Hemostatic Outcome Definitions in Pediatric Extracorporeal Membrane Oxygenation: Challenges in Cohorts From Rotterdam (2019-2023) and Melbourne (2016-2022).

Objectives: To determine if a priori standardization of outcome hemostatic definitions alone was adequate to enable useful comparison between two cohorts of pediatric extracorporeal membrane oxygenation (ECMO) patients, managed according to local practice and protocol.

Design: Comparison of two separate prospective cohort studies performed at different centers with standardized outcome definitions agreed upon a priori.

Setting: General and cardiac PICUs at the Royal Children's Hospital (RCH) in Melbourne, Australia, and the Sophia Children's Hospital (SCH) in Rotterdam, The Netherlands.

A retrospective review of sildenafil in the Australia and New Zealand Fontan registry: indications and treatment responses.

Introduction: Rates of morbidity and mortality are high in the setting of Fontan physiology and effective medical therapies are not well-established. Clinical trials assessing phosophodiesterase-5-inhibitors, such as sildenafil, have not demonstrated major benefit in patients with a Fontan-type circulation but have only included stable, well-functioning people.

Methods: We sought to retrospectively characterize the people followed by the ANZ Fontan Registry prescribed sildenafil >30 days post Fontan-surgery.

Deriving fontan-specific normative exercise data from well-functioning adolescents.

Background: Exercise capacity is reduced in patients with a Fontan circulation compared to their healthy peers secondary to altered haemodynamics of the Fontan circulation. Cardiopulmonary exercise testing is routinely used in Fontan patients to assess their cardiopulmonary response to exercise and help guide management decisions, however their results are routinely compared to normative data derived from healthy individuals.

Method: Using data available from the Pediatric Heart Network, we performed a retrospective study to derive Fontan-specific normative values in a subgroup of well-functioning adolescent patients.

Correlation of right ventricular outflow tract residual disease on post-op transesophageal echocardiography and discharge transthoracic echocardiography after tetralogy of fallot repair.

Post-operative transesophageal echocardiography (TEE) is standard of care to evaluate for residual disease and quality of surgical repair. The residual lesion score (RLS) as defined by the pediatric heart network explored the impact of residual lesions on outcomes using discharge transthoracic echocardiogram (TTE). In tetralogy of fallot with pulmonary stenosis (TOF/PS), the residual right ventricular outflow tract (RVOT) gradient is one marker of quality of repair.

Connecting transcriptomics with computational modeling to reveal developmental adaptations in pediatric human atrial tissue.

Nearly 1% of babies are born with congenital heart disease-many of whom will require heart surgery within the first few years of life. A detailed understanding of cardiac maturation can help to expand our knowledge on cardiac diseases that develop during gestation, identify age-appropriate drug therapies, and inform clinical care decisions related to surgical repair and postoperative management. Yet, to date, our knowledge of the temporal changes that cardiomyocytes undergo during postnatal development is limited.

Multiple Pediatric Extracorporeal Membrane Oxygenation Runs and Futility. What Are the Limits?

Despite high mortality rates, pediatric extracorporeal membrane oxygenation (ECMO) redeployments are frequently discussed in everyday clinical care. We aim to investigate predictors of mortality in those patients. Clinical data from a single pediatric center were retrospectively analyzed.

The Children's National Hospital Outcomes Registry: a plea for a benchmarking tool that provides longitudinal outcomes for patients and families.

Surgical advancements in paediatric cardiovascular surgery have led to improved survival rates for those patients with the most complex CHDs leading to greater numbers of patients who are living well into adulthood. Despite this new era of long-term survival, our current reporting systems continue to focus largely on using short-term postoperative outcomes as the criteria to both rate and rank hospitals. Using such limited criteria to rate and rank hospitals may mislead the intended audiences: patients and families.

Transcatheter occlusion of venovenous collaterals in the total cavopulmonary Fontan circulation.

Background: Venovenous collaterals are abnormal connections between the systemic and pulmonary venous systems. They are commonly seen in the Fontan circulation and may lead to significant hypoxaemia. Transcatheter closure of venovenous collaterals is a potential but controversial treatment as the long-term benefits and outcomes are not well understood.

Atypical Cardiac Anatomy Leading to Subvalvar Aortic Stenosis in a 19-Month-Old Boy With a Murmur.

