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. 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.

2023 World Heart Federation guidelines for the echocardiographic diagnosis of rheumatic heart disease.

Rheumatic heart disease (RHD) is an important and preventable cause of morbidity and mortality among children and young adults in low-income and middle-income countries, as well as among certain at-risk populations living in high-income countries. The 2012 World Heart Federation echocardiographic criteria provided a standardized approach for the identification of RHD and facilitated an improvement in early case detection. The 2012 criteria were used to define disease burden in numerous epidemiological studies, but researchers and clinicians have since highlighted limitations that have prompted a revision. In this updated version of the guidelines, we incorporate evidence from a scoping review, an expert panel and end-user feedback and present an approach for active case finding for RHD, including the use of screening and confirmatory criteria. These guidelines also introduce a new stage-based classification for RHD to identify the risk of disease progression. They describe the latest evidence and recommendations on population-based echocardiographic active case finding and risk stratification. Secondary antibiotic prophylaxis, echocardiography equipment and task sharing for RHD active case finding are also discussed. These World Heart Federation 2023 guidelines provide a concise and updated resource for clinical and research applications in RHD-endemic regions.

Abbreviated Echocardiographic Screening for Rheumatic Heart Disease by Nonexperts with and without Offsite Expert Review: A Diagnostic Accuracy Study.

BACKGROUND: Early detection of rheumatic heart disease (RHD) through echocardiographic screening can facilitate early access to effective treatment, which reduces the risk for progression. Accurate, feasible approaches to echocardiographic screening that can be incorporated into routine health services are needed. The authors hypothesized that offsite expert review could improve the diagnostic accuracy of nonexpert-obtained echocardiographic images. METHODS: This prospective cross-sectional study was performed to evaluate the diagnostic accuracy of health worker-conducted single parasternal long-axis view with a sweep of the heart using hand-carried ultrasound for the detection of RHD in high-risk populations in Timor-Leste and Australia. In the primary analysis, the presence of any mitral or aortic regurgitation met the criteria for a positive screening result. Sensitivity and specificity were calculated for a screen-and-refer approach based on nonexpert practitioner assessment (approach 1) and for an approach using offsite expert review of nonexpert practitioner-obtained images to decide onward referral (approach 2). Each participant had a reference test performed by an expert echocardiographer on the same day as the index test. Diagnosis of RHD was determined by a panel of three experts, using 2012 World Heart Federation criteria. RESULTS: The prevalence of borderline or definite RHD among 3,329 participants was 4.0% (95% CI, 3.4%-4.7%). The sensitivity of approach 1 for borderline or definite RHD was 86.5% (95% CI, 79.5%-91.8%), and the specificity was 61.4% (95% CI, 59.7%-63.1%). Approach 2 achieved similar sensitivity (88.4%; 95% CI, 81.5%-93.3%) and improved specificity (77.1%; 95% CI, 75.6%-78.6%). CONCLUSION: Nonexpert practitioner-obtained single parasternal long-axis view with a sweep of the heart images, reviewed by an offsite expert, can detect borderline and definite RHD on screening with reasonable sensitivity and specificity. Brief training of nonexpert practitioners with ongoing support could be used as an effective strategy for scaling up echocardiographic screening for RHD in high-risk settings.

Does leaving native antegrade pulmonary blood flow at the time of the superior cavopulmonary connection impact long-term outcomes after the Fontan?

