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.

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.

Coronary Artery Aneurysm Rupture in Kawasaki Disease and SARS-CoV-2 Infection.

• CAA rupture is a rare, life-threatening complication of KD. • SARS-CoV-2 and KD may have a synergistic effect resulting in severe inflammation. • Recognizing specific echocardiogram findings is necessary to diagnosis CAA rupture.

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.

Hybrid approach to pulmonary vein stenting after Fontan palliation.

Pulmonary vein stenosis is poorly tolerated in patients who have undergone Fontan palliation and typically requires surgical or transcatheter intervention. Percutaneous transcatheter approaches to intervention can be technically difficult due to challenging anatomy. A hybrid per-atrial transcatheter approach for stenting pulmonary veins provides a direct approach to the pulmonary veins and has the potential to improve safety and efficacy of this complex intervention.

Update on Transcatheter Device Closure of Congenital Septal Defects.

Purpose Of Review: The goal of this paper is to review currently available devices for closure of atrial septal defects (ASDs) and ventricular septal defects (VSDs).

Recent Findings: Favorable results from the ASSURED trial resulted in FDA approval for the most recently developed device for transcatheter ASD closure in the United States. Further studies are required to assist in the development or approval of safe devices for transcatheter perimembranous VSD closure in pediatric patients.

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.

Whole-Chest Three-Dimensional Modeling Aids Hybrid Pulmonary Valve Replacement Following Double Switch Operation.

The double switch operation for congenitally corrected transposition of the great arteries (CC-TGA) has been associated with high rates of reintervention, including the need for pulmonary valve replacement. Hybrid interventional approaches can avoid bypass when complex anatomy complicates traditional catheter-based approaches. We present a case of successful transcatheter pulmonary valve replacement via hybrid per-ventricular approach with pre-procedural planning aided by 3D segmentation of skeletal and cardiac anatomy in a patient with surgically corrected CC-GTA.

Electrocardiogram Before Tricyclic Antidepressant Use: Minimal Impact in Pediatric Functional Gastrointestinal Disorders.

Objectives: The aim of this study was to determine the effect of electrocardiogram (ECG) findings on the initiation of tricyclic antidepressants (TCAs) for functional gastrointestinal disorders (FGIDs) and to evaluate cardiac outcomes related to low dose TCA use.

Methods: We performed a retrospective chart review of all pediatric outpatients at a tertiary pediatric hospital with an ECG ordered by a pediatric gastroenterologist when considering initiation of a TCA between January 2011 and February 2018. We collected demographics, previous cardiovascular testing results, TCA dosing, and pertinent outcomes, including cardiology referrals, emergency department, and hospital admissions, and death during the study period.

Postoperative Renal Failure, Shunt Type, and Mortality After Norwood Palliation.

Background: The Single Ventricle Reconstruction Trial demonstrated increased risk of death or heart transplant 1 year after Norwood among subjects randomly assigned to modified Blalock-Taussig shunts compared with right ventricle to pulmonary artery (RV-PA) shunts. We used the Single Ventricle Reconstruction Trial public use database to evaluate incidence and risk factors for postoperative renal failure and relationships between renal failure, shunt type, and outcomes after Norwood.

Methods: Postoperative renal failure was defined a priori as a threefold rise in creatinine from baseline, or dialysis use, within 7 days of Norwood.

Transition to Ductal Stenting for Single Ventricle Patients Led to Improved Survival: An Institutional Case Series.

Background: The use of systemic-to-pulmonary shunts (SPS) in neonates with single ventricle heart defects and ductal-dependent pulmonary blood flow (ddPBF) was historically associated with high morbidity and mortality at our center. As a result, we transitioned to the preferential use of ductus arteriosus stents (DS) when feasible. This report describes our initial results with this strategy.

Outcomes of Operator-Directed Sedation and Anesthesiologist Care in the Pediatric/Congenital Catheterization Laboratory: A Study Utilizing Data From the IMPACT Registry.

Objectives: The objective of this study was to assess contemporary use of operator directed sedation (ODS) and anesthesiologist care (AC) in the pediatric/congenital cardiac catheterization laboratory (PCCL), specifically evaluating whether the use of operator-directed sedation was associated with increased risk of major adverse events.

Background: The safety of ODS relative to AC during PCCL procedures has been questioned.

