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Background The Fontan procedure is the final in a series of staged palliations for single-ventricle congenital heart disease, which encompasses rare and heterogeneous cardiac lesions. It represents an unusual and novel physiological state characterised by absence of a subpulmonary ventricle. Aims The population is growing steadily, prompting creation of this registry to study their epidemiology, demographic trends, treatment and outcomes. Methods This multicentre, binational, prospective and retrospective, web-based registry involving all congenital cardiac centres in the region has identified nearly all Fontan patients in Australia and New Zealand. Patients identified retrospectively were approached for recruitment. New recipients are automatically enrolled prospectively unless they choose to opt-out. Follow-up data are collected yearly. Results Baseline data were obtained in 1072 patients as at 1 January 2011. Ninety-nine patients died; 64 were lost to follow up. Forty-four per cent of patients lost were between 20 and 30 years of age. The size of the Fontan population is increasing steadily. Among 973 living patients, 541 (56%) gave consent for prospective collection of follow up. Between 1 January 2011 and 1 January 2013, an additional 47 subjects were enrolled prospectively. The current proportion of patients operated with hypoplastic left heart syndrome is currently 29% and is growing rapidly. Conclusion The population surviving after the Fontan procedure has been growing in recent decades, especially since survival with hypoplastic left heart syndrome has improved. The Australia and New Zealand Fontan Registry provides population-based data, and only large databases like this will give opportunities for understanding the population and performing prospective trials. [ABSTRACT FROM AUTHOR]

Introduction: Rheumatic heart disease (RHD) is underdiagnosed globally resulting in missed treatment opportunities and adverse clinical outcomes. We describe the protocol for a study which aims to co-design, implement and conduct an evaluation of a task-sharing approach to echocardiographic active case finding for early detection and management of RHD in high-risk settings in Australia and Timor-Leste., Methods and Analysis: Echocardiograms will be obtained by trained local staff using hand-held echocardiographic devices employing the 'Single Parasternal Long Axis view with a Sweep of the Heart' (SPLASH) technique and interpreted by experts remote from the site of acquisition. Approximately 1500 children and pregnant women will be screened across high-risk communities in Australia and Timor-Leste over an 18-month period. The study will use a type II effectiveness-implementation hybrid design. A tailored package of implementation strategies will be co-designed with communities and health services and mapped onto a Theory of Change framework. The clinical effectiveness will be assessed as the change in the proportion of the target population that are prescribed secondary prophylaxis for RHD by the end of the study compared with baseline. The implementation will be assessed as the adoption, penetration, sustainability, fidelity and cost of the programme with a mixed-methods theory-based and economic evaluation. Data will include numbers of normal, abnormal and uninterpretable SPLASH echocardiograms obtained, numbers of participants progressing through the cascade of care, interviews with staff and programme costs., Ethics and Dissemination: Ethical approval has been obtained from the Human Research Ethics Committee of the NT Department of Health and Menzies School of Health Research, Darwin (HREC-2022-4479), the Western Australian Aboriginal Health Ethics Committee (HREC-1237) and the Instituto Nasional Saude Publika Timor-Leste Ethics and Technical Committee (03-UEPD/INSP-TL/V/2023). Informed consent is required to be enrolled. Study findings will be disseminated in the communities involved and submitted for publication., Trial Registration Number: NCT06002243., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ.)

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. Clinical data were provided by the Australia and New Zealand Fontan Registry., Results: Participants (mean age: 11.5 ± 2.6 years, 62% male) reported lower overall HRQOL (P < .001), and lower scores across all HRQOL domains (all P < .0001), compared with normative data. Median global QOL score was 7.0 (IQR 5.8-8.0), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of congenital heart disease explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes., Conclusions: Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population., Competing Interests: Declaration of Competing Interest This work was supported by HeartKids Australia and Australian National Health and Medical Research Council (NHMRC) Project (APP1065794) and Partnership (APP1076849) Grants. The authors have no conflicts of interest to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

