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.

Utility of routine chest radiographs after chest drain removal in paediatric cardiac surgical patients-a retrospective analysis of 1076 patients.

Objectives: Chest drains are routinely placed in children following cardiac surgery. The purpose of this study was to determine the incidence of a clinically relevant pneumothorax and/or pleural effusion after drain removal and to ascertain if a chest radiograph can be safely avoided following chest drain removal.

Methods: This single-centre retrospective cohort study included all patients under 18 years of age who underwent cardiac surgery between January 2015 and December 2019 with the insertion of mediastinal and/or pleural drains.

Systematic Review of Instruments Assessing Psychosocial Adaptation and Outcomes Among Families of Children With Congenital Heart Disease.

Objective: This systematic review identified instruments quantitatively assessing psychosocial adaptation and outcomes in families of children with congenital heart disease (CHD) and evaluated instrument psychometrics.

Methods: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and a prospectively registered protocol, electronic databases (CINAHL, Embase, PubMed/MEDLINE, PsycINFO, and SCOPUS) were searched from inception until June 20, 2021 for peer-reviewed articles published in English, reporting quantitative data on psychosocial outcomes among parents/caregivers, siblings, or family system. Instrument characteristics and psychometrics were extracted, and adapted COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) criteria were applied to assess instrument quality.

Fontan operation at less than 3 years of age is not a risk factor for long-term failure.

Objectives: The age at which the Fontan operation is performed varies globally. Over the last decade, the median age of patients having the Fontan in Australia and New Zealand has been 4.6 years, including 6% of patients younger than 3 years.

Synopsis of an integrated guidance for enhancing the care of familial hypercholesterolaemia: an Australian perspective.

Introduction: Familial hypercholesterolaemia (FH) is a common, heritable and preventable cause of premature coronary artery disease, with significant potential for positive impact on public health and healthcare savings. New clinical practice recommendations are presented in an abridged guidance to assist practitioners in enhancing the care of all patients with FH.

Main Recommendations: Core recommendations are made on the detection, diagnosis, assessment and management of adults, children and adolescents with FH.

Essentials of a new clinical practice guidance on familial hypercholesterolaemia for physicians.

Familial hypercholesterolaemia (FH) is a common, heritable and preventable cause of premature coronary artery disease. New clinical practice recommendations are presented to assist practitioners in enhancing the care of all patients with FH. Core recommendations are made on the detection, diagnosis, assessment and management of adults, children and adolescents with FH.

Balloon dilatation versus surgical valvotomy for congenital aortic stenosis: a propensity score matched study.

Background: Balloon valvuloplasty and surgical aortic valvotomy have been the treatment mainstays for congenital aortic stenosis in children. Choice of intervention often differs depending upon centre bias with limited relevant, comparative literature.

Objectives: This study aims to provide an unbiased, contemporary matched comparison of these balloon and surgical approaches.

Integrated Guidance for Enhancing the Care of Familial Hypercholesterolaemia in Australia.

Familial hypercholesterolaemia (FH) is a dominant and highly penetrant monogenic disorder present from birth that markedly elevates plasma low-density lipoprotein (LDL)-cholesterol concentration and, if untreated, leads to premature atherosclerosis and coronary artery disease (CAD). There are approximately 100,000 people with FH in Australia. However, an overwhelming majority of those affected remain undetected and inadequately treated, consistent with FH being a leading challenge for public health genomics.

Long-term outcomes following Fontan takedown in Australia and New Zealand.

Objective: Fontan takedown remains an option for the management of Fontan failure. We sought to evaluate early and late outcomes after Fontan takedown.

Methods: The Australia and New Zealand Fontan Registry was interrogated to identify all patients who had a Fontan takedown.

Long-term Out-of-Hospital Health Care Use for Fontan Survivors Across Childhood.

Background: Surgery is the cornerstone of treatment for single-ventricle patients, but life just begins for most when the staged procedures are completed. A package of care, including primary care, out-of-hospital specialist visits, and medication, is required for patients living with Fontan. This study quantified the current state of out-of-hospital health care use across childhood for Fontan patients using evidence from Australia.

Modes of late mortality in patients with a Fontan circulation.

Objectives: The mechanisms of attrition of the Fontan population have been poorly characterised and it is unclear whether some of the deaths are potentially preventable. We analysed the circumstances of late death in patients with a Fontan circulation, with a special focus on identifying lesions amenable to intervention that may have contributed to the decline of their circulation.

