Dapagliflozin Use in Children with Advanced Heart Failure Undergoing Heart Transplantation: A Matched Case-Control Study.

Dapagliflozin has been associated with euglycemic ketoacidosis in adults with diabetes contributing to poor outcomes when continued prior to surgery. It is unknown if preoperative use of dapagliflozin may lead to adverse events (AE) in nondiabetic children with advanced heart failure (HF) undergoing heart transplantation (HTx). We performed a single-center, matched case-control analysis of nondiabetic primary pediatric HTx recipients < 21 years-old who underwent HTx and followed through postoperative day (POD) 3.

Prevalence of iron deficiency and anemia in pediatric heart transplant recipients.

Introduction: The prevalence of iron deficiency and anemia in the setting of modern-day maintenance immunosuppression in pediatric heart transplant (HTx) recipients is unclear. The primary aim was to determine the prevalence of iron deficiency (serum ferritin < 30 ng/mL ± transferrin saturation < 20%) and anemia per World Health Organization diagnostic criteria and associated risk factors.

Methods: Single-center, cross-sectional analysis of 200 consecutive pediatric HTx recipients (<21 years old) from 2005 to 2021.

A quantitative assessment of renal function utilizing albuminuria in pediatric heart transplant recipients.

Introduction: Kidney disease is common after pediatric heart transplantation. Serum creatinine-based glomerular filtration rate is the most frequently reported measure of kidney function. Albuminuria is an additional marker of kidney dysfunction and is not well described in this population.

Analysis of Platelet Function Testing in Children Receiving Aspirin for Antiplatelet Effects.

Aspirin (ASA) remains the most common antiplatelet agent used in children. VerifyNow Aspirin Test® (VN) assesses platelet response to ASA, with therapeutic effect defined by the manufacturer as ≤ 549 aspirin reaction units (ARU). Single-center, observational, analysis of 195 children (< 18 years-old) who underwent first VN between 2015 and 2020.

Mycophenolic acid therapeutic drug monitoring using area under the curve in pediatric heart transplant recipients.

Introduction: Pharmacokinetics of mycophenolic acid (MPA) display substantial interpatient variability, with up to 10-fold difference of exposure in individual patients under a fixed-dose regimen. MPA trough level (C0) monitoring is common in clinical practice but has not proven sufficiently informative in predicting MPA exposure or patient outcomes, especially in children. No limited sampling strategies (LSSs) have been generated from pediatric heart transplant (HTx) recipients to estimate MPA AUC.

Successful implementation of telehealth visits in the paediatric heart failure and heart transplant population.

The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and Pediatric Heart Transplant Society (PHTS) convened a working group at the beginning of 2020 during the COVID-19 pandemic, with the aim of using telehealth as an alternative medium to provide quality care to a high-acuity paediatric population receiving advanced cardiac therapies. An algorithm was developed to determine appropriateness, educational handouts were developed for both patients and providers, and post-visit surveys were collected. Telehealth was found to be a viable modality for health care delivery in the paediatric heart failure and transplant population and has promising application in the continuity of follow-up, medication titration, and patient education/counselling domains.

Assessment of the adverse effects of sirolimus versus everolimus in pediatric heart transplant recipients.

Background: Literature is limited comparing adverse effects (AEs) of the proliferation signal inhibitors (PSIs) sirolimus (SRL) and everolimus (EVL) in pediatric heart transplant (HTx) recipients.

Methods: Single-center, observational cohort analysis assessing first use of SRL or EVL in pediatric HTx recipients <21 years of age with up to 2 years follow-up between 2009 and 2020.

Results: Eighty-seven patients were included, with 52 (59.

Treating Pediatric Myocarditis with High Dose Steroids and Immunoglobulin.

There is considerable variability in practice among pediatric centers for treatment of myocarditis. We report outcomes using high dose steroids in conjunction with IVIG. This is a single center retrospective study of children < 21 years of age diagnosed with myocarditis and treated with high dose steroids and IVIG from January 2004-April 2021.

Early Clinical Experience with Dapagliflozin in Children with Heart Failure.

Pediatric heart failure (HF) is associated with significant morbidity and mortality. Medical treatment for pediatric HF is largely derived from adult studies. Previously, there has been no described use of dapagliflozin in pediatric HF patients.

Waitlist and posttransplant outcomes of critically ill infants awaiting heart transplantation managed without ventricular assist device support.

Background: Infants listed for heart transplant are at high risk for waitlist mortality. While waitlist mortality for children has decreased in the current era of increased ventricular assist device use, outcomes for small infants supported by ventricular assist device remain suboptimal. We evaluated morbidity and survival in critically ill infants listed for heart transplant and managed without ventricular assist device support.

Association Between Cytomegalovirus Serostatus, Antiviral Therapy, and Allograft Survival in Pediatric Heart Transplantation.

Cytomegalovirus (CMV) is an important complication of heart transplantation and has been associated with graft loss in adults. The data in pediatric transplantation, however, is limited and conflicting. We conducted a large-scale cohort study to better characterize the relationship between CMV serostatus, CMV antiviral use, and graft survival in pediatric heart transplantation.

The Diagnostic Role of Echocardiographic Strain Analysis in Patients Presenting with Chest Pain and Elevated Troponin: A Multicenter Study.

Background: Myocarditis presenting as acute chest pain with elevated troponins without significant cardiac compromise is rare in previously healthy children, often referred to as myopericarditis. Diagnosis is challenging, as conventional echocardiographic measures of systolic function can be normal. The aim of this study was to demonstrate the diagnostic utility of strain imaging in this scenario.

