Assessment of echocardiographic interpretation of dual-lumen cannula during venovenous extracorporeal membrane oxygenation use for pediatric respiratory failure.

Purpose: Echocardiography is considered essential during cannulation placement and manipulations. Literature evaluating transthoracic echocardiography (TTE) usage during pediatric VV-ECMO is scant. The purpose of this study is to describe the use of echocardiography during VV-ECMO at a large, quaternary children's hospital.

Nonsense Variant PRDM16-Q187X Causes Impaired Myocardial Development and TGF-β Signaling Resulting in Noncompaction Cardiomyopathy in Humans and Mice.

Background: PRDM16 plays a role in myocardial development through TGF-β (transforming growth factor-beta) signaling. Recent evidence suggests that loss of PRDM16 expression is associated with cardiomyopathy development in mice, although its role in human cardiomyopathy development is unclear. This study aims to determine the impact of PRDM16 loss-of-function variants on cardiomyopathy in humans.

Atypical infiltrates on endomyocardial biopsy are associated with adverse outcomes in pediatric heart transplantation.

Background: The significance of atypical infiltrates (eosinophils or plasma cells) on endomyocardial biopsy (EMB) after pediatric heart transplant (HTx) is not known. We hypothesized that atypical infiltrates are associated with worse post-HTx outcomes.

Methods: We performed a retrospective cohort study of consecutive patients <21 years old who underwent primary HTx between 2013 and 2017.

Extracorporeal Membrane Oxygenation Cannulation Timing in the Pediatric Myocarditis Population: An Exploratory Analysis From the Extracorporeal Life Support Organization Registry.

Unlabelled: Children presenting with acute myocarditis may experience rapid clinical deterioration requiring extracorporeal membrane oxygenation (ECMO); however, our understanding of best practices and timing of ECMO initiation are lacking. We explored the relationships between pre-cannulation factors and survival in this high-acuity patient population.

Design: Retrospective review of a large international registry.

Arrhythmogenic cardiomyopathy is under-recognized in end-stage pediatric heart failure: A 36-year single-center experience.

Background: Although ventricular failure is a late finding in adults with AC, we hypothesize that this is a presenting symptom in pediatric heart failure patients who undergo HT and that their ventricular arrhythmia burden could differentiate AC from other cardiomyopathies.

Methods: We performed a single-center retrospective cohort study reviewing 457 consecutive pediatric (≤18 years) HT recipients at our institution. Explanted hearts were examined to establish the primary diagnosis, based on pathologic findings.

Relation of Low Chloride Concentration to Diuretic Efficiency and Transplant-Free Survival in Children Hospitalized With Heart Failure.

Serum chloride plays an important role in fluid homeostasis and is associated with impaired diuretic responsiveness and mortality in adults with heart failure (HF). We sought to characterize the relationship of serum chloride and diuretic efficiency (DE) and to determine its prognostic importance in children hospitalized with acute decompensated HF (ADHF). We studied DE, defined as net fluid output/kg+constant per mg of loop diuretic/kg, in 200 children hospitalized with ADHF.

Ventricular assist device support for children with chemotherapy-induced cardiomyopathy and advanced heart failure: Perspectives gained from a single-center experience.

Background: Guidance and data on ventricular assist device (VAD) support for children with chemotherapy-induced cardiomyopathy, particularly within the first 2 years after chemotherapy, are limited.

Methods: We performed a single-center retrospective case series, reviewing medical records of children <18 years of age with chemotherapy-induced cardiomyopathy and advanced heart failure (HF) who received durable VAD support.

Results: Six patients met inclusion criteria-5 HeartWare™ HVAD, 1 Berlin Heart EXCOR .

Does Cardiac Catheterization Facilitate Hemodynamic Optimization of Pediatric Patients on Continuous-Flow Ventricular Assist Devices?

Understanding optimal ventricular assist device (VAD) parameters for pediatric patients is valuable given the inherent issue of patient-device size mismatch and heterogeneous cardiac anatomy in children. We evaluated our center's experience of continuous-flow VAD (CF-VAD) optimization using cardiac catheterization. We performed a retrospective analysis of all patients on CF-VAD support who underwent hemodynamic heart catheterization from 2013 to 2018.

Global Longitudinal Strain Analysis of the Single Right Ventricle: Leveling the Playing Field.

Background: All available echocardiographic methods to assess single systemic right ventricular systolic function have limitations. Subjective grading is prone to bias and varies among readers. Quantitative methods that require significant manual input, such as fractional area change (FAC), are often not reproducible.

LMOD2-related dilated cardiomyopathy presenting in late infancy.

Leiomodin-2 (LMOD2) is an important regulator of the thin filament length, known to promote elongation of actin through polymerization at pointed ends. Mice with Lmod2 deficiency die around 3 weeks of age due to severe dilated cardiomyopathy (DCM), resulting from decreased heart contractility due to shorter thin filaments. To date, there have been three infants from two families reported with biallelic variants in LMOD2, presenting with perinatal onset DCM.

