Hybrid approach for harmony transcatheter pulmonary valve replacement.

The Harmony™ Transcatheter Pulmonary Valve (Medtronic) was recently approved by the Food and Drug Administration for transcatheter pulmonary valve replacement in native right ventricular outflow tracts. Despite this milestone, some patients have main pulmonary arteries that are severely dilated and continue to require surgical pulmonary valve replacement. The hybrid approach combines surgical creation of a landing zone, transcatheter valve deployment, and suture stabilization of the implanted valve.

Management of Ventral Brainstem Compression in Chiari Malformation Type I.

Craniovertebral junction (CVJ) abnormalities are associated with the Chiari malformation type I. These abnormalities may lead to ventral brainstem compression which can be reduced with traction and posterior reduction using instrumentation. In other cases, the irreducible CVJ pathology with persistent ventral brainstem compression requires ventral decompression.

Refractory Pediatric Fibrostenotic Eosinophilic Esophagitis Treated With Dupilumab.

Eosinophilic esophagitis (EoE) is a progressive inflammatory disease of the esophagus. Untreated or uncontrolled disease over time can lead to the development of fibrosis and formation of strictures. Once the patient develops strictures, it is difficult to treat with the available medical therapies and will often require esophageal dilations.

Clinical approach to antibody-mediated rejection from the pediatric heart transplant society.

This document is designed to outline the definition, pathogenesis, diagnostic modalities and therapeutic measures to treat antibody-mediated rejection in children postheart transplant METHODS: Literature review was conducted by a Pediatric Heart Transplant Society (PHTS) working group to identify existing pediatric and adult studies on antibody-mediated rejection (AMR). In addition, the centers participating in PHTS were asked to submit their approach to diagnosis and management of pediatric AMR. This document synthesizes information gathered from both these sources to highlight a practical approach to diagnosing and managing a child with AMR postheart transplant.

Left ventricular hypertrophy in pediatric patients on maintenance dialysis: a report of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS).

Background: Cardiovascular disease is the leading cause of death in pediatric patients with kidney failure. Left ventricular hypertrophy (LVH) is recognized as the most common cardiovascular abnormality in these patients. There remains a paucity of longitudinal studies evaluating LVH during maintenance dialysis in children.

Acute kidney injury requiring kidney replacement therapy in childhood lupus nephritis: a cohort study of the Pediatric Nephrology Research Consortium and Childhood Arthritis and Rheumatology Research Alliance.

Background: Acute kidney injury (AKI) is common in lupus nephritis (LN) and a risk factor for development of chronic kidney disease. In adults with LN, AKI severity correlates with the incidence of kidney failure and patient survival. Data on AKI outcomes in children with LN, particularly those requiring kidney replacement therapy (KRT), are limited.

A Rare Presentation of New-Onset Type 1 Diabetes Mellitus in a Developmentally Delayed Child With an Overlap of Diabetic Ketoacidosis and Hyperglycemic Hyperosmolar State.

Diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar state (HHS) are serious complications associated with diabetes mellitus (DM). HHS is a common diagnosis in adults but rare in children. DKA is a usual presentation for new-onset type 1 DM, although HHS is rarely a manifestation of new-onset type 1 DM.

Permissive Cardiotoxicity: The Clinical Crucible of Cardio-Oncology.

The field of cardio-oncology was born from the necessity for recognition and management of cardiovascular diseases among patients with cancer. This need for this specialty continues to grow as patients with cancer live longer as a result of lifesaving targeted and immunologic cancer therapies beyond the usual chemotherapy and/or radiation therapy. Often, potentially cardiotoxic anticancer treatment is necessary in patients with baseline cardiovascular disease.

Parenting and addictions: Current insights from human neuroscience.

Purpose: A growing body of human research has documented associations between the maternal brain and maternal substance use and addictions. This neuroscience-informed approach affords the opportunity to unpack potential neurobiological mechanisms that may underscore challenges in maternal caregiving behavior among mothers with addictions and provide new directions for parenting interventions.

Findings: Consistent with theoretical models of parenting and addictions, five studies evidence both hypo- and hyper-reactivity to infant affective cues across neuroimaging methods and tasks that incorporate both infant face and cry stimuli.

How to diagnose and treat acute pulmonary hypertension when you have no cardiology support.

Acute pulmonary hypertension (aPH) is a complex, physiology-driven disorder that causes critical illness in newborns, the hallmark of which is elevated pressure in the pulmonary vascular bed. Several underlying hemodynamic phenotypes exist, including classic arterial aPH with resistance-driven elevations in pulmonary arterial pressure (PAP), alongside flow-driven aPH from left-to-right shunt lesions, and primary left ventricular dysfunction with pulmonary venous hypertension and elevated left atrial pressure. Targeted neonatal echocardiography (TnECHO) is an important tool for evaluation of hemodynamics in aPH and is highly useful for evaluating modulators of disease and targeting cardiovascular therapy.