A 19-month-old boy presented with a murmur and was found to have an unusual etiology of subvalvar aortic stenosis with discrete subaortic membrane and anomalous attachment of the anterior mitral valve papillary muscle to the interventricular septum. Preoperative suspicion for mitral valve involvement impacted surgical planning.

Efficacy and safety of macitentan in Fontan-palliated patients: 52-week randomized, placebo-controlled RUBATO Phase 3 trial and open-label extension.

Objectives: The efficacy and safety of macitentan, an endothelin receptor antagonist, were assessed in a 52-week, prospective, multicenter, double-blind, randomized, placebo-controlled, parallel-group study assessing the efficacy and safety of macitentan in Fontan-palliated adult and adolescent patients (RUBATO-DB) and an open-label extension trial (RUBATO-OL).

Methods: Patients aged 12 years and older with New York Heart Association functional class II or III underwent total cavopulmonary connection more than 1 year before screening and showed no signs of Fontan failure/clinical deterioration. In RUBATO-DB, the primary efficacy end point was change in peak oxygen consumption from baseline to week 16; secondary end points were change from baseline over 52 weeks in peak oxygen consumption and change in mean count/minute of daily physical activity via accelerometer from baseline to week 16.

Comprehensive Characterization of Surface-Bound Proteins and Measurement of Fibrin Fiber Thickness on Extracorporeal Membrane Oxygenation Circuits Collected From Patients.

Objective: To characterize surface-bound proteins and to measure the thickness of fibrin fibers bound to extracorporeal membrane oxygenation (ECMO) circuits used in children.

Design: Single-center observational prospective study, April to November 2021.

Setting: PICU, Royal Children's Hospital, Melbourne, Australia.

Left Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair.

Background: Whether patients with moderate left atrioventricular valve regurgitation (LAVVR) after surgical repair of complete atrioventricular septal defect (CAVSD) should be observed or undergo reoperation remains unclear.

Methods: Moderate LAVVR was diagnosed in 87 of 220 patients who underwent CAVSD repair: 47 during the initial hospital stay and 40 after a median of 7 months (interquartile range, 2-18 months) after the initial operation.

Results: Of these 87 patients who had moderate LAVVR, 15 died, for an overall mortality of 17%.

Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?

Objectives: The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy.

Methods: We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution.

The Use of the CardioCel 3D 60° Patch for Aortic Arch Reconstruction in Infancy-A Word of Caution.

Background: There are limited data on outcomes after implantation of the CardioCel 3D 60° patch in great vessel repair. After anecdotally witnessing an increase in negative outcomes, we reviewed our experience using this patch in our neonate and infant patients undergoing aortic arch repair.

Methods: Aortic arch repair with implantation of the CardioCel 3D 60° patch was performed in 24 patients between July 2018 and July 2021.

Validating the Concept of Mechanical Circulatory Support with a Rotary Blood Pump in the Inferior Vena Cava in an Ovine Fontan Model.

Right-sided mechanical support of the Fontan circulation by existing devices has been compounded by the cross-sectional design of vena cava anastomosis to both pulmonary arteries. Our purpose was to investigate whether increasing inferior vena cava (IVC) flow with a rotary blood pump in the IVC only in an ovine animal model of Fontan would lead to acceptable superior vena cava (SVC) pressure. To achieve this, a Fontan circulation was established in four female sheep by anastomosing the SVC to the main pulmonary artery (MPA) and by interposing a Dacron graft between the IVC and the MPA.

Immunofluorescence Protocol for Characterization of Platelet and Leukocyte Binding in Extracorporeal Membrane Oxygenation (ECMO) Circuits.

The continuous contact between blood and the foreign surface of the extracorporeal membrane oxygenation (ECMO) circuit contributes to hemostatic, inflammatory, and other physiological disturbances observed during ECMO. Although previous studies have extensively investigated blood samples from patients on ECMO, cell adsorption to the ECMO circuit as an additional factor that could potentially influence clinical outcomes, has largely been overlooked. Here we provide a detailed immunofluorescence (IF) protocol designed to characterize cellular binding on ECMO circuits collected from patients.

Wellbeing in Children and Adolescents with Fontan Physiology.

Objective: To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes.

Study Design: Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures.