Revisiting the Effect of Udenafil on Exercise Performance in Patients With Fontan Circulation: Results of a Post Hoc Analysis of the FUEL Trial.

Background: In the FUEL (Fontan Udenafil Exercise Longitudinal) trial, a positive treatment effect was identified for outcomes at the ventilatory anaerobic threshold but not for the primary outcome, oxygen consumption (Vo) at peak exercise. This disparate response may be explained by the physiologic challenge of improving peak Vo in participants with near-normal baseline exercise performance.

Methods: Participants were divided into subgroups by baseline predicted peak Vo (<80% versus ≥80%).

Methylprednisolone for Infant Heart Surgery: Subpopulation Analyses of a Randomized Controlled Trial.

Objectives: Evaluate benefits and harms of prophylactic intraoperative methylprednisolone in subpopulations undergoing infant heart surgery.

Design: Subpopulation analyses of The Steroids to Reduce Systemic Inflammation after Infant Heart Surgery (STRESS) trial, a double-blind randomized placebo-controlled trial.

Setting: Twenty-four congenital heart centers.

Reexploring the STRESS Trial: Subgroup Postoperative Outcomes Following Methylprednisolone for Infant Heart Surgery.

Unlabelled: Objective Assess the association between intraoperative methylprednisolone and specific postoperative outcomes among subgroups undergoing infant heart surgery.

Design: Subpopulation analyses of The Steroids to Reduce Systemic Inflammation after Infant Heart Surgery trial, a double-blind randomized placebo-controlled trial.

Setting: 24 congenital heart centers.

A Bayesian Re-analysis of the Steroids to Reduce Systemic Inflammation after Infant Heart Surgery (STRESS) Trial.

Background: Prophylactic steroids are often used to reduce the systemic inflammatory response to cardiopulmonary bypass in infants undergoing heart surgery. The STRESS trial found that the likelihood of a worse outcome did not differ between infants randomized to methylprednisolone (n=599) versus placebo (n=601) in a risk-adjusted primary analysis (adjusted odds ratio [OR], 0.86; 95% CI, 0.

National practice patterns for the use of regional anesthesia for pediatric cardiac surgery: An analysis of the Society of Thoracic Surgeons congenital heart surgery database.

Background: Complications associated with suboptimal pain management after pediatric cardiac surgery have increased interest in regional anesthesia (RA). We sought to evaluate national trends and explore the association of RA with postoperative outcomes following pediatric cardiac surgery.

Methods: Patients <18 years in the Society of Thoracic Surgeons Congenital Cardiac Anesthesia Society Database from 01/2016-05/2023 were analyzed.

Urine biomarkers, acute kidney injury, and fluid overload in neonatal cardiac surgery.

Background: Cardiac surgery-associated acute kidney injury (CS-AKI) and fluid overload (FO) are common among neonates who undergo cardiopulmonary bypass, and increase mortality risk. Current diagnostic criteria may delay diagnosis. Thus, there is a need to identify urine biomarkers that permit earlier and more accurate diagnosis.

Additive value of invasive haemodynamic assessment for predicting post-operative outcomes after Fontan.

Routine pre-Fontan cardiac catheterization remains standard practice at most centres. However, with advances in non-invasive risk assessment, an invasive haemodynamic assessment may not be necessary for all patients.Using retrospective data from patients undergoing Fontan palliation at our institution, we developed a multivariable model to predict the likelihood of a composite adverse post-operative outcome including prolonged length of stay ≥ 30 days, hospital readmission within 6 months, and death and/or transplant within 6 months.

Preoperative Hemodynamics Impact the Benefit of Fenestration on Fontan Postoperative Length of Stay.

Background: Utilization of Fontan fenestration varies considerably by center.

Objectives: Using a multicenter Pediatric Heart Network dataset linking surgical and preoperative hemodynamic variables, the authors evaluated factors associated with use of Fontan fenestration and the impact of fenestration on post-Fontan length of stay (LOS).

Methods: Patients 2 to 6 years old at Fontan surgery from 2010 to 2020 with catheterization<1 year prior were included.

Surgical Modulation of Pulmonary Artery Shear Stress: A Patient-Specific CFD Analysis of the Norwood Procedure.

Purposr: This study created 3D CFD models of the Norwood procedure for hypoplastic left heart syndrome (HLHS) using standard angiography and echocardiogram data to investigate the impact of shunt characteristics on pulmonary artery (PA) hemodynamics. Leveraging routine clinical data offers advantages such as availability and cost-effectiveness without subjecting patients to additional invasive procedures.

Methods: Patient-specific geometries of the intrathoracic arteries of two Norwood patients were generated from biplane cineangiograms.

Evaluating registry-based trial economics: Results from the STRESS clinical trial.

Background: Registry-based trials have the potential to reduce randomized clinical trial (RCT) costs. However, observed cost differences also may be achieved through pragmatic trial designs. A systematic comparison of trial costs across different designs has not been previously performed.

Population Pharmacokinetics of Caffeine in Neonates with Congenital Heart Disease and Associations with Acute Kidney Injury.

Cardiac surgery-associated acute kidney injury (CS-AKI) occurs in approximately 65% of neonates undergoing cardiac surgery on cardiopulmonary bypass and contributes to morbidity and mortality. Caffeine may reduce CS-AKI by counteracting adenosine receptor upregulation after bypass, but pharmacokinetics (PK) in this population are unknown. The goal of our analysis is to address knowledge gaps in age-, disease-, and bypass-related effects on caffeine disposition and explore preliminary associations between caffeine exposure and CS-AKI using population PK modeling techniques and an opportunistic, electronic health record-integrated trial design.