Objectives: Antegrade pulmonary blood flow (APBF) may be left or eliminated at the time of the superior cavopulmonary connection (SCPC). Our aim was to assess the impact of leaving native APBF at the SCPC on long-term Fontan outcomes. Methods: In the Australia and New Zealand Fontan Registry (1985-2021), 587 patients had pre-existing native APBF at the SCPC. At the SCPC, 302 patients had APBF eliminated (APBF-) and 285 patients had APBF maintained (APBF+). The incidence of Fontan failure (composite end point of Fontan takedown, transplant, plastic bronchitis, protein losing enteropathy and death) and atrioventricular (AV) valve repair/replacement post SCPC was compared between the 2 groups. Results: Sex, predominant-ventricle morphology, isomerism, primary diagnosis, and age/type of Fontan were similar between groups. APBF- versus APBF+ had a higher incidence of arch obstruction/coarctation (17% vs 7%) and previous pulmonary artery band (54% vs 45%) and a lower rate of Fontan fenestration (27% vs 41%). The risk of Fontan failure was similar between the 2 groups (hazard ratio [HR], 1.01; 95% confidence interval [CI], 0.58-1.78; P = .96). The risk of AV-valve repair/replacement was greater in APBF+ versus APBF- (HR, 2.32; CI, 1.13-4.75; P = .022). The risk of AV-valve repair/replacement remained after adjustment for arch obstruction/coarctation, previous pulmonary artery band and Fontan fenestration (HR, 2.27; CI, 1.07-4.81; P = .033). Conclusions: Maintaining APBF at the time of the SCPC does not impact the risk of Fontan failure but may increase the incidence of AV-valve repair and/or replacement post-SCPC.

Long-term outcomes of the arterial switch operation.

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.

Single-View Echocardiography by Nonexpert Practitioners to Detect Rheumatic Heart Disease: A Prospective Study of Diagnostic Accuracy.

BACKGROUND: Echocardiographic screening can detect asymptomatic cases of rheumatic heart disease (RHD), facilitating access to treatment. Barriers to implementation of echocardiographic screening include the requirement for expensive equipment and expert practitioners. We aimed to evaluate the diagnostic accuracy of an abbreviated echocardiographic screening protocol (single parasternal-long-axis view with a sweep of the heart) performed by briefly trained, nonexpert practitioners using handheld ultrasound devices. METHODS: Participants aged 5 to 20 years in Timor-Leste and the Northern Territory of Australia had 2 echocardiograms: one performed by an expert echocardiographer using a GE Vivid I or Vivid Q portable ultrasound device (reference test), and one performed by a nonexpert practitioner using a GE Vscan handheld ultrasound device (index test). The accuracy of the index test, compared with the reference test, for identifying cases with definite or borderline RHD was determined. RESULTS: There were 3111 enrolled participants; 2573 had both an index test and reference test. Median age was 12 years (interquartile range, 10-15); 58.2% were female. Proportion with definite or borderline RHD was 5.52% (95% CI, 4.70-6.47); proportion with definite RHD was 3.23% (95% CI, 2.61-3.98). Compared with the reference test, sensitivity of the index test for definite or borderline RHD was 70.4% (95% CI, 62.2-77.8), specificity was 78.1% (95% CI, 76.4-79.8). CONCLUSIONS: Nonexpert practitioners can be trained to perform single parasternal-long-axis view with a sweep of the heart echocardiography. However, the specificity and sensitivity are inadequate for echocardiographic screening. Improved training for nonexpert practitioners should be investigated.

Pre- and Post-operative determinants of transplantation-free survival after Fontan. The Australia and New Zealand experience.

BACKGROUND: This review identifies the predictors of late mortality and heart transplantation that remain relevant in the contemporary population of patients with a Fontan circulation, focusing on the potential impact of post-Fontan morbidities on the late outlook of these patients. METHODS AND RESULTS: A total of 1561 patients who had survived the Fontan operation in Australia or New Zealand from 1975 to 2018 were included in this review. Over a median duration of 11.4 years, there was a total of 117 deaths (7%) and 32 heart transplantations (2%). Freedom from death and heart transplantation at 10, 20 and 35 years post Fontan surgery were 94% (95% CI 93-95%), 87% (95 %CI 85-90%) and 66% (95 %CI 57-78%) respectively. Being male, having an atriopulmonary Fontan, pre-Fontan atrioventricular valve intervention, or prolonged pleural effusions post Fontan were predictive of late death or heart transplantation. However, time-dependent variables such as the development of atrial arrhythmia, protein/losing enteropathy or late ventricular dysfunction were stronger predictors of the same outcome. Patients who developed a time-dependent risk factor had a freedom from death and heart transplantation rate of 54% (95 %CI 43-66) at 15 years and 44% (95 %CI 33-57) at 25 years post Fontan. However, 95% (95 %CI 91-99) of patients without any of the identified risk factors were free from death or heart transplantation rate at 25 years post Fontan. CONCLUSION: In conclusion, the occurrence of post-operative complications such as PLE, arrhythmia and ventricular dysfunction will likely precede the late demise of these patients.