Immunosuppressant Drugs and Their Effects on Children Undergoing Solid Organ Transplant.

More than 112,000 men, women, and children are awaiting solid organ transplant (SOT) as of March 2020, and more than 39,000 transplants were performed in the United States in 2019. Approximately 2,000 children undergo SOT every year in the United States, and the number of children awaiting SOT continues to increase. Immunosuppression is the mainstay of prevention and treatment of solid organ rejection, a significant source of morbidity and mortality after SOT.

Trends in Body Mass Index and Association With Outcomes in Pediatric Patients on Continuous Flow Ventricular Assist Device Support.

Data are limited regarding body mass index (BMI) in pediatric patients supported by ventricular assist devices (VAD) and associated clinical outcomes and complications. We performed a retrospective single-center cohort study including patients aged ≤21 years on durable continuous-flow VAD support for ≥30 days from 2009 to 2020. Patients were classified based on BMI percentile at implant using the US Centers for Disease Control and Prevention criteria: underweight (<5th percentile), healthy weight (5th-<85th percentile, reference group), overweight (85th-<95th percentile), and obese (≥95th percentile).

Outcomes following use of pediatric and young adult donor hearts with bicuspid aortic valves: A single-center case series.

Background: Valvular disease in pediatric and young adult donor hearts may be a relative contraindication to graft use. Outcomes following the use of donor hearts with bicuspid aortic valve (BAV) have not been previously reported in children. We describe 4 cases of pediatric heart transplantation (HTx) utilizing a donor heart with a BAV.

Management of Advanced Heart Failure in Children with Cancer Therapy-Related Cardiac Dysfunction.

The evolution of cancer therapies has led to marked improvement in survival of those affected by childhood malignancies, while also increasing the recognition of early and late toxicities associated with cancer therapies. Cardiotoxicity can include cardiomyopathy/heart failure, coronary artery disease, stroke, pericardial disease, arrhythmias, and valvular and vascular dysfunction as a result of exposure to chemotherapy and/or radiation. Anthracyclines remain the most common cause of chemotherapy-induced cardiomyopathy (CCM) with varying clinical presentations including: acute, early onset, and late-onset.

Poor efficacy of oral iron replacement therapy in pediatric patients with heart failure.

Introduction: Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure.

Simultaneous pediatric heart-kidney transplant outcomes in the US: A-25 year National Cohort Study.

Background: Pediatric sHKTx remains uncommon in the US. We examined outcomes of pediatric sHKTx compared to PHTx alone. Our objective was to identify a threshold eGFR that justified pediatric sHKTx.

Pediatric heart-liver transplant outcomes in the United States: A 25-year National Cohort Study.

Background: Pediatric HLT remains uncommon in the United States and criteria for HLT are unclear. The objectives of this study were to review the indications, and outcomes of pediatric HLT.

Methods: Data from the Scientific Registry of Transplant Recipients heart and liver databases were used to identify 9245 pediatric isolated heart transplants (PHT), 14 134 pediatric isolated liver transplant (PLT), and 20 pediatric HLT (16 patients underwent sHLT [same organ donor] and four patients with a history of PHT followed by PLT [different organ donors]; age ≤21 years) between 1992 and 2017.

"Compassionate" Cases of the Jarvik 2015 Ventricular Assist Device.

The Jarvik 2015 Ventricular Assist Device (VAD) (Jarvik Inc, New York, NY) is the first and currently only continuous-flow VAD specifically designed for small children, and it is being evaluated in the so-called Pump for Kids, Infants, and Neonates (PumpKIN) trial. Due to the strict inclusion criteria of the trial, there have been a group of patients who failed to meet the criteria and therefore received the Jarvik 2015 VAD under the designation of "compassionate use." This is the same phenomenon seen previously during the Berlin Heart EXCOR trial.

Amino Acid-Level Signal-to-Noise Analysis Aids in Pathogenicity Prediction of Incidentally Identified -Encoded Titin Truncating Variants.

Background: , the largest gene in the human body, encodes TTN (titin), a protein that plays key structural, developmental, and regulatory roles in skeletal and cardiac muscle. Variants in , particularly truncating variants (TTNtvs), have been implicated in the pathogenicity of cardiomyopathy. Despite this link, there is also a high burden of TTNtvs in the ostensibly healthy general population.

Hospital outcomes for pediatric heart transplant recipients undergoing tracheostomy: A multi-institutional analysis.

Tracheostomy is associated with increased mortality and resource utilization in children with CHD. However, the prevalence and hospital outcomes of tracheostomy in children with HTx are not known. We describe the prevalence and compare the post-HTx hospital outcomes of pediatric patients with Pre-TT and Post-TT to those without tracheostomy.