Search

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., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Crown Copyright © 2021 Published by Elsevier B.V.)

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., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

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., Competing Interests: Competing interests: None declared.

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.

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 ± 9.3 and 14.1 ± 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 m 2 ) in 46/131 (35%) and moderate (mGFR 30-59 ml/min/1.73 m 2 ) 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 (β = 0.89, 95% CI 0.54-1.25, p = 0.002) and decreased mGFR (β = -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., (Copyright © 2018 Elsevier B.V. All rights reserved.)

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.

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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. Inpatient costing records from the birth year through to Fontan completion were available for 156 patients. The most frequent primary diagnoses were hypoplastic left heart syndrome (33%), double inlet left ventricle (13%), and tricuspid atresia (12%)., Results: During the staged surgical treatment period, children had a mean of 10±6 inpatient admissions and spent 85±64days in hospital. Among these admissions, 3±5 were for non-cardiac conditions, totaling 21±41 inpatient days. Whilst cardiac surgeries were the major reason for resource use (77% of the total cost), other cardiac care that is not surgical contributed 5% and non-cardiac admissions 18% of the total cost. The three most prevalent non-cardiac diagnostic admission categories were 'Respiratory system', 'Digestive system', and 'Ear, nose, mouth and throat', affecting 28%, 21% and 34% of the patients respectively. Multivariate regression estimated that admissions for each of these categories resulted in an increased cost of $34,563 (P=0.08), $52,438 (P=0.05) and $10,525 (P=0.53) per patient respectively for the staged surgical treatment period., Conclusions: Non-cardiac admissions for single-ventricle patients are common and have substantial resource implications. Further research assessing the causes of admission and extent to which admissions are preventable is warranted., (Copyright © 2017 Elsevier B.V. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objectives: Echocardiography is a sensitive test for rheumatic heart disease (RHD) screening; however the natural history of RHD detected on screening has not been established. We aimed to evaluate the progression of screening-detected RHD in Fiji., Methods: All young people previously diagnosed with RHD through screening, with echocardiograms available for review, were eligible. All baseline echocardiograms were reported again. Participants underwent follow-up echocardiography. A paediatric cardiologist determined the diagnosis using the World Heart Federation criteria and assessed the severity of regurgitation and stenosis., Results: Ninety-eight participants were recruited (mean age, 17 years; median duration of follow-up, 7.5 years). Two other children had died from severe RHD. Fourteen of 20 (70%) definite RHD cases persisted or progressed, including four (20%) requiring valve surgery. Four (20%) definite RHD cases improved to borderline RHD and two (10%) to normal. Four of 17 (24%) borderline cases progressed to definite RHD (moderate: 2; severe: 2) and two (12%) improved to normal. Four of the 55 cases reclassified as normal at baseline progressed to borderline RHD. Cases with a follow-up interval >5 years were more likely to improve (37% vs 6%, p=0.03)., Conclusions: The natural history of screening-detected RHD is not benign. Most definite RHD cases persist and others may require surgery or succumb. Progression of borderline cases to severe RHD demonstrates the need for monitoring and individualised consideration of prophylaxis. Robust health system structures are needed for follow-up and delivery of secondary prophylaxis if RHD screening is to be scaled up., Competing Interests: None.