Methods: Between 1975 and 2018, a total of 105 patients from a Bi-National Registry died beyond 1 year after Fontan completion, at a median age of 18.

Heterotaxy Is Not a Risk Factor for Adverse Long-Term Outcomes After Fontan Completion.

Background: Heterotaxy is considered a risk factor for poor outcomes after the Fontan operation. However, long-term data to support this notion are lacking. The aims of this study were to ascertain the long-term outcomes of patients with heterotaxy after hospital discharge after Fontan completion and to compare these outcomes with those of a contemporary nonheterotaxy cohort.

Neurodevelopmental and health-related quality-of-life outcomes in adolescence after surgery for congenital heart disease in infancy.

Aim: To assess outcomes in adolescence after surgery for congenital heart disease (CHD) in infancy. Domains analysed included cognition and executive function, social and emotional well-being, adaptive behaviour, academic achievement, and health-related quality of life (HRQoL).

Method: Twenty-one participants (10 males, 11 females) ranged in age from 14 to 17 years (mean 15y 4.

Augmentation of the pulmonary arteries at or prior to the Fontan procedure is not associated with worse long-term outcomes: a propensity-matched analysis from the Australia-New Zealand Fontan Registry†.

Objectives: Long-term data on the impact of pulmonary artery (PA) augmentation in patients who underwent the Fontan procedure are lacking. The aim of this study was to examine whether surgical or transcatheter PA augmentation at or prior to the Fontan procedure adversely affects the outcomes.

Methods: Data of 1436 patients from the Australia-New Zealand Fontan Registry (1975-2015) were analysed.

Hepatic and renal end-organ damage in the Fontan circulation: A report from the Australian and New Zealand Fontan Registry.

Background: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined.

Methods: A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR).

Results: Mean age and time since Fontan were 19.

Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure.

The objective of this study was to characterize late outcomes of the lateral tunnel (LT) Fontan procedure. The outcomes of all patients who underwent an LT Fontan procedure in Australia and in New Zealand were analyzed. Original files were reviewed and outcomes data were obtained through a binational registry.

Hospital Inpatient Costs for Single Ventricle Patients Surviving the Fontan Procedure.

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.

Comparison of outcomes in Australian indigenous and non-indigenous children and adolescents undergoing cardiac surgery.

Background: Population-based registries report 95% 5-year survival for children undergoing surgery for CHD. This study investigated paediatric cardiac surgical outcomes in the Australian indigenous population.

Methods: All children who underwent cardiac surgery between May, 2008 and August, 2014 were studied.

Hospital costs and cost implications of co-morbid conditions for patients with single ventricle in the period through to Fontan completion.

Background: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed.

Methods: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions.

Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure.

Objective: To review our experience of patients with an atrio-pulmonary Fontan circulation to determine their long-term outcomes.

Methods And Results: A retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry was performed. There were 215 patients surviving hospital discharge after an atrio-pulmonary Fontan completion.

The Fontan epidemic: Population projections from the Australia and New Zealand Fontan Registry.

Background: The number and age demographic of the future Fontan population is unknown.

Methods: Population projections were calculated probabilistically using microsimulation. Mortality hazard rates for each Fontan recipient were calculated from survivorship of 1353 Fontan recipients in the Australia and New Zealand Fontan Registry, based on Fontan type, age at Fontan, gender and morphology.

No difference between aspirin and warfarin after extracardiac Fontan in a propensity score analysis of 475 patients.

Objectives: The most effective method of long-term thromboprophylaxis after the Fontan procedure is not clear. We compared the rates of thromboembolic events between patients receiving aspirin and warfarin after an extracardiac conduit Fontan procedure in a bi-national registry.

Methods: All patients who underwent an extracardiac conduit Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified.

Use of ACE inhibitors in Fontan: Rational or irrational?

Background: Despite a lack of evidence supporting the use of angiotensin-converting enzyme (ACE) inhibitors in patients with a Fontan circulation, their use is frequent. We decided to identify the rationale for ACE inhibitor therapy in patients within the Australia and New Zealand Fontan Registry.

Methods: All patients in the Registry taking an ACE inhibitor at last follow up were identified, and a review of medical records was undertaken to determine the rationale for treatment initiation and reasons for treatment continuation or dose increase.