Protein-losing enteropathy and plastic bronchitis after the Fontan procedure.

OBJECTIVES: Protein losing enteropathy and plastic bronchitis are severe complications in Fontan circulation, with 5-year survival ranging from 46% to 88%. We report risk factors and outcomes of protein losing enteropathy and plastic bronchitis in patients undergoing the Fontan. METHODS: We performed a retrospective analysis of 1561 patients from the Australia New Zealand Fontan Registry. Two end points were death and cardiac transplantation examined with Cox regression (if no competing risks) or cumulative incidence curves and cause-specific Cs regression. RESULTS: A total of 55 patients with protein losing enteropathy/plastic bronchitis were included. Their median age at the Fontan was 5.7 years, and time to onset after the Fontan for protein losing enteropathy was 5.0 years and plastic bronchitis was 1.7 years. Independent predictors for developing protein losing enteropathy/plastic bronchitis were right-ventricular morphology with hypoplastic left-heart syndrome (hazard ratio, 2.30; confidence interval, 1.12-4.74), older age at Fontan (hazard ratio, 1.13; confidence interval, 1.03-1.23), and pleural effusions after Fontan (hazard ratio, 2.43; confidence interval, 1.09-5.41); left-ventricular morphology was protective (hazard ratio, 0.36; confidence interval, 0.18-0.70). In the protein losing enteropathy/plastic bronchitis population, freedom from death or transplantation after protein losing enteropathy/plastic bronchitis diagnosis at 5, 10, and 15 years was 70% (confidence interval, 58-85), 65% (confidence interval, 51-83), and 43% (confidence interval, 26-73), respectively; only older age (hazard ratio, 1.23; confidence interval, 1.01-1.52) was an independent predictor. Twenty-six surgical interventions were performed in 20 patients, comprising Fontan revisions (n = 5), fenestrations (n = 11), Fontan conversions (n = 5), atrioventricular valve repairs (n = 3), and hepatic vein diversion (n = 2). CONCLUSIONS: Protein losing enteropathy and plastic bronchitis remain severe complications, preferably affecting patients with dominant right single ventricle, with older age at Fontan being a predictor of developing protein losing enteropathy/plastic bronchitis and poorer prognosis. Heart transplantation remains the ultimate treatment, with 30% dying or requiring transplantation within 5 years, and the remaining being stable for long periods.

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. METHODS: Patients recruited from the Fontan Registry were linked with the administrative Medicare (universal health insurance) data. Frequency of medical and pharmaceutical care and costs were estimated. RESULTS: Data for 115 patients with 12,726 medical and 8,336 pharmaceutical claims were obtained. From age 0 to 20 years, patients on average visited a general practitioner an estimated 6.4 times per year (95% confidence interval [CI], 5.9-7.0 times per year), and specialists, including cardiologists, 2.8 times per year (95% CI, 2.5-3.0 times per year). Average use of allied health professional care peaked at age 6 to 9 years (1.0 visits per year; 95% CI, 0.7-1.4 visits per year) with psychologic services being most prominent. For pharmaceuticals, an average of 13.3 prescriptions filled per patient per year throughout childhood was observed (95% CI, 12.4-14.2 prescriptions per year). Overall, out-of-hospital doctor visits of all types averaged 11 visits per year from birth to 20 years. A decline in care was observed when patients reached 18 years across services. CONCLUSIONS: Patterns of out-of-hospital health care use were observed; however, current guidelines are silent about whether this is optimal. Further research is needed to better understand the comprehensive needs of this population.

Management of fever in Australian children: a population-based sample survey.

