Longitudinal Changes in Estimated Glomerular Filtration Rate Following Repeat Heart Transplant in Children and Young Adults.

Background: Chronic kidney disease (CKD) is common after heart transplantation (HT). There are scarce studies looking at longitudinal changes in estimated glomerular filtration rate (eGFR) after repeat HT (RT) and risk factors for the presence of CKD3 post-RT.

Methods: First-time HT recipients (FT) were matched with RT, based on age at transplant, sex, race, and transplant era.

Changes in estimated glomerular filtration rate over the first year following repeat heart transplant in children and young adults.

Background: Renal function is reduced in patients undergoing heart transplant due to hemodynamic compromise, cardiorenal syndrome, and nephrotoxin exposure. No current studies evaluate renal function in retransplants.

Methods: We reviewed all heart transplants at our center from 1995 to 2021 and matched first-time heart transplants with retransplants, based on age at transplant, sex, and race.

An Analysis of 186 Transplants for Pediatric or Congenital Heart Disease: Impact of Pretransplant VAD.

Background: We reviewed our management strategy and outcome data for all 181 patients with pediatric or congenital heart disease who received 186 heart transplants from January 1, 2011, to March 1, 2022, and evaluated the impact of pretransplant ventricular assist device (VAD).

Methods: Continuous variables are presented as mean (SD); median [interquartile range] (range). Categorical variables are presented as number (percentage).

Heart Transplantation in Patients Less Than 18 Years of Age: Comparison of 2 Eras Over 36 Years and 323 Transplants at a Single Institution.

Background: We reviewed our management strategy and outcome data for all 311 patients less than 18 years of age who underwent 323 heart transplants at our institution (1986 to 2022) in order to assess changes in patterns of practice and outcomes over time and to compare two consecutive eras: era 1 (154 heart transplants [1986 to 2010]) and era 2 (169 heart transplants [2011 to 2022]).

Study Design: Descriptive comparisons between the two eras were performed at the level of the heart transplant for all 323 transplants. Kaplan-Meier survival analyses were performed at the level of the patient for all 311 patients, and log-rank tests were used to compare groups.

Assessment of parental decision making in congenital heart disease, cardiomyopathy and heart transplantation: an observational study analysing decisional characteristics and preferences.

Objective: We explore shared decision making (DM) in guardians of children with heart disease by assessing the desired weight of influence on DM and factors that may alter the relative weight of parent or medical team influence.

Methods: Guardians of patients <21 years and admitted >1 week in the paediatric cardiac intensive care unit (PCICU) were recruited. Twelve vignettes were designed including technical (antibiotic selection, intubation, peripherally inserted central catheter placement, ventricular assist device placement, heart transplant, organ rejection, heart rhythm abnormalities and resuscitation effort) and non-technical vignettes (cessation of life-sustaining therapies, depression treatment, obesity and palliative care referral).

An Analysis of 183 Heart Transplants for Pediatric or Congenital Heart Disease-Impact of High Panel Reactive Antibody.

Background: We reviewed our management strategy and outcome data for all 179 patients with pediatric and/or congenital heart disease who underwent 183 heart transplants from January 1, 2011, to December 31, 2021, and evaluated the impact of elevated panel reactive antibody (PRA).

Methods: High PRA was defined as PRA >10%. Univariate associations with long-term survival were assessed with Cox proportional hazards models.

Palliation Plus Ventricular Assist Device Insertion in 15 Neonates and Infants With Functionally Univentricular Circulation.

Background: We report 15 high-risk neonates and infants with functionally univentricular circulation stabilized with initial surgical palliation plus ventricular assist device (VAD) insertion (PALLIATION+VAD) in preparation for transplantation.

Methods: Fifteen functionally univentricular patients with ductal-dependent systemic circulation (8 hypoplastic left heart syndrome, 1 hypoplastic left heart syndrome-related malformation: 7 neonates, 2 infants) or ductal-dependent pulmonary circulation (6 hypoplastic right heart syndrome: 5 neonates, 1 infant) presented with anatomical and/or physiological features associated with increased risk for conventional univentricular palliation (large coronary sinusoids with ventricular-dependent coronary circulation, severe systemic atrioventricular valvar regurgitation, cardiogenic shock, or restrictive atrial septum). PALLIATION+VAD for patients with ductal-dependent systemic circulation was: VAD insertion plus application of bilateral pulmonary bands, stent placement in the arterial duct, and atrial septectomy, if needed.

Risk factors associated with post-transplant superior caval vein stenosis in paediatric heart transplantation.

Superior caval vein stenosis is a known complication following paediatric heart transplantation. Herein, we sought to assess the incidence of superior caval vein stenosis and need for intervention in a single centre paediatric heart transplantation programme. A retrospective review was performed to identify variables associated with superior caval vein stenosis and need for intervention.

Family functioning in pediatric heart transplantation: Variables associated with poor outcomes.

Family functioning is integral in a child's life and is linked to quality of life in health as well as disease. This has been scarcely studied in pediatric orthotopic heart transplantation (pOHT). In this study, we evaluate demographic and clinical factors associated with family functioning in this patient population.

Postoperative vasoactive inotropic score is predictive of outcomes in pediatric heart transplantation.

Vasoactive inotrope score (VIS) is scarcely studied in pediatric orthotopic heart transplantation (pOHT). We conducted a retrospective review of pOHT (<21 years) recipients. Max VIS and mean VIS were calculated at 0-24 and 24-48 hours post-pOHT.

Thenar Muscle Oxygen Saturation Using Vascular Occlusion Test: A Novel Technique to Study Microcirculatory Abnormalities in Pediatric Heart Failure Patients.