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study.

Objective: In the SVR trial (Single Ventricle Reconstruction), newborns with hypoplastic left heart syndrome were randomly assigned to receive a modified Blalock-Taussig-Thomas shunt (mBTTS) or a right ventricle-to-pulmonary artery shunt (RVPAS) at Norwood operation. Transplant-free survival was superior in the RVPAS group at 1 year, but no longer differed by treatment group at 6 years; both treatment groups had accumulated important morbidities. In the third follow-up of this cohort (SVRIII [Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type]), we measured longitudinal outcomes and their risk factors through 12 years of age.

Improving Outcomes for Infants After Cardiopulmonary Bypass Surgery for Congenital Heart Disease: A Commentary on Recent Randomized Controlled Trials.

The recent NITRIC and STRESS trials demonstrate opportunities to perform pragmatic large randomized trials in congenital heart disease. We discuss lessons learnt from these trials which can inform future trial design and conduct in the field of pediatric heart surgery.

Center-level factors associated with shorter length of stay following stage 1 palliation: An analysis of the national pediatric cardiology quality improvement collaborative registry.

Background: Stage 1 single ventricle palliation (S1P) has the longest length of stay (LOS) of all benchmark congenital heart operations. Center-level factors contributing to prolonged hospitalization are poorly defined.

Methods: We analyzed data from infants status post S1P included in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry.

Outcomes of the Kawashima: A Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis.

Background: We aimed to evaluate the effect of age at operation on postoperative outcomes in children undergoing a Kawashima operation.

Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for Kawashima procedures from January 1, 2014, to June 30, 2020. Patients were stratified by age at operation in months: 0 to <4, 4 to <8, 8 to <12, and >12.

A point-of-care pharmacokinetic/pharmacodynamic trial in critically ill children: Study design and feasibility.

Background: High-quality, efficient, pharmacokinetic (PK), pharmacodynamic (PD), and safety studies in children are needed. Point-of-care trials in adults have facilitated clinical trial participation for patients and providers, minimized the disruption of clinical workflow, and capitalized on routine data collection. The feasibility and value of point-of-care trials to study PK/PD in children are unknown, but appear promising.

Using Pharmacokinetic Modeling and Electronic Health Record Data to Predict Clinical and Safety Outcomes after Methylprednisolone Exposure during Cardiopulmonary Bypass in Neonates.

Background: Infants undergoing cardiac surgery with cardiopulmonary bypass (CPB) frequently receive intraoperative methylprednisolone (MP) to suppress CPB-related inflammation; however, the optimal dosing strategy and efficacy of MP remain unclear.

Methods: We retrospectively analyzed all infants under 90 days-old who received intra-operative MP for cardiac surgery with CPB from 2014-2017 at our institution. We combined real-world dosing data from the electronic health record (EHR) and two previously developed population pharmacokinetic/pharmacodynamic models to simulate peak concentration (Cmax) and area under the concentration-time curve for 24 h (AUC24) for MP and the inflammatory cytokines interleukin-6 (IL-6) and interleukin-10 (IL-10).

The Fontan Udenafil Exercise Longitudinal Trial: Subgroup Analysis.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.

Contemporary Patterns of Care in Tetralogy of Fallot: Analysis of The Society of Thoracic Surgeons Data.

Background: Against the background of earlier studies, recent patterns in surgical management of tetralogy of Fallot (TOF) were assessed.

Methods: A retrospective review of The Society of Thoracic Surgeons (STS) Congenital Database (2010-2020) was performed on patients aged <18 years with TOF or pulmonary stenosis and primary procedure TOF surgical repair or palliation. Procedural frequencies were examined by epoch.

Association of Urine Biomarkers With Acute Kidney Injury and Fluid Overload in Infants After Cardiac Surgery: A Single Center Ancillary Cohort of the Steroids to Reduce Systemic Inflammation After Infant Heart Surgery Trial.

Unlabelled: To examine the association between three perioperative urine biomarker concentrations (urine cystatin C [uCysC], urine neutrophil gelatinase-associated lipocalin [uNGAL], and urine kidney injury molecule 1 [uKIM-1]), and cardiac surgery-associated acute kidney injury (CS-AKI) and fluid overload (FO) in infants with congenital heart disease undergoing surgery on cardiopulmonary bypass. To explore how urine biomarkers are associated with distinct CS-AKI phenotypes based on FO status.

Design: Ancillary prospective cohort study.

Single Ventricle Reconstruction III: Brain Connectome and Neurodevelopmental Outcomes: Design, Recruitment, and Technical Challenges of a Multicenter, Observational Neuroimaging Study.

Unlabelled: Patients with hypoplastic left heart syndrome who have been palliated with the Fontan procedure are at risk for adverse neurodevelopmental outcomes, lower quality of life, and reduced employability. We describe the methods (including quality assurance and quality control protocols) and challenges of a multi-center observational ancillary study, SVRIII (Single Ventricle Reconstruction Trial) Brain Connectome. Our original goal was to obtain advanced neuroimaging (Diffusion Tensor Imaging and Resting-BOLD) in 140 SVR III participants and 100 healthy controls for brain connectome analyses.

Prenatal Diagnosis, Management, and Treatment of Fetal Cardiac Disease.

Prenatal diagnosis of congenital heart disease makes it possible to optimize and coordinate care of the fetus and pregnant person. Benefits encompass the full spectrum of pre- and perinatal care, from counseling to optimization of pregnancy care and fetal intervention. Prenatal diagnosis reduces the likelihood of postnatal hemodynamic compromise and improves long-term neurodevelopmental outcomes.