BACKGROUND: Fever in childhood is a common acute presentation requiring clinical triage to identify the few children who have serious underlying infection. Clinical practice guidelines (CPGs) have been developed to assist clinicians with this task. This study aimed to assess the proportion of care provided in accordance with CPG recommendations for the management of fever in Australian children. METHODS: Clinical recommendations were extracted from five CPGs and formulated into 47 clinical indicators for use in auditing adherence. Indicators were categorised by phase of care: assessment, diagnosis and treatment. Patient records from children aged 0 to 15 years were sampled from general practices (GP), emergency departments (ED) and hospital admissions in randomly-selected health districts in Queensland, New South Wales and South Australia during 2012 and 2013. Paediatric nurses, trained to assess eligibility for indicator assessment and adherence, reviewed eligible medical records. Adherence was estimated by individual indicator, phase of care, age-group and setting. RESULTS: The field team conducted 14,879 eligible indicator assessments for 708 visits by 550 children with fever in 58 GP, 34 ED and 28 hospital inpatient settings. For the 33 indicators with sufficient data, adherence ranged from 14.7 to 98.1%. Estimated adherence with assessment-related indicators was 51.3% (95% CI: 48.1-54.6), 77.5% (95% CI: 65.3-87.1) for diagnostic-related indicators and 72.7% (95% CI: 65.3-79.3) for treatment-related indicators. Adherence for children < 3 months of age was 73.4% (95% CI: 58.0-85.8) and 64.7% (95% CI: 57.0-71.9) for children 3-11 months of age, both significantly higher than for children aged 4-15 years (53.5%; 95% CI: 50.0-56.9). The proportion of adherent care for children attending an ED was 77.5% (95% CI: 74.2-80.6) and 76.7% (95% CI: 71.7-81.3) for children admitted to hospital, both significantly higher than for children attending a GP (40.3%; 95% CI: 34.6-46.1). CONCLUSIONS: This study reports a wide range of adherence by clinicians to 47 indicators of best practice for the management of febrile children, sampled from urban and rural regions containing 60% of the Australian paediatric population. Documented adherence was lowest for indicators related to patient assessment, for care provided in GP settings, and for children aged 4-15 years.

Single Parasternal-Long-Axis-View-Sweep Screening Echocardiographic Protocol to Detect Rheumatic Heart Disease: A Prospective Study of Diagnostic Accuracy.

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.

Inter-rater and intra-rater reliability and agreement of echocardiographic diagnosis of rheumatic heart disease using the World Heart Federation evidence-based criteria.

OBJECTIVE: Different definitions have been used for screening for rheumatic heart disease (RHD). This led to the development of the 2012 evidence-based World Heart Federation (WHF) echocardiographic criteria. The objective of this study is to determine the intra-rater and inter-rater reliability and agreement in differentiating no RHD from mild RHD using the WHF echocardiographic criteria. METHODS: A standard set of 200 echocardiograms was collated from prior population-based surveys and uploaded for blinded web-based reporting. Fifteen international cardiologists reported on and categorised each echocardiogram as no RHD, borderline or definite RHD. Intra-rater and inter-rater reliability was calculated using Cohen's and Fleiss' free-marginal multirater kappa (κ) statistics, respectively. Agreement assessment was expressed as percentages. Subanalyses assessed reproducibility and agreement parameters in detecting individual components of WHF criteria. RESULTS: Sample size from a statistical standpoint was 3000, based on repeated reporting of the 200 studies. The inter-rater and intra-rater reliability of diagnosing definite RHD was substantial with a kappa of 0.65 and 0.69, respectively. The diagnosis of pathological mitral and aortic regurgitation was reliable and almost perfect, kappa of 0.79 and 0.86, respectively. Agreement for morphological changes of RHD was variable ranging from 0.54 to 0.93 κ. CONCLUSIONS: The WHF echocardiographic criteria enable reproducible categorisation of echocardiograms as definite RHD versus no or borderline RHD and hence it would be a suitable tool for screening and monitoring disease progression. The study highlights the strengths and limitations of the WHF echo criteria and provides a platform for future revisions.

Clinical indicators for common paediatric conditions: Processes, provenance and products of the CareTrack Kids study.

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.

Assessing the Quality of the Management of Tonsillitis among Australian Children: A Population-Based Sample Survey.