Heart failure (HF) is associated with microcirculatory changes secondary to neuro-humoral imbalance, vascular stiffness and increased sympathetic tone. Near Infra-Red Spectroscopy (NIRS) derived Thenar muscle tissue oxygenation levels (StO) can provide an estimate of the functional status of microcirculation. There is a paucity of literature regarding evaluation of microcirculation in pediatric subjects with HF.

Anti-LFA-1 induces CD8 T-cell dependent allograft tolerance and augments suppressor phenotype CD8 cells.

The induction of tolerance to transplanted organs is a major objective in transplantation immunology research. Lymphocyte function-associated antigen-1 (LFA-1) interactions have been identified as a key component of the T-cell activation process that may be interrupted to lead to allograft tolerance. In mice, αLFA-1 mAb is a potent monotherapy that leads to the induction of donor-specific transferable tolerance.

Hypoplastic left heart in Turner's syndrome: a primary indication for transplant?

Survival for hypoplastic left heart syndrome patients following the Norwood procedure is 71-90%. Mortality in patients with Turner's syndrome and hypoplastic left heart syndrome after conventional palliation (Norwood operation) has been reported as high as 80%. This questions the approach of traditional staged palliation.

End-organ recovery post-ventricular assist device can prognosticate survival.

Background: This study examines our institutional ventricular assist devices (VADs) experience over two decades to understand trends towards predictors of mortality.

Methods: Retrospective study of patients aged 0-21years supported with a VAD from January 1996 to May 2015. Patient data was examined pre and post-VAD implant among survivors and non-survivors.

Ectopic expression of Fas Ligand on cardiomyocytes renders cardiac allografts resistant to CD4(+) T-cell mediated rejection.

Fas Ligand limits inflammatory injury and permits allograft survival by inducing apoptosis of Fas-bearing lymphocytes. Previous studies have shown that the CD4(+) T-cell is both sufficient and required for murine cardiac allograft rejection. Here, utilizing a transgenic mouse that over-expresses Fas Ligand specifically on cardiomyocytes as heart donors, we sought to determine if Fas Ligand on graft parenchymal cells could resist CD4(+) T-cell mediated rejection.

Steroid avoidance in pediatric heart transplantation results in excellent graft survival.

Background: Maintenance steroid (MS) use in pediatric heart transplantation (HT) varies across centers. The purpose of this study was to evaluate the impact of steroid-free maintenance immunosuppression (SF) on graft outcomes in pediatric HT.

Methods: Patients younger than 18 years in the United States undergoing a first HT during 1990 to 2010 were analyzed for conditional 30-day graft loss (death or repeat HT) and death based on MS use by multivariable analysis.

The hemodynamic response to dexmedetomidine loading dose in children with and without pulmonary hypertension.

Background: Dexmedetomidine, an α-2 receptor agonist, is widely used in children with cardiac disease. Significant hemodynamic responses, including systemic and pulmonary vasoconstriction, have been reported after dexmedetomidine administration. Our primary goal of this prospective, observational study was to quantify the effects of dexmedetomidine initial loading doses on mean pulmonary artery pressure (PAP) in children with and without pulmonary hypertension.

Early predictors of survival to and after heart transplantation in children with dilated cardiomyopathy.

Background: The importance of clinical presentation and pretransplantation course on outcome in children with dilated cardiomyopathy listed for heart transplantation is not well defined.

Methods And Results: The impact of age, duration of illness, sex, race, ventricular geometry, and diagnosis of myocarditis on outcome in 261 children with dilated cardiomyopathy enrolled in the Pediatric Cardiomyopathy Registry and Pediatric Heart Transplant Study was studied. End points included listing as United Network for Organ Sharing status 1, death while waiting, and death after transplantation.

Graft survival is better without prior surgery in cardiac transplantation for functionally univentricular hearts.

Background: The effect of surgical history on graft outcomes in patients with functionally univentricular hearts (UH) is not well understood. We compared graft outcomes after heart transplantation in children with a UH between patients who received allografts without prior cardiac surgery (Group A) and patients who underwent transplantation after prior cardiac surgery (Group B).

Methods: We reviewed all patients who received allografts for UH at our institution from 1990 to 2009.

CD4 T cells mediate cardiac xenograft rejection via host MHC Class II.

Background: Previous studies have shown that acute CD4 T-cell-mediated cardiac allograft rejection requires donor major histocompatibility complex (MHC) Class II expression and can be independent of "indirect" antigen presentation. However, other studies suggested that indirect antigen presentation to CD4 T cells may play a primary role in cellular xenograft immunity. Thus, the relative roles of direct/indirect CD4 T cell reactivity against cardiac xenografts are unclear.

Pediatric cardiac retransplant: differing patterns of primary graft failure by age at first transplant.

Objective: This study compared graft failure leading to retransplant in infants versus older children at initial heart transplant.

Methods: Twenty-six retransplant recipients were compared by age at first transplant: infant group (<1 year) and pediatric group (≥1 year).

Results: Early retransplant survival was 92%.

Outcome of acute graft rejection associated with hemodynamic compromise in pediatric heart transplant recipients.

We sought to analyze the outcome of hemodynamically significant acute graft rejection in pediatric heart transplant recipients from a single-center experience. Acute graft rejection remains a major cause of morbidity and mortality for patients who undergo orthotopic heart transplantation and has been associated with the severity of the rejection episode. A retrospective review of all children experiencing a hemodynamically significant rejection episode after orthotopic heart transplantation was performed.