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. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care., Competing Interests: Statement of Competing Interests The authors declare no known competing interests., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Objectives: The arterial switch operation (ASO) has excellent early outcomes in the modern era. We sought to determine the long-term outcomes in patients who underwent an ASO at a single institution., Methods: Patients who underwent an ASO between 1983 and 2015 were identified from the hospital database and retrospectively reviewed using hospital records., Results: From 1983 to 2015, 844 patients with a biventricular circulation underwent an ASO. There were 28 (3.3%, 28/844) early deaths. Follow-up was available for 94% (729/774) of local patients after hospital discharge. Median follow-up was 15 years (interquartile range, 8-20 years). There were 187 (26%, 187/729) patients with more than 20 years of follow-up and 95 (13%, 95/729) patients with more than 25 years of follow-up. Overall survival was 95% (95% confidence interval [CI], 94%-97%) at 10 and 25 years after the ASO. At 25 years after ASO, freedom from overall reintervention was 77% (95% CI, 73%-81%), freedom from reoperation on the neoaortic root or neoaortic valve was 92% (95% CI, 88%-95%), and freedom from coronary reoperation was 99% (95% CI, 98%-99.7%). Left ventricular (LV) systolic function was normal in 595 of 609 (98%) of patients who had LV function quantified at latest follow-up. Of the 95 patients with more than 25 years of follow-up after ASO, 6 (6.3%) had at least moderate neoaortic valve regurgitation (AR) and 8 (8.4%) had undergone replacement of the neoaortic valve., Conclusions: Overall, survivors of ASO have excellent late survival and normal LV systolic function into adult life. However, AR and reoperation on the neoaortic valve remains an issue for older patients., (Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.)

Background: Echocardiographic screening in school-aged children can detect rheumatic heart disease (RHD) prior to the manifestation of symptoms of heart failure. The challenge is making this practical and affordable on a global scale. This study aims to evaluate the diagnostic utility of an ultra-abbreviated echocardiographic screening protocol involving a single parasternal-long-axis-view-sweep of the heart (SPLASH) in two dimensional (2D) and colour Doppler imaging (index test)., Methods: This prospective study of diagnostic accuracy compared the diagnostic utility of the index screening test with a comprehensive reference test (standard echocardiographic screening protocols) as per World Heart Federation (WHF) echocardiographic criteria. School students in Timor-Leste aged 5-20 years were enrolled. Both index and reference test images were acquired by cardiologists on Vivid I or Q machines (GE Healthcare, Marlborough, MA, USA)., Results: A total of 1,365 participants were screened; median age was 11 years. The estimated prevalence of definite and borderline RHD was 35.2 per 1,000. Congenital heart disease was identified in 11 children (0.8%) with two needing cardiac surgery. Abnormal SPLASH views were found in 109/1365 (7.99%). No cases of RHD or significant congenital heart disease were missed. Sensitivity and specificity of the abbreviated protocol for detecting RHD were 1.0 and 0.95 respectively., Conclusions: A simplified echocardiography screening protocol using SPLASH is highly sensitive and specific and could significantly improve the efficiency of RHD screening. It has the potential to expedite training of health workers whilst protecting the modesty of students., (Copyright © 2019 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.)

Background: In order to determine the extent to which care delivered to children is appropriate (in line with evidence-based care and/or clinical practice guidelines (CPGs)) in Australia, we developed a set of clinical indicators for 21 common paediatric medical conditions for use across a range of primary, secondary and tertiary healthcare practice facilities., Methods: Clinical indicators were extracted from recommendations found through systematic searches of national and international guidelines, and formatted with explicit criteria for inclusion, exclusion, time frame and setting. Experts reviewed the indicators using a multi-round modified Delphi process and collaborative online wiki to develop consensus on what constituted appropriate care., Results: From 121 clinical practice guidelines, 1098 recommendations were used to draft 451 proposed appropriateness indicators. In total, 61 experts (n = 24 internal reviewers, n = 37 external reviewers) reviewed these indicators over 40 weeks. A final set of 234 indicators resulted, from which 597 indicator items were derived suitable for medical record audit. Most indicator items were geared towards capturing information about under-use in healthcare (n = 551, 92%) across emergency department (n = 457, 77%), hospital (n = 450, 75%) and general practice (n = 434, 73%) healthcare facilities, and based on consensus level recommendations (n = 451, 76%). The main reason for rejecting indicators was 'feasibility' (likely to be able to be used for determining compliance with 'appropriate care' from medical record audit)., Conclusion: A set of indicators was developed for the appropriateness of care for 21 paediatric conditions. We describe the processes (methods), provenance (origins and evolution of indicators) and products (indicator characteristics) of creating clinical indicators within the context of Australian healthcare settings. Developing consensus on clinical appropriateness indicators using a Delphi approach and collaborative online wiki has methodological utility. The final indicator set can be used by clinicians and organisations to measure and reflect on their own practice., Competing Interests: The authors have declared that no competing interests exist.

