-funded Pumps for Kids, Infants, and Neonates (PumpKIN) study, for bridge-to-heart transplant.

Dexmedetomidine Withdrawal Syndrome in Children in the PICU: Systematic Review and Meta-Analysis.

Objectives: To systematically review literature describing the clinical presentation, risk factors, and treatment for dexmedetomidine withdrawal in the PICU (PROSPERO: CRD42022307178).

Data Sources: MEDLINE/PubMed, Cochrane, Web of Science, and Scopus databases were searched.

Study Selection: Eligible studies were published from January 2000 to January 2022 and reported clinical data for patients younger than 21 years old following discontinuation of dexmedetomidine after greater than or equal to 24 hours of infusion.

Educating Surgeons to Construct Valved Conduits: What Does It Take to Achieve Reproducible Results?

Background: Despite the demonstrated utility of surgeon-fashioned polytetrafluoroethylene (PTFE) valved conduits, methods for educating surgeons to reproducibly construct these conduits are lacking. We present a surgeon education process and early outcomes for children receiving surgeon-fashioned PTFE valved conduits during the initial learning curve.

Methods: The educational curriculum included 4 hours of proctored instruction/supervised valve construction, followed by 2 hours of individual practice.

Capturing Adult Congenital Heart Disease: Framework for Development of an Adult Congenital Heart Disease Mortality Risk Model.

Background: As a formative step toward development of adult congenital heart disease (ACHD) risk-adjusted metrics, we describe The Society of Thoracic Surgeons (STS) ACHD population, procedural frequency, and early mortality.

Methods: Adults (≥18 years) with CHD (2014-2019) were identified in the STS Adult Cardiac Surgery (ASCD) and the Congenital Heart Surgery (CHSD) Databases. After deduplication, variable mapping, data concatenation, and harmonization of preprocedure factors, procedures were grouped, and unadjusted mortality was catalogued for the overall cohort and the cohort excluding patients with an isolated bicuspid aortic valve (BAV).

A shifting landscape: Practice patterns and outcomes of cystic fibrosis and non-cystic fibrosis pediatric lung transplantation.

Background: New drugs may further decrease the need for lung transplant (LTx) in pediatric patients with cystic fibrosis (CF), but few studies highlight pediatric non-CF LTx characteristics and outcomes.

Methods: The ISHLT registry was used to report morbidity, graft failure, and survival for primary pediatric (<18 years) LTx performed 1990-2017. Recipient/donor characteristics and long-term outcomes were analyzed for CF and non-CF recipients.

A Minimal Opioid Postoperative Management Protocol in Congenital Cardiac Surgery: Safe and Effective.

There is evidence that reducing opioid exposure in children undergoing cardiac surgery may enhance postoperative recovery. We aimed to describe a minimal opioid postoperative management protocol in children undergoing cardiac surgery and our early outcomes with this strategy. We reviewed the medical records of children (6 months-18 years) who underwent elective cardiac surgery through a median sternotomy with cardiopulmonary bypass at our institution between 2016 and 2018.

A 22-Month-Old Girl With 3 Weeks of Dyspnea.

A previously healthy 22-month-old girl presented to the ED with a 3-week history of dyspnea on exertion. A chest radiograph showed a right upper-lobe opacity suspicious for pneumonia (Fig 1A). The patient was prescribed amoxicillin but returned to the ED 7 days later with cough and persistent dyspnea and tachypnea.

Aortic Valve Replacement in Young and Middle-Aged Adults: Current and Potential Roles of TAVR.

Background: Contemporary practice patterns and outcomes for aortic valve replacement (AVR) among young and middle-aged adults are unknown given guideline modifications for surgical AVR (SAVR) and increasing transcatheter AVR (TAVR) acceptance. This study describes SAVR and TAVR use and outcomes using The Society of Thoracic Surgeons (STS) National Databases.

Methods: Adults 18 to 55 years of age in the Congenital Heart Surgery Database (CHSD) and the Adult Cardiac Surgery Database (ACSD) who underwent SAVR or TAVR from 2013 to 2018 were included.

Impact of Circuit Size on Coagulation and Hemolysis Complications in Pediatric Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation (ECMO) circuit volume, patient size, and blood flow may influence coagulation and hemolysis complications. We performed a single-center retrospective analysis of ECMO patients over a 6.5 year period.

Surgery for Dysphagia Lusoria in Children.

Aberrant right subclavian artery, or arteria lusoria, is a common congenital anomaly of the aortic arch and may be associated with dysphagia. Surgical treatment, particularly in children, remains controversial. Current surgical interventions include supraclavicular and thoracotomy approaches, as well as hybrid endovascular techniques.

Cardiac venous malformation in a teenager.

We present the case of a 17-year-old boy with a cardiac venous malformation. This case highlights the diagnostic challenges of such tumours and demonstrates the potential efficacy of a watch-and-wait management approach.