Methods: A multicenter, retrospective cohort study was performed studying procedures habitually performed with ODS or AC at IMPACT (Improving Adult and Congenital Treatment) registry hospitals using ODS for ≥5% of cases.

Mustard Baffle Revision With Systemic Ventricular Assist Device Placement.

Systemic right ventricle failure often develops in patients with dextro-transposition of the great arteries who have undergone atrial-level switch procedures. An adult with dextro-transposition of the great arteries with Mustard repair in childhood presented with end-stage heart failure due to systemic right ventricle failure, Mustard baffle leak, and unrepaired partial anomalous pulmonary venous return. She underwent device closure of the baffle leak by catheterization, followed by baffle revision and concomitant systemic ventricular assist device placement by adult and pediatric heart surgeons.

Preprocedural three-dimensional planning aids in transcatheter ductal stent placement: A single-center experience.

Objectives: Describe the use of three-dimensional (3D) patent ductus arteriosus (PDA) modeling to better define ductal anatomy to improve preprocedural planning for ductal stent placement.

Background: Ductal stenting is an alternative to surgical shunting in patients with ductal dependent pulmonary blood flow. Ductal anatomy is often complex with extreme tortuosity and risk of pulmonary artery isolation, thus increasing procedural risks.

Estimating radiation exposure during paediatric cardiac catheterisation: a potential for radiation reduction with air gap technique.

Introduction: The air gap technique (AGT) is an approach to radiation dose optimisation during fluoroscopy where an "air gap" is used in place of an anti-scatter grid to reduce scatter irradiation. The AGT is effective in adults but remains largely untested in children. Effects are expected to vary depending on patient size and the amount of scatter irradiation produced.

Postoperative Transcatheter Interventions in Children Undergoing Congenital Heart Surgery.

Background Postoperative transcatheter interventions (TCIs) are performed after congenital heart surgery to treat residual or recurrent anatomic lesions. We used the Society of Thoracic Surgeons Congenital Heart Surgery Database to evaluate rates of postoperative TCIs, center variability, and to determine whether center approaches to postoperative TCI might be associated with outcomes. Methods and Results Patients <18 years undergoing an index operation (2010-2016) were included.

Palliating Premature Infants With Obstructed Total Anomalous Pulmonary Venous Connection via Catheterization.

Obstructed total anomalous pulmonary venous connection (TAPVC) involves anomalous drainage of all pulmonary veins to a location other than the left atrium and typically causes significant respiratory symptoms requiring urgent surgical repair. Premature infants born with obstructed TAPVC are often not considered to be suitable surgical candidates due to their size and therefore pose a significant treatment dilemma. Catheter-based interventions provide a temporizing option for infants with obstructed TAPVC, allowing for hemodynamic stabilization and growth until surgical intervention is feasible.

The Use of Echocardiography for Pediatric Patients Presenting with Syncope.

Objectives: To assess the frequency, yield, and cost of echocardiograms meeting "rarely appropriate" criteria.

Study Design: Retrospective, single-center study of pediatric patients presenting with syncope. Patients were categorized according to the appropriate use criteria and based upon location of care (emergency department only, primary care setting only, or referred to a pediatric cardiologist).

Evaluating Appropriate Use of Pediatric Echocardiograms for Chest Pain in Outpatient Clinics.

Background: Echocardiography is often used in the evaluation of pediatric chest pain, but the incidence of cardiac pathology is low. In 2014, the American College of Cardiology published appropriate use criteria (AUC) for echocardiography including recommendations for pediatric chest pain. We evaluated the frequency and diagnostic yield of echocardiograms performed for each AUC indication and cost associated with echocardiograms performed for indications meeting the "rarely appropriate" criteria.

Novel Rod Domain Duplication in Dystrophin Resulting in X-Linked Dilated Cardiomyopathy.

Background: X-linked dilated cardiomyopathy is a rare, cardio-specific form of dystrophinopathy allelic to Duchenne and Becker muscular dystrophy that results in heart failure without skeletal muscle weakness.

Patient Description: We describe a previously healthy 16-year-old boy who presented with palpitations progressing to heart failure who was ultimately found to have a novel duplication of exons 13-16 in the dystrophin gene resulting in diagnosis of X-linked dilated cardiomyopathy.

Results: The patient was diagnosed with X-linked dilated cardiomyopathy through clinical diagnosis and genetic testing.