Background: Rheumatic heart disease (RHD) remains a leading cause of cardiovascular morbidity and mortality in children and young adults in disadvantaged populations. The emergence of echocardiographic screening provides the opportunity for early disease detection and intervention. Using our own multistate model of RHD progression derived from Australian RHD register data, we performed a cost-utility analysis of echocardiographic screening in indigenous Australian children, with the dual aims of informing policy decisions in Australia and providing a model that could be adapted in other countries., Methods and Results: We simulated the outcomes of 2 screening strategies, assuming that RHD could be detected 1, 2, or 3 years earlier by screening. Outcomes included reductions in heart failure, surgery, mortality, disability-adjusted life-years, and corresponding costs. Only a strategy of screening all indigenous 5- to 12-year-olds in half of their communities in alternate years was found to be cost-effective (incremental cost-effectiveness ratio less than AU$50 000 per disability-adjusted life-year averted), assuming that RHD can be detected at least 2 years earlier by screening; however, this result was sensitive to a number of assumptions. Additional modeling of improved adherence to secondary prophylaxis alone resulted in dramatic reductions in heart failure, surgery, and death; these outcomes improved even further when combined with screening., Conclusions: Echocardiographic screening for RHD is cost-effective in our context, assuming that RHD can be detected ≥2 years earlier by screening. Our model can be adapted to any other setting but will require local data or acceptable assumptions for model parameters., (© 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

Introduction: Australian and international clinical practice guidelines are available for common paediatric conditions. Yet there is evidence that there are substantial variations between the guidelines, recommendations (appropriate care) and the care delivered. This paper describes a study protocol to determine the appropriateness of the healthcare delivered to Australian children for 16 common paediatric conditions in acute and primary healthcare settings., Methods and Analysis: A random sample of 6000-8000 medical records representing a cross-section of the Australian paediatric population will be reviewed for appropriateness of care against a set of indicators within three Australian states (New South Wales, Queensland and South Australia) using multistage, stratified sampling. Medical records of children aged <16 years who presented with at least one of the study conditions during 2012 and 2013 will be reviewed., Ethics and Dissemination: Human Research Ethics Committee approvals have been received from the Sydney Children's Hospital Network, Children's Health Queensland Hospital and Health Service and Women's and Children's Hospital Network (South Australia). An application is under review for the Royal Australian College of General Practitioners. The authors will submit the results of the study to relevant journals and offer oral presentations to researchers, clinicians and policymakers at national and international conferences., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Introduction: Despite the widespread availability of clinical guidelines, considerable gaps remain between the care that is recommended (appropriate care) and the care provided. This protocol describes a research methodology to develop clinical indicators for appropriate care for common paediatric conditions., Methods and Analysis: We will identify conditions amenable to population-level appropriateness of care research and develop clinical indicators for each condition. Candidate conditions have been identified from published research; burden of disease, prevalence and frequency of presentation data; and quality of care priority lists. Clinical indicators will be developed through searches of national and international guidelines, and formatted with explicit criteria for inclusion, exclusion, time frame and setting. Experts will review the indicators using a wiki-based approach and modified Delphi process. A formative evaluation of the wiki process will be undertaken., Ethics and Dissemination: Human Research Ethics Committee approvals have been received from Sydney Children's Hospital Network, Children's Health Queensland Hospital and Health Service, and the Women's and Children's Health Network (South Australia). Applications are under review with Macquarie University and the Royal Australian College of General Practitioners. We will submit the results of the study to relevant journals and offer national and international presentations., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background: Acute rheumatic fever (ARF) is an important cause of heart disease in Indigenous people of northern and central Australia. However, little is known about ARF in children across all Australian population groups. This national prospective study was conducted to determine patterns of disease, and populations and regions at highest risk., Methods: The Australian Paediatric Surveillance Unit surveillance model was used to collect data on children with ARF across Australia. Children up to 15 years of age were included if they had an ARF episode diagnosed between October 1, 2007 and December 31, 2010 that met the case definition., Results: ARF was identified in 151 children: 131 Indigenous Australians, 10 non-Indigenous Australians, 8 Pacific Islanders and 1 African (1 unknown). Common presenting features were joint symptoms, fever and carditis. Sydenham chorea was reported in 19% of children. Aseptic monoarthritis was a major manifestation in 19% of high-risk children. Seven non-Indigenous Australian children presented with classic, highly specific features compared with 23% of high-risk children, suggesting that subtle presentations of ARF are being missed in non-Indigenous children. Recent sore throat was reported in 33% of cases, including 25% of remote Indigenous children. There were delays in presentation to care and referral to higher-level care across urban/rural and remote areas., Conclusions: ARF may be more common than previously thought among low-risk children. These data should prompt an awareness of ARF diagnosis and management across all regions, including strategies for primary prevention. There should be renewed emphasis on treatment of sore throat in high-risk groups.