National Practice Patterns and Early Outcomes of Aortic Valve Replacement in Children and Teens.

Background: Several options exist for aortic valve replacement (AVR) in children and teens, but contemporary practice patterns and outcome data are lacking. We describe national AVR practice patterns and early outcomes.

Methods: Children (aged 1 to 18 years) in The Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing AVR from 2000 to 2016 were included.

Acute In Vivo Evaluation of the Pittsburgh Pediatric Ambulatory Lung.

Respiratory failure is a significant problem within the pediatric population. A means of respiratory support that readily allows ambulation could improve treatment. The Pittsburgh Pediatric Ambulatory Lung (P-PAL) is being developed as a wearable pediatric pump-lung for long-term respiratory support and has previously demonstrated positive benchtop results.

Paracorporeal Lung Devices: Thinking Outside the Box.

Extracorporeal Membrane Oxygenation (ECMO) is a resource intensive, life-preserving support system that has seen ever-expanding clinical indications as technology and collective experience has matured. Clinicians caring for patients who develop pulmonary failure secondary to cardiac failure can find themselves in unique situations where traditional ECMO may not be the ideal clinical solution. Existing paracorporeal ventricular assist device (VAD) technology or unique patient physiologies offer the opportunity for thinking "outside the box.

Preclinical performance of a pediatric mechanical circulatory support device: The PediaFlow ventricular assist device.

Objectives: The PediaFlow (HeartWare International, Inc, Framingham, Mass) is a miniature, implantable, rotodynamic, fully magnetically levitated, continuous-flow pediatric ventricular assist device. The fourth-generation PediaFlow was evaluated in vitro and in vivo to characterize performance and biocompatibility.

Methods: Supported by 2 National Heart, Lung, and Blood Institute contract initiatives to address the limited options available for pediatric patients with congenital or acquired cardiac disease, the PediaFlow was developed with the intent to provide chronic cardiac support for infants as small as 3 kg.

Cost-Effectiveness of Ventricular Assist Device Destination Therapy for Advanced Heart Failure in Duchenne Muscular Dystrophy.

Destination ventricular assist device therapy (DT-VAD) is well accepted in select adults with medically refractory heart failure (HF) who are not transplant candidates; however, its use in younger patients with progressive diseases is unclear. We sought to evaluate the cost-effectiveness of DT-VAD in Duchenne muscular dystrophy (DMD) patients with advanced HF. We created a Markov-state transition model (5-year horizon) to compare survival, costs, and quality of life (QOL) between medical management and DT-VAD in DMD with advanced HF.

Surfactant Administration During Pediatric Cardiac Extracorporeal Membrane Oxygenation.

We investigated the safety and efficacy of surfactant during extracorporeal membrane oxygenation (ECMO) in children with cardiac disease. ECMO patients administered surfactant (surfactant group) were compared with patients who did not receive (control). Criteria to administer surfactant were based on a decreased lung compliance of <0.

In Vitro Characterization of the Pittsburgh Pediatric Ambulatory Lung.

Acute and chronic respiratory failure are a significant source of pediatric morbidity and mortality. Current respiratory support options used to bridge children to lung recovery or transplantation typically render them bedridden and can worsen long-term patient outcomes. The Pittsburgh Pediatric Ambulatory Lung (P-PAL) is a wearable pediatric blood pump and oxygenator (0.

Airway ciliary dysfunction: Association with adverse postoperative outcomes in nonheterotaxy congenital heart disease patients.

Objective: Heterotaxy (HTX) congenital heart disease (CHD) patients with ciliary dysfunction (CD) have been shown to have increased postoperative respiratory morbidity. We hypothesized that non-HTX CHD infants with CD also will have increased postoperative morbidity, particularly respiratory complications.

Methods: Sixty-three infants with non-HTX CHD undergoing cardiac surgery were enrolled.

Cell-Free Plasma Hemoglobin and Male Gender Are Risk Factors for Acute Kidney Injury in Low Risk Children Undergoing Cardiopulmonary Bypass.

Objectives: To determine the relationship between the production of cell-free plasma hemoglobin and acute kidney injury in infants and children undergoing cardiopulmonary bypass for cardiac surgery.

Design: Prospective observational study.

Setting: Twelve-bed cardiac ICU in a university-affiliated children's hospital.

Brain Dysplasia Associated with Ciliary Dysfunction in Infants with Congenital Heart Disease.

Objective: To test for associations between abnormal respiratory ciliary motion (CM) and brain abnormalities in infants with congenital heart disease (CHD) STUDY DESIGN: We recruited 35 infants with CHD preoperatively and performed nasal tissue biopsy to assess respiratory CM by videomicroscopy. Cranial ultrasound scan and brain magnetic resonance imaging were obtained pre- and/or postoperatively and systematically reviewed for brain abnormalities. Segmentation was used to quantitate cerebrospinal fluid and regional brain volumes.