Gastric Xylobezoar Treated with Continuous Enteral Coca-Cola® Infusion.

Xylobezoar is a rare clinical condition in which undigested paper becomes trapped in the gastrointestinal tract causing varying degrees of gastrointestinal obstruction. This condition can be suspected in children with a history of gastric surgeries, decreased gastrointestinal motility, or pica. Xylobezoar presents with symptoms ranging from chronic abdominal pain to gastrointestinal perforation.

Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study.

Background: Multicenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE).

Methods: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009 and 2019 in 19 centers were reviewed.

Factors associated with pediatric genetic counselors' practices related to bullying screening.

Bullying is reported in around 20% of children according to the US Department of Education and has been reported in the histories of individuals with genetic disorders. To our knowledge, there has never been a study surveying whether genetic counselors screen their pediatric patients for bullying. This is despite guidelines that pediatric healthcare providers should screen for bullying.

Gelatin sponge to close a tract after large-bore transhepatic access: tips for success.

Percutaneous transhepatic venous access has been utilised for numerous transcatheter cardiac procedures. Traditionally, a large transhepatic tract requires the placement of permanent occlusion devices or coils. We describe a successful closure using a simple technique (Surgifoam) without the need for metal hardware placement.

Synergistic association of fluid overload and acute kidney injury on outcomes in pediatric cardiac ECMO: a retrospective analysis of the KIDMO database.

Background: Acute kidney injury (AKI) and fluid overload (FO) are associated with poor outcomes in children receiving extracorporeal membrane oxygenation (ECMO). Our objective is to evaluate the impact of AKI and FO on pediatric patients receiving ECMO for cardiac pathology.

Methods: We performed a secondary analysis of the six-center Kidney Interventions During Extracorporeal Membrane Oxygenation (KIDMO) database, including only children who underwent ECMO for cardiac pathology.

Pediatric-to-Adult Transfer of Care in Patients With Pancreas Disease: Recommendations for Care and Research Opportunities.

Young adults who have experienced recurrent acute pancreatitis and chronic pancreatitis as children or adolescents are vulnerable to poor follow-up and disease management during the transfer from the pediatric to adult healthcare system. Although formalized transition programs for young adults have been developed and described for other disease conditions, no such program has been described for young adults with pancreatic disease. This document is the first expert opinion outlining the important aspects of a transitional care and transfer program tailored to youth with recurrent acute and chronic pancreatitis.

Single-Center Experience of Hybrid Pulmonary Valve Replacement Using Left Anterior Thoracotomy With Pulmonary Artery Plication in Patients With Large Right Ventricular Outflow Tract.

Background Until recently, a large right ventricle outflow tract interfered with the feasibility of standard transcatheter pulmonary valve replacement (PVR). We are describing our experience using a hybrid approach for PVR using a left anterior thoracotomy approach to allow for plication of the main pulmonary artery followed by a transcatheter PVR using a Sapien S3 valve. Methods and Results This is a single-center, retrospective review of patients who were evaluated to be appropriate for a hybrid PVR approach.

Partially unroofed coronary sinus in an infant with pulmonary vein stenosis: mixed blessing.

Transcatheter intervention on the pulmonary vein often requires performing transseptal puncture, which is a risky procedure. We describe a rare association of a partially unroofed coronary sinus with pulmonary vein stenosis causing desaturation in a young infant where the presence of the unroofed coronary sinus allowed for a novel interventional approach to the stenotic left pulmonary vein, avoiding the high risk of transseptal puncture.

WASp modulates RPA function on single-stranded DNA in response to replication stress and DNA damage.

Perturbation in the replication-stress response (RSR) and DNA-damage response (DDR) causes genomic instability. Genomic instability occurs in Wiskott-Aldrich syndrome (WAS), a primary immunodeficiency disorder, yet the mechanism remains largely uncharacterized. Replication protein A (RPA), a single-strand DNA (ssDNA) binding protein, has key roles in the RSR and DDR.

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed.

Phenotypic and genetic spectrum of ATP6V1A encephalopathy: a disorder of lysosomal homeostasis.

Vacuolar-type H+-ATPase (V-ATPase) is a multimeric complex present in a variety of cellular membranes that acts as an ATP-dependent proton pump and plays a key role in pH homeostasis and intracellular signalling pathways. In humans, 22 autosomal genes encode for a redundant set of subunits allowing the composition of diverse V-ATPase complexes with specific properties and expression. Sixteen subunits have been linked to human disease.