The CHD severity classification system: development of a tool to assist with disease stratification for CHD research.

Background: Complexity stratification for CHD is an integral part of clinical research due to its heterogenous clinical presentation and outcomes. To support our ongoing research efforts into CHD requiring disease severity stratifications, a simplified CHD severity classification system was developed and verified, with potential utility for clinical researchers without specialist CHD knowledge or access to clinical/medical records.

Method: A two-tiered analysis approach was undertaken.

The Kids Heart BioBank: supporting 20 years of patient care and research into CHD.

Introduction: The cause of most CHD is unknown and considered complex, implicating genetic and environmental factors in disease causation. The Kids Heart BioBank was established in 2003 to accelerate genetic investigations into CHD.

Methods: Recruitment includes patients undergoing interventions for CHD at The Children's Hospital at Westmead.

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.

Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery.

Using novel data linkage of congenital heart disease biobank data with administrative health data to identify cardiovascular outcomes to inform genomic analysis.

Introduction: Contemporary care of congenital heart disease (CHD) is largely standardised, however there is heterogeneity in post-surgical outcomes that may be explained by genetic variation. Data linkage between a CHD biobank and routinely collected administrative datasets is a novel method to identify outcomes to explore the impact of genetic variation.

Objective: Use data linkage to identify and validate patient outcomes following surgical treatment for CHD.

Molecular-guided therapy for the treatment of patients with relapsed and refractory childhood cancers: a Beat Childhood Cancer Research Consortium trial.

Background: Children with relapsed central nervous system (CNS tumors), neuroblastoma, sarcomas, and other rare solid tumors face poor outcomes. This prospective clinical trial examined the feasibility of combining genomic and transcriptomic profiling of tumor samples with a molecular tumor board (MTB) approach to make real‑time treatment decisions for children with relapsed/refractory solid tumors.

Methods: Subjects were divided into three strata: stratum 1-relapsed/refractory neuroblastoma; stratum 2-relapsed/refractory CNS tumors; and stratum 3-relapsed/refractory rare solid tumors.

Mother-Infant Dyadic Synchrony and Interaction Patterns After Infant Cardiac Surgery.

Objective: Parents and their infants with complex congenital heart disease (CHD) face relational challenges, including marked distress, early separations, and infant hospitalizations and medical procedures, yet the prevalence of parent-infant interaction difficulties remains unclear. Using a standardized observational paradigm, this study investigated mother-infant dyadic synchrony, interactional patterns, and associated predictors in mother-infant pairs affected by CHD, compared with typically-developing pairs.

Methods: In this prospective, longitudinal cohort study, mothers and their infants requiring cardiac surgery before age 6-months (n=110 pairs) and an age- and sex-matched Australian community sample (n=85 pairs) participated in a filmed, free-play interaction at 6.

Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries.

Recommendations for developing effective and safe paediatric and congenital heart disease services in low-income and middle-income countries: a public health framework.

The global of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs.

Australian paediatric health professionals' experiences during the COVID-19 pandemic.

Background: The COVID-19 pandemic has significantly increased stress and strain on health professionals. With a focus on paediatric cardiac care, this study explored health professionals' concerns about COVID-19, perceptions of the impact of pandemic on healthcare, and experiences of psychological stress.

Methods: Paediatric health professionals working at a large quaternary hospital in Australia were invited to complete a survey between June 2020 and February 2021.

The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings.

Introduction: Advances in the care of patients with single-ventricle congenital heart disease have led to a new generation of individuals living with a Fontan circulation. For people with Fontan physiology, physical, psychological and neurodevelopmental challenges are common. The objective of this study is to describe and develop a deeper understanding of the factors that contribute to quality of life (QOL) among children, adolescents and adults living with a Fontan circulation across Australia and New Zealand, their parents and siblings.

Insights into the genetic architecture underlying complex, critical congenital heart disease.

Congenital heart disease (CHD) has a multifactorial aetiology, raising the possibility of an underlying genetic burden, predisposing to disease but also variable expression, including variation in disease severity, and incomplete penetrance. Using whole genome sequencing (WGS), the findings of this study, indicate that complex, critical CHD is distinct from other types of disease due to increased genetic burden in common variation, specifically among established CHD genes. Additionally, these findings highlight associations with regulatory genes and environmental "stressors" in the final presentation of disease.