OBJECTIVE: The aims of this study were twofold: (1) to design and validate a set of clinical indicators of appropriate care for tonsillitis and (2) to measure the level of tonsillitis care that is in line with guideline recommendations in a sample of Australian children. STUDY DESIGN: A set of tonsillitis care indicators was developed from available national and international guidelines and validated in 4 stages. This research used the same design as the CareTrack Kids study, which was described in detail elsewhere. SETTING: Samples of patient records from general practices, emergency departments, and hospital admissions were assessed. SUBJECTS AND METHODS: Patient records of children aged 0 to 15 years were assessed for the presence of, and adherence to, the indicators for care delivered in 2012 and 2013. RESULTS: Eleven indicators were developed. The records of 821 children (mean age, 5.0 years; SD, 4.0) with tonsillitis were screened. The reviewers conducted 2354 eligible indicator assessments across 1127 visits. Adherence to 6 indicators could be assessed and ranged from 14.3% to 73.2% (interquartile range 31.5% to 72.2%). CONCLUSION: Our main findings are consistent with the international literature: the treatment of many children who present with confirmed or suspected tonsillitis is inconsistent with current guidelines. Future research should consider how the indicators could be applied in a structured and automated manner to increase the reliability and efficiency of record reviews and help raise clinicians' awareness of appropriate tonsillitis management.

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 19.8 ±â€¯9.3 and 14.1 ±â€¯7.6 years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (r = 0.24, p = 0.015) and ACR (r = 0.29, p = 0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, p = 0.002). Renal impairment was mild (mGFR 60-89 ml/min/1.73 m2) in 46/131 (35%) and moderate (mGFR 30-59 ml/min/1.73 m2) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (ß = 0.89, 95% CI 0.54-1.25, p = 0.002) and decreased mGFR (ß = -0.77, 95% CI -1.29-0.24, p = 0.005). CONCLUSIONS: In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion.

Quality of Health Care for Children in Australia, 2012-2013.

Importance: The quality of routine care for children is rarely assessed, and then usually in single settings or for single clinical conditions. Objective: To estimate the quality of health care for children in Australia in inpatient and ambulatory health care settings. Design, Setting, and Participants: Multistage stratified sample with medical record review to assess adherence with quality indicators extracted from clinical practice guidelines for 17 common, high-burden clinical conditions (noncommunicable [n = 5], mental health [n = 4], acute infection [n = 7], and injury [n = 1]), such as asthma, attention-deficit/hyperactivity disorder, tonsillitis, and head injury. For these 17 conditions, 479 quality indicators were identified, with the number varying by condition, ranging from 9 for eczema to 54 for head injury. Four hundred medical records were targeted for sampling for each of 15 conditions while 267 records were targeted for anxiety and 133 for depression. Within each selected medical record, all visits for the 17 targeted conditions were identified, and separate quality assessments made for each. Care was evaluated for 6689 children 15 years of age and younger who had 15 240 visits to emergency departments, for inpatient admissions, or to pediatricians and general practitioners in selected urban and rural locations in 3 Australian states. These visits generated 160 202 quality indicator assessments. Exposures: Quality indicators were identified through a systematic search of local and international guidelines. Individual indicators were extracted from guidelines and assessed using a 2-stage Delphi process. Main Outcomes and Measures: Quality of care for each clinical condition and overall. Results: Of 6689 children with surveyed medical records, 53.6% were aged 0 to 4 years and 55.5% were male. Adherence to quality of care indicators was estimated at 59.8% (95% CI, 57.5%-62.0%; n = 160 202) across the 17 conditions, ranging from a high of 88.8% (95% CI, 83.0%-93.1%; n = 2638) for autism to a low of 43.5% (95% CI, 36.8%-50.4%; n = 2354) for tonsillitis. The mean adherence by condition category was estimated as 60.5% (95% CI, 57.2%-63.8%; n = 41 265) for noncommunicable conditions (range, 52.8%-75.8%); 82.4% (95% CI, 79.0%-85.5%; n = 14 622) for mental health conditions (range, 71.5%-88.8%); 56.3% (95% CI, 53.2%-59.4%; n = 94 037) for acute infections (range, 43.5%-69.8%); and 78.3% (95% CI, 75.1%-81.2%; n = 10 278) for injury. Conclusions and Relevance: Among a sample of children receiving care in Australia in 2012-2013, the overall prevalence of adherence to quality of care indicators for important conditions was not high. For many of these conditions, the quality of care may be inadequate.

Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death.

OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. RESULTS: Late follow-up data were available for 120 patients. The median length of follow-up after the Fontan procedure was 9.1 years (interquartile range 4.2-15.4 years). Late death occurred in 9% of patients (11/120). Six were sudden, unexpected deaths; 4 of those occurred in patients known to have right ventricle-dependent coronary circulation (RVDCC). Those with RVDCC had a higher incidence of sudden death (4/20 vs 2/100; P = 0.007). RVDCC was associated with late death (P = 0.01) and the development of myocardial ischaemia after Fontan completion (P < 0.001). The 10-year survival rate was 77% (95% confidence interval 56-100%) for patients with RVDCC vs 96% (95% confidence interval 92-100%) for patients without RVDCC. CONCLUSIONS: Long-term survival of patients with pulmonary atresia with intact ventricular septum after the Fontan procedure is excellent, but patients with RVDCC remain susceptible to coronary ischaemia and sudden death. Closer surveillance and investigation for exercise-induced ischaemia may be necessary.

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. Between 1980 and 2014, a total of 301 patients underwent an LT Fontan procedure across 6 major centers. There were 13 hospital mortalities, 21 late deaths, 8 Fontan conversions and revisions, 8 Fontan takedowns, and 4 heart transplantations. Overall survival at 15 and 25 years was 90% (95% confidence interval [CI]: 86%-93%) and 80% (95% CI: 69%-91%), respectively. Protein-losing enteropathy or plastic bronchitis was observed in 14 patients (5%). Freedom from late failure at 15 and 25 years was 88% (95% CI: 84%-92%) and 82% (95% CI: 76%-87%), respectively. Independent predictors of late Fontan failure were prolonged pleural effusions post Fontan operations (hazard ratio [HR] 3.06, 1.05-8.95, P = 0.041), age >7 years at Fontan (vs 3-5 years, HR 9.7, 2.46-38.21, P = 0.001) and development of supraventricular tachycardia (HR 4.67, 2.07-10.58, P < 0.001). Freedom from tachy- or bradyarrhythmias at 10 and 20 years was 87% (95% CI: 83%-91%) and 72% (95% CI: 66%-79%), respectively. Thromboembolic events occurred in 45 patients (16%, 26 strokes), and freedom from symptomatic thromboembolism at 10 and 20 years was 93% (95% CI: 89%-96%) and 80% (95% CI: 74%-86%), respectively. Over a 25-year period, the LT technique has achieved excellent late survival. As this population ages, it is at an increasing risk of failure and adverse events. We are likely to see an increasing proportion requiring heart transplantation and late reintervention.

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 (20.7%), tricuspid atresia (19.7%), and double inlet left ventricle (17.1%). The mean hospital cost for a Fontan patient from birth to 18 years of age was estimated to be $390,601 (95% confidence interval [CI] $264,703 to $516,499), corresponding to 164 (95% CI 98 to 231) inpatient days. The cost incurred from birth through to Fontan completion (the staged procedures period) was $219,482 (95% CI $202,410 to $236,553) and the cost thereafter over 15 years was $146,820 (95% CI $44,409 to $249,231), corresponding to 82 (95% CI 72 to 92) and 65 (95% CI 18 to 112) inpatient days, respectively. Costs were higher in male and hypoplastic left heart syndrome patients in the staged procedures period (p <0.001). Having fenestration was associated with higher costs in the staged procedures period (p <0.001) and lower cost after Fontan over 15 years (p = 0.66). In conclusion, patients with single ventricle congenital heart disease continue to demand considerable inpatient resources after the staged procedures period. Over 40% of the pediatric hospital costs for Fontan patients were estimated to occur after the last planned surgery.