Three priority areas in the prevention, diagnosis and management of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) were identified and discussed in detail: 1. Echocardiography and screening/diagnosis of RHD – Given the existing uncertainty it remains premature to advocate for or to incorporate echocardiographic screening for RHD into Australian clinical practice. Further research is currently being undertaken to evaluate the potential for echocardiography screening. 2. Secondary prophylaxis – Secondary prophylaxis (long acting benzathine penicillin injections) must be seen as a priority. Systems-based approaches are necessary with a focus on the development and evaluation of primary health care-based or led strategies incorporating effective health information management systems. Better/novel systems of delivery of prophylactic medications should be investigated. 3. Management of advanced RHD – National centres of excellence for the diagnosis, assessment and surgical management of RHD are required. Early referral for surgical input is necessary with multidisciplinary care and team-based decision making that includes patient, family, and local health providers. There is a need for a national RHD surgical register and research strategy for the assessment, intervention and long-term outcome of surgery and other interventions for RHD., (Copyright © 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Background: There are limited data on the epidemiology and viral aetiology of bronchiolitis in Central Australia and respiratory syncytial virus (RSV) immunoprophylaxis in an Australian population., Objective: To (i) determine the incidence and the viral aetiology of bronchiolitis hospitalisations and (ii) report on the usage of RSV immunoprophylaxis in selected high-risk infants and children in Central Australia., Methodology: A retrospective review was performed of all hospital separations for bronchiolitis for a three-year period, 1998-2000. Respiratory viruses in the nasopharyngeal aspirates were identified from the cases in the year 2000. A combined retrospective chart review and prospective follow up study was undertaken of all the infants and children who received RSV immunoprophylaxis at the Alice Springs Hospital, Central Australia., Results: Incidence of bronchiolitis hospitalisation in infants for 1998, 1999 and 2000 were 176, 200 and 180 per 1000, respectively. Nine high-risk children had RSV immunoprophylaxis on a total of 46 occasions and there were two mild RSV-related illnesses in them. None had severe lower respiratory tract illness., Conclusion: The incidence of bronchiolitis in Central Australia is extremely high. The usage of RSV immunoprophylaxis may be justified in selected high-risk children living in high endemic areas.

Objective: To determine the incidence of congenital heart defects (CHD) in Aboriginal and non-Aboriginal infants in Central Australia and to compare this with the incidence elsewhere in Australia., Design and Setting: Data on cases were obtained from patient records of the Alice Springs Hospital, Central Australia, the sole referral centre for paediatric and initial cardiac diagnostic services for the region., Participants: Patients with CHD proven by echocardiography reported between 1 January 1993 and 30 June 2000., Main Outcome Measures: Incidence of CHD using all live births in Central Australia as the denominator., Results: 108 patients with CHD were detected among 6156 live births (incidence, 17.5 per 1000; 95% CI, 14.9-21.7 per 1000); 57 of 2991 were Aboriginal (19.0 per 1000; 95% CI, 14.4-24.6 per 1000) and 51 of 3165 were non-Aboriginal (16.1 per 1000; 95% CI, 12.0-21.1 per 1000). The difference between the two groups was not statistically significant (relative risk, 1.18; 95% CI, 0.81-1.72). CHD incidence in Central Australia was significantly higher than that reported for other parts of Australia (4.3 per 1000 live births in New South Wales and the Australian Capital Territory, 1981-1984; 7.65 and 12 per 1000 total births in Western Australia, 1980-1989, and South Australia, 1993-2000, respectively)., Conclusions: The high rates of CHD in Central Australia may partly reflect the high utilisation of echocardiography for assessing minor lesions. However, the incidence of both major and minor types of CHD was significantly higher than previously reported from other regions of Australia. The role of socioenvironmental factors in this high incidence should be explored.

Childhood bronchiectasis not related to underlying disease is still common in some populations in affluent countries. The aims of the study were to: 1) describe demographics, 2) evaluate the effectiveness of routine investigations, and 3) determine the relationship between spirometry and radiology scoring systems, in children with chronic suppurative lung disease (CSLD) living in Central Australia. Data of children living in Central Australia aged 70%) and early hospitalisation for pneumonia were common (median age, 0.5 years). Previous admissions for pneumonia were almost universally present and significantly more common than bronchiolitis (95% CI for proportional difference, 0.4-0.51). Although the majority did not have a treatable underlying cause, investigations had significant impact on management in 12.3% of children. None of the chest HRCT scores related to any spirometry data. In conclusion, CSLD is unacceptably common in indigenous children of this region, commences early in life, and is associated with significant comorbidities. Spirometry data do not reflect the severity of lung disease in HRCT scans. While improvement in the living standards is of utmost importance, the medical management that includes thorough investigations of these children should not be neglected., (Copyright 2003 Wiley-Liss, Inc.)