Human leukocyte antigen eplet mismatching is associated with increased risk of graft loss and rejection after pediatric heart transplant.

Background: While mismatching between donor and recipient human leukocyte antigen (HLA) alleles has been associated with increased graft loss in pediatric heart recipients, it is actually the surface amino acid structures, termed eplets, which determine the antigenicity of each HLA molecule. We hypothesized that HLA eplet mismatch analysis is a better predictor of adverse outcomes after pediatric heart transplant than conventional allele mismatch comparison.

Methods: A retrospective review of the Pediatric Heart Transplant Society database identified pediatric heart recipients (<18 years at listing) with complete donor and recipient HLA typing (A, B, and DR).

Clinical and hemodynamic characteristics of the pediatric failing Fontan.

Aim: To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant.

Methods: In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with "normal" hearts (FNH).

Estimating filling pressures in paediatric heart transplant recipients using echocardiographic parameters and B-type natriuretic peptide.

Background: Longitudinal evaluation of allograft diastolic function in paediatric heart transplant recipients is important for early detection of acute rejection, cardiac allograft vasculopathy, and graft dysfunction. Mean diastolic right atrial and pulmonary capillary wedge pressures obtained at catheterisation are the reference standards for assessment. Echocardiography is non-invasive and more suitable for serial surveillance, but individual parameters have lacked accuracy.

An ethical analysis of obesity as a determinant of pediatric heart transplant candidacy.

Background: Inclusion of BMI as criterion in the determination of heart transplant candidacy in children is a clinical and ethical challenge. Childhood obesity is increasing and children with heart disease are not spared. Currently, many adult heart transplant centers consider class II obesity and higher (BMI > 35 kg/m ) to be a relative contraindication for transplantation due to risk of poor outcome after transplant.

Sleep-disordered breathing in pediatric heart transplant recipients.

Background: Sleep-disordered breathing is commonly found in adults with heart failure both before and after HTx. Untreated sleep-disordered breathing post-transplant has been linked to late graft dysfunction, reduced quality of life, and increased morbidity. Sleep-disordered breathing has not been investigated in pediatric HTx recipients.

Assessment of rejection risk following subtherapeutic calcineurin inhibitor levels after pediatric heart transplantation.

CNIs are the mainstay of immunosuppressive therapy after pediatric HTx. While regular laboratory surveillance is performed to ensure blood levels are within targeted range, the risk of acute rejection associated with subtherapeutic CNI levels has never been quantified. This is a retrospective single-center review of 8413 CNI trough levels in 138 pediatric HTx recipients who survived >1 year after HTx.

A fatal case of bortezomib-induced lung toxicity in a young adult heart transplant recipient.

Bortezomib is approved for the treatment of multiple myeloma but increasingly used in heart transplant (HTx) recipients with antibody-mediated rejection (AMR). Severe pulmonary toxicity is a rare complication in multiple myeloma patients treated with bortezomib, but has not been described in a solid organ transplant recipient. A 20-year-old man 7 years post-HTx presented with acute rejection with hemodynamic compromise.

Management of the sensitized pediatric heart transplant patient.

Despite advancements in transplant immunosuppression and techniques for managing critically ill patients awaiting heart transplantation, children who are immunologically sensitized to human leukocyte antigen remain at increased risk for morbidity and mortality, both while awaiting and after heart transplant. In this review we will discuss the epidemiology of sensitization, review the immunologic basis and methods of human leukocyte antigen antibody detection, describe outcomes for sensitized pediatric transplant candidates, and consider both pre- and post-transplant management options for sensitized patients.

Outcomes of low-intensity biopsy surveillance for rejection in paediatric cardiac transplantation.

Background: Significant inter-centre variability in the intensity of endomyocardial biopsy surveillance for rejection following paediatric cardiac transplantation has been reported. Our aim was to determine if low-intensity biopsy surveillance with two scheduled biopsies in the first year would produce outcomes similar to published registry outcomes.

Methods: A retrospective study of paediatric recipients transplanted between 2008 and 2014 using a low-intensity biopsy protocol consisting of two surveillance biopsies at 3 and 12-13 months in the first post-transplant year, then annually thereafter.

Donor-specific anti-HLA antibody production following pediatric ABO-incompatible heart transplantation.

ABO-i heart transplantation can be performed in infants with end-stage heart failure to increase organ availability. The development of newly detected DSAs is associated with decreased cardiac graft survival, and the effect of ABO-i transplantation on DSA production is unknown. We examined DSA production and rejection frequency in infant recipients of ABO-i and ABO-c heart transplants via a retrospective cohort study of infant heart transplant recipients transplanted at a single pediatric center between January 2004 and November 2014.

Hybrid stage 1 palliation as a bridge to cardiac transplantation in patients with high-risk single ventricle physiology.

Background: The hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) was first described in 1993 as a bridge to heart transplant for HLHS. There are limited data on this strategy as primary heart transplantation for HLHS has become less common.

Methods: This is an observational, single-center study comparing pre- and post-transplant outcomes of patients listed for transplant following hybrid palliation with those following surgical stage 1 palliation.

Diagnostic role of strain imaging in atypical myocarditis by echocardiography and cardiac MRI.

Background: The diagnosis of myocarditis presenting as isolated acute chest pain with elevated troponins but normal systolic function is challenging with significant drawbacks even for the gold-standard endomyocardial biopsy.

Objective: This study aimed to evaluate the diagnostic role of strain imaging by echocardiography and cardiac MRI in these patients.

Materials And Methods: This was a retrospective review of children with cardiac MRI for acute chest pain with elevated troponins compared to normal controls.