Fetal heart block: Vaginal delivery an option.

Background: Fetal heart block (FHB) occurs in approximately 1:20 000 births and is associated with significant morbidity and mortality. Vaginal delivery with intrapartum fetal scalp lactate monitoring is offered as an option at our centre for selected cases, in contrast with the published literature advocating caesarean birth.

Aims: To review perinatal outcomes at delivery for FHB at a tertiary referral hospital.

Development of Clinical-Grade Antibodies against Tumor-Specific Mutations to Target Neuroblastoma.

Objective: Tumor heterogeneity is a fundamental problem in treating cancer with monotargeting therapy, including chemical, antibody, and T cell therapies. Our goal is to target multiple mutated peptides found in a patient's cancer to increase antibody therapy effectiveness.

Methods: Tumor samples were derived from patients with neuroblastoma.

Identification of Let-7 miRNA Activity as a Prognostic Biomarker of SHH Medulloblastoma.

Medulloblastoma (MB) is the most common pediatric embryonal brain tumor. The current consensus classifies MB into four molecular subgroups: sonic hedgehog-activated (SHH), wingless-activated (WNT), Group 3, and Group 4. MYCN and let-7 play a critical role in MB.

Rapidly Enlarging Aortic Root Pseudoaneurysm in a Child With Endocarditis and Repaired Congenital Heart Disease.

A child with repaired double outlet right ventricle presented with bacteremia. Despite unsuspecting echocardiography on admission and clinical improvement on antibiotics, repeat routine echocardiography detected an aortic pseudoaneurysm, requiring a Ross-Konno operation. In repaired congenital heart defects with bacteremia, close echocardiographic surveillance is required to detect aortic pseudoaneurysm.

The Ross/Ross-Konno procedure in infancy is a safe and durable solution for aortic stenosis.

Objective: The objective of this study was to characterize early and midterm outcomes after the Ross/Ross-Konno procedure performed in infancy for severe aortic valve disease.

Methods: Between January 1995 and December 2018, 35 infants younger than 1 year (13 neonates) underwent a Ross/Ross-Konno procedure. Patients were followed up to a median of 4.

A new era of genetic testing in congenital heart disease: A review.

Genetic and genomic testing in pediatric CHD is becoming increasingly routine, and can have important psychosocial, clinical and reproductive implications. In this paper we highlight important challenges and considerations when providing genetics consults and testing in pediatric CHD and illustrate the role of a dedicated CHD genetics clinic. Key lessons include that a) a genetic diagnosis can have clinical utility that justifies testing early in life, b) adequate genetic counselling is crucial to ensure families are supported, understand the range of possible results, and are prepared for new or unexpected health information, and c) further integration of the clinical genetics and cardiology workflows will be required to effectively manage the burgeoning information arising from genetic testing.

Outcomes Following Heterotopic Placement of Right Ventricle to Pulmonary Artery Conduits.

Background: We sought to evaluate the outcomes following right ventricle to pulmonary artery (RV-PA) conduit placement in pediatric patients, excluding those with a RV-PA conduit for the Ross procedure which is associated with improved conduit durability, partly related to its orthotopic position.

Methods: Outcomes for 119 patients who underwent RV-PA conduit placement at a single institution from January 2004 to December 2016 were reviewed. Primary outcome measures were reintervention-free survival (RFS) and overall survival.

Selective serotonin reuptake inhibitor or serotonin-norepinephrine reuptake inhibitors and epidemiological characteristics associated with prenatal diagnosis of congenital heart disease.

Objective: Identify early pregnancy associations of congenital heart disease (CHD) in a multiethnic cohort.

Methods: This retrospective observational cohort study compared the general obstetric population to women who gave birth at a referral centre in Australia between 2012 and 2017, after 20 weeks' of gestation, with a pregnancy affected by CHD. We defined mood disorder and anxiety as a history of self-reported or medically diagnosed anxiety, depression, postpartum depression or bipolar disorder.