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. Medication status and routine follow-up and echocardiographic data were obtained from all patients. Survival analysis with propensity score matching and adjustment was performed to determine the treatment effect of warfarin compared with that of aspirin beyond the first year of follow-up, after which time patients had settled on their long-term thromboprophylaxis strategy., Results: Of 570 eligible patients, the data of 475 patients who were regularly followed up without mechanical valve replacement were available for analysis. Long-term thromboprophylaxis consisted of warfarin in 301 patients (63%), aspirin in 157 (33%) and none in 17 (4%). The 10-year rate of freedom from all thromboembolic events was 91% [95% confidence interval (CI) 88-94%]. Thromboembolic events beyond the first year of follow-up occurred in 18 patients (6 on aspirin and 12 on warfarin). After (i) propensity score adjustment and (ii) matching yielding 164 pairs, the hazard rates of thromboembolic events beyond the first year were not statistically different between the warfarin and aspirin groups [(i) hazard ratio (HR) 2.3, 95% CI 0.7-7.4, P = 0.2 and (ii) HR 1.5, 95% CI 0.5-4.7, P = 0.5, respectively]., Conclusions: No difference in the hazard rates of late thromboembolic events was observed between aspirin and warfarin beyond the first year after the extracardiac conduit Fontan procedure., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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. Projected rates of new Fontan procedures were generated from historical rates of Fontan procedures per population births., Results: At the end of 2014, the living Fontan population of Australia and New Zealand was 1265 people from an Australian and New Zealand regional population of 28 million (4.5 per 100,000 population). Of those, 165 (13%) received an atrio-pulmonary (AP) procedure, 262 (21%) a lateral tunnel (LT) procedure and 838 (66%) an extra-cardiac conduit (ECC) procedure. This population is expected to grow to 1917 (95% CI: 1846: 1986) by 2025 (5.8 per 100,000 population), with 149 (8%) AP procedures, 254 (13%) LT procedures, and 1514 (79%) ECC procedures. By 2045, the living Fontan population is expected to reach 2986 (95% CI: 2877: 3085; 7.2 per 100,000 population). The average age of the Fontan population is expected to increase from 18years in 2014 to 23years (95% CI: 22-23) by 2025, and 31years (95% CI: 30-31) by 2045., Conclusion: The Australian and New Zealand population of patients alive after a Fontan procedure will double over the next 20years increasing the demand for heart-failure services and cardiac transplantation. Greater consideration for the needs of this mostly adult Fontan population will be necessary., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

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., Results: In 2015, 36% of the surviving patients in the Registry (462/1268) were taking an ACE inhibitor. Indications for initiation of therapy were ventricular systolic or diastolic dysfunction (29%), atrioventricular valve regurgitation (19%), preservation of normal ventricular function (7%), prolonged effusions at Fontan (6%), hypertension (6%), other (6%) and unknown (2%). No indication was stated in the remaining patients (25%). Those with hypoplastic left heart syndrome were more likely to be on an ACE inhibitor than those with an alternative primary morphology (70% vs 32%; p<0.001). Only 36% of the patients treated with an ACE inhibitor at last follow up (166/462) had an indication that would generally justify treatment in a two-ventricle circulation., Conclusion: It is likely that the use of ACE inhibitors in patients with a Fontan circulation is excessive within our region. The coordination of prospective, multicentre studies and initiatives such as the Australia and New Zealand Fontan Registry will facilitate further investigations to guide treatment decisions in the growing Fontan population., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Introduction: A high-quality health system should deliver care that is free from harm. Few large-scale studies of adverse events have been undertaken in children's healthcare internationally, and none in Australia. The aim of this study is to measure the frequency and types of adverse events encountered in Australian paediatric care in a range of healthcare settings., Methods and Analysis: A form of retrospective medical record review, the Institute of Healthcare Improvement's Global Trigger Tool, will be modified to collect data. Records of children aged <16 years managed during 2012 and 2013 will be reviewed. We aim to review 6000-8000 records from a sample of healthcare practices (hospitals, general practices and specialists)., 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 the Women's and Children's Hospital Network in South Australia. An application is under review with the Royal Australian College of General Practitioners. The authors will submit the results of the study to relevant journals and undertake national and international oral presentations to researchers, clinicians and policymakers., (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: The life expectancy of patients undergoing a Fontan procedure is unknown., Methods and Results: Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975-1995), 271 lateral tunnels (1988-2006), and 532 extracardiac conduits (1997-2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%-93%) for AP and 97% (95% confidence interval [CI], 94%-99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%-82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P<0.001; 95% CI, 2.4-16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%-76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P<0.001; 95% CI, 2.0-7.1). Ten-year freedom from failure was 79% (95% CI, 61%-89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%-95%) for other morphologies., Conclusions: The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure., (© 2014 American Heart Association, Inc.)

Objectives: To identify factors associated with hospital and long-term outcomes in a binational cohort of extracardiac conduit (ECC) Fontan recipients., Methods: All patients who underwent an ECC Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified, and perioperative, follow-up, echocardiographic and reintervention data collected. Risk factors for early and late mortality, failure and adverse outcomes were analysed., Results: A total of 570 patients were identified, and late follow-up was available in 529 patients. The mean follow-up was 6.7 years (standard deviation: 3.5) and completeness of the follow-up was 98%. There were seven hospital mortalities (1%) and 21 patients (4%) experienced early failure (death, Fontan takedown/revision or mechanical circulatory support). Prolonged length of stay occurred in 10% (57 patients), and prolonged effusions in 9% (51 patients). Overall survival at 14 years was 96% (95% confidence interval [CI]: 93-98%), and late survival for patients discharged with intact Fontan was 98% (95% CI: 94-99%). The rates of late failure (late death, transplantation, takedown, New York Heart Association class III/IV or protein-losing enteropathy) and adverse events (late failure, reoperation, percutaneous intervention, pacemaker, thromboembolic event or supraventricular tachycardia) per 100 patient-years were 0.8 and 3.8, and their 14-year freedoms were 83% (95% CI: 70-91%) and 53% (95% CI: 41-64%), respectively. After adjustment for confounders, hypoplastic left heart syndrome (HLHS) was strongly associated with prolonged effusions (OR: 2.9, 95% CI: 1.4-5.9), late failure (hazard ratio [HR]: 2.8, 95% CI: 1.1-7.5) and adverse events (HR: 3.6, 95% CI: 1.3-7.5)., Conclusions: The extracardiac Fontan procedure provides excellent survival into the second decade of life, but half of patients will suffer a late adverse event by 14 years. Patients with HLHS are at higher risk of late adverse events than other morphological groups, but their survival is still excellent., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: This study examined changes in practice and analyzed risk factors for adverse early outcomes after Fontan surgery through use of a binational, population-based registry., Methods: Demographic, preoperative, and perioperative data were collected from all participating institutions of the Australia and New Zealand Fontan Registry. Patient and operative characteristics were analyzed with multivariable logistic regression for impact on early mortality, early Fontan failure (death, takedown, or mechanical support), effusions (prolonging hospital stay >30 days or requiring surgical reintervention), and stay longer than 30 days., Results: Overall mortality was 3.5% (37/1071) and declined throughout the study period, from 8% (1975-1990) to 4% (1991-2000) and 1% (2001-2010). There were no differences between the extracardiac and lateral tunnel modifications for any outcome. After 2006, the extracardiac conduit was performed exclusively, with 1.3% mortality. The proportion of patients with hypoplastic left heart syndrome rose to 17% in the current era, and this group had more effusions (odds ratio, 3.0; 95% confidence interval, 1.4-6.6) and stayed on average 2 days longer in the hospital. Hypoplastic left heart syndrome was also an independent risk factor for composite adverse early outcome (death, failure, prolonged effusions, or prolonged stay >30 days; odds ratio, 2.6; 95% confidence interval 1.4-4.8 respectively)., Conclusions: The extracardiac conduit is now the exclusive Fontan modification performed in Australia and New Zealand. Even with a higher proportion of high-risk cases, perioperative outcomes are excellent in the modern era. Hypoplastic left heart syndrome confers a higher risk of prolonged pleural effusion and early composite adverse outcome., (Copyright © 2014 The American Association for Thoracic Surgery. All rights reserved.)

Objectives: The study sought to evaluate the late outcomes of a policy of transatrial repair delayed beyond the neonatal period., Background: Long-term outcomes of transatrial repair of tetralogy of Fallot are unknown., Methods: The records of 675 consecutive patients undergoing a transatrial repair of tetralogy of Fallot between 1980 and 2005 were reviewed, their follow-up updated and survival confirmed from national death registries. One-third (220 of 675) had undergone previous palliation. Median age at repair was 2 years in the first 8 years, and 1 year from 1988 onward. A transannular incision was performed in 75% of cases and autologous pericardium was the material used to patch this incision in 92% of cases., Results: There were 7 hospital deaths (1%). Eight patients died during follow-up (2 sudden unexpected and 6 noncardiac deaths). Mean follow-up was 11.7 ± 6.3 years. Twenty-five years' survival was 97% (95% confidence interval [CI]: 95% to 98%). Twenty-five years' freedom from implantation of a valved conduit was 84.6% (95% CI: 77.8% to 89.5%). By multivariable analysis, prior palliation and younger age at repair were predictive of implantation of a valved conduit (hazard ratio: 2.4, 95% CI: 1.3 to 4.6, p = 0.008; hazard ratio: 0.70, 95% CI: 0.50 to 0.96, p = 0.03, respectively)., Conclusions: During long-term follow-up, transatrial repair of tetralogy of Fallot was associated with a minimal risk of sudden death and low rate of reintervention for right ventricular dilation and residual outflow tract obstruction., (Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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., (© 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.)

Objectives: This study sought to compare outcomes after surgical valvuloplasty and balloon dilation of the aortic valve in neonates and infants., Background: Surgical techniques of aortic valve repair have improved and there is today controversy on the best approach to treat neonatal congenital aortic valve stenosis., Methods: Retrospective review of data and follow-up of 123 consecutive neonates and infants (35 females, 88 males) undergoing intervention for congenital aortic stenosis., Results: From 1977 to 2009, 123 consecutive neonates (<30 days) and infants (31 days to 1 year) underwent relief of congenital aortic stenosis. Median age at procedure was 27 days (6 to 76 days). Twenty-year survival was 80 ± 7%. Fifty-four patients required a re-intervention and freedom from re-intervention was 55 ± 6% at 10 years and 40 ± 6% at 20 years. By multivariate analysis, having the relief of stenosis by balloon valvuloplasty and undergoing initial treatment as a neonate were predictive of re-intervention. Freedom from re-intervention at 5 years was 27% after balloon valvuloplasty versus 65% after surgery. At latest follow-up, an additional 16 patients had moderate or severe stenosis and 8 had regurgitation. Freedom from re-intervention or stenosis was 39 ± 5% at 15 years. By multivariate analysis, balloon valvuloplasty (p < 0.001) and treatment as a neonate (p = 0.003) were again predictive of stenosis or re-intervention. Thirty-five patients ultimately needed a valve replacement. Significant predictor of the requirement of valve replacement was unicuspid aortic valve (p < 0.001). Freedom from valve replacement was 55 ± 7% at 20 years., Conclusions: Surgical valvuloplasty remains the best approach to treat neonates and infants with congenital aortic stenosis. After surgery, a higher proportion of patients remain free of re-intervention than after interventional catheterization and the relief of their stenosis lasts longer., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: Late outcomes of repair of coarctation with arch hypoplasia have not yet been described. Hypertension and arch reobstruction frequently occur after standard coarctation repair and thus we sought to determine the long-term results of repair in the subset of patients with arch hypoplasia at a single institution over a 20-year period., Methods: We reviewed the files of the 305 consecutive patients quoted to have arch hypoplasia who had undergone a coarctation repair in a single institution between 1984 and 2004. Repair was performed through a sternotomy in 74 patients (24%), 58 of them undergoing a repair consisting of an end-to-side anastomosis., Results: Early mortality was 9% but there was only 1 death among patients without a major associated anomaly. Eight patients required reintervention before discharge due to residual obstruction. The follow-up was available in 96% of the patients. Only 45% of the patients over 15 years of age had cardiology review in the last 2 years. Survival at 10 and 20 years was 94% (95% CI: 91-97%) and 92% (95% CI: 86-95%), respectively. There were a total of 66 late reinterventions in 49 (18%) patients. Ten- and 20-year freedom from reintervention was 84% (95% CI: 78-88%) and 72% (95% CI: 63-80%), respectively. Ten- and 20-year freedom from reobstruction was 75% (95% CI: 69-80%) and 45% (95% CI: 34-55%), respectively. Patients undergoing end-to-side repair from sternotomy had less arch reobstruction than those undergoing extended end-to-end repair by thoracotomy (92 vs 61% freedom from reobstruction at 10 years, P < 0.001). Only 68% of the patients were normotensive at the last follow-up. Arch obstruction on echocardiogram was associated with an increased prevalence of hypertension (P = 0.018)., Conclusions: After coarctation repair, half of the patients with hypoplasia of the transverse arch will develop arch reobstruction and a third will become hypertensive. The technique of end-to-side repair performed through a sternotomy seems to alleviate these issues, and could be offered to a larger proportion of patients with hypoplasia of the aortic arch. Many of these patients are lost to follow-up during adolescence, at a time when ongoing care seems mandatory.

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: Outcomes after atrioventricular (AV) valve operations in patients with functional single ventricles are unclear., Methods: From 1988 to 2010, 76 consecutive patients with single ventricles underwent AV valve operations for regurgitation at a single institution. Five replacements, 66 repairs, and 5 valve closures were performed at a median age of 1 year (range, 1 day-14 years) on 43 tricuspid, 9 mitral, and 24 common AV valves., Results: Hospital mortality was 17% (13/76). The follow-up was 100% complete. There were 15 late deaths. There were 48 survivors with a mean follow-up of 8.3±6 years. One- and 10-year Kaplan-Meier survival after AV valve operations was 72% (95% confidence interval [CI], 60%-81%) and 61% (95% CI, 48%-71%), respectively. Independent predictors of overall mortality were presence of a common AV valve (p=0.03), requirement for postoperative mechanical circulatory support (p=0.02), and timing of valve operations between initial palliation and performance of a bidirectional cavopulmonary shunt (BCPS) (p=0.047). Ten-year freedom from valve reoperation and from thromboembolic events of hospital survivors was 56% (95% CI, 38%-70%) and 70% (95% CI, 56%-80%), respectively. At last follow-up, 11 of 48 surviving patients (23%) had moderate to severe regurgitation, and pacemaker implantation was required in 6 patients. Only 34 patients reached the stage of Fontan completion., Conclusions: AV valve regurgitation is a considerable burden for the patient with a single ventricle, especially when appearing at a young age. A quarter of patients died within the first year after operation, and they had considerable morbidity in terms of reoperation, thromboembolic events, and pacemaker implantation. Their chances of reaching Fontan completion seemed decreased., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: At present, all colonic polyps are removed and sent for histopathological evaluation, resulting in laboratory and reporting costs. Recent American Society for Gastrointestinal Endoscopy (ASGE) guidelines have set standards for in-vivo diagnosis in place of conventional histopathology, and all future technologies will have to be tested against these standards. Data on flexible spectral imaging color enhancement (FICE) were very limited. This study aims to evaluate the accuracy of FICE and indigo carmine (IC) for in-vivo histology prediction for polyps of less than 10 mm in size and to assess the economic impact of this strategy., Methods: In a screening population, polyps of less than 10 mm were assessed using white light (WLI) by FICE, by IC, and the predicted diagnosis was recorded. Polyps were then removed and sent for histological analysis. Accuracy of the predicted rescope interval was calculated using British Society of Gastroenterology and ASGE guidelines. Two models for using in-vivo diagnosis were proposed and savings in terms of histopathology costs calculated., Results: A total of 232 polyps of less than 10 mm were examined. FICE improved the accuracy of in-vivo diagnosis of adenoma to 88% compared with 75% with WLI (P<0.0001). IC after FICE improved this further to 94%. Rescope interval could be set correctly using FICE or IC in 97% of cases by British Society of Gastroenterology guidelines or 97% with FICE and 99% with IC using ASGE guidelines. A saving of £678,253 (€762767) per annum could be made within the UK national screening population., Conclusion: FICE and IC significantly improves the in-vivo diagnosis of colonic polyps over WLI and can lead to significant cost savings.

Background: The arterial switch operation (ASO) is associated with poorer outcomes in patients with Taussig-Bing anomaly (TBA) compared with transposition of the great arteries (TGA). We describe the outcomes after ASO in patients with TBA at a single institution., Methods: Between 1983 and 2009, 57 patients with TBA underwent the ASO at the Royal Children's Hospital in Melbourne., Results: Hospital mortality was 5.3% (3 of 57). Larger weight at operation (p=0.015), pulmonary artery banding prior to ASO (p=0.049) and concurrent pulmonary artery banding (p=0.049) were risk factors of early death. Actuarial survival was 94% at 15 years. Follow-up was 84% complete with a mean follow-up of 9.8±6.7 years (range, 6 days to 19.1 years). There was no late mortality. Reintervention was required in 24.4% (11 of 45). Longer cross-clamp time (p=0.027) was a risk factor for reintervention. Freedom from reintervention was 75.3% at 15 years. After ASO, 2.2% (1 of 45) presented with sub-neopulmonary obstruction and 13.3% (6 of 45) had moderate or more neoaortic insufficiency (neo-AI). Surgery prior to ASO was a risk factor for sub-neopulmonary obstruction (p=0.049) and moderate or more neo-AI (p=0.016). Freedom from moderate or more neo-AI was 91.1% at 10 years., Conclusions: Early mortality has improved over time with no mortality occurring in the last decade. Although patients are doing well on late follow-up, many patients require reintervention and show progression of neo-AI. Close long-term follow-up is warranted as patients are likely to require further reintervention in the second decade after TBA repair., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To determine the burden of hospitalised, radiologically confirmed pneumonia (World Health Organization protocol) in Northern Territory Indigenous children., Design, Setting and Participants: Historical, observational study of all hospital admissions for any diagnosis of NT resident Indigenous children, aged between > or = 29 days and < 5 years, 1 April 1997 to 31 March 2005., Intervention: All chest radiographs taken during these admissions, regardless of diagnosis, were assessed for pneumonia in accordance with the WHO protocol., Main Outcome Measure: The primary outcome was endpoint consolidation (dense fluffy consolidation [alveolar infiltrate] of a portion of a lobe or the entire lung) present on a chest radiograph within 3 days of hospitalisation., Results: We analysed data on 24,115 hospitalised episodes of care for 9492 children and 13,683 chest radiographs. The average annual cumulative incidence of endpoint consolidation was 26.6 per 1000 population per year (95% CI, 25.3-27.9); 57.5 per 1000 per year in infants aged 1-11 months, 38.3 per 1000 per year in those aged 12-23 months, and 13.3 per 1000 per year in those aged 24-59 months. In all age groups, rates of endpoint consolidation in children in the arid southern region of NT were about twice that of children in the tropical northern region., Conclusion: The rates of severe pneumonia in hospitalised NT Indigenous children are among the highest reported in the world. Reducing this unacceptable burden of disease should be a national health priority.

Objective: To evaluate the efficacy of supplementation with zinc and vitamin A in Indigenous children hospitalised with acute lower respiratory infection (ALRI)., Design: Randomised controlled, 2-by-2 factorial trial of supplementation with zinc and vitamin A., Setting and Participants: 187 Indigenous children aged < 11 years hospitalised with 215 ALRI episodes at Alice Springs Hospital (April 2001 to July 2002)., Interventions: Vitamin A was administered on Days 1 and 5 of admission at a dose of 50 000 IU (infants under 12 months), or 100 000 IU; and zinc sulfate was administered daily for 5 days at a daily dose of 20 mg (infants under 12 months) or 40 mg., Main Outcome Measure: Time to clinical recovery from fever and tachypnoea, duration of hospitalisation, and readmission for ALRI within 120 days., Results: There was no clinical benefit of supplementation with vitamin A, zinc or the two combined, with no significant difference between zinc and no-zinc, vitamin A and no-vitamin A or zinc + vitamin A and placebo groups in time to resolution of fever or tachypnoea, or duration of hospitalisation. Instead, we found increased morbidity; children given zinc had increased risk of readmission for ALRI within 120 days (relative risk, 2.4; 95% CI, 1.003-6.1)., Conclusion: This study does not support the use of vitamin A or zinc supplementation in the management of ALRI requiring hospitalisation in Indigenous children living in remote areas. Even in populations with high rates of ALRI and poor living conditions, vitamin A and zinc therapy may not be useful. The effect of supplementation may depend on the prevalence of deficiency of these micronutrients in the population.

An infant, born at 35 weeks' gestation to a woman who sniffed petrol, had a cord blood lead level eight times the accepted limit. Treatment with oral dimercaptosuccinic acid promptly reduced his blood lead levels. To our knowledge, this is the first reported case of congenital lead poisoning secondary to maternal petrol sniffing. We suggest that at-risk pregnancies should be identified, cord blood lead levels tested, and chelation therapy and developmental follow-up offered to affected infants.

Objective: To evaluate the role of zinc and vitamin A supplementation in the recovery of Indigenous children hospitalised for acute diarrhoea., Design: A randomised controlled 2 by 2 factorial trial of supplementation with zinc and vitamin A., Setting and Participants: Aboriginal children (aged < 11 years) hospitalised for acute diarrhoea at Alice Springs Hospital, Northern Territory, April 2001-July 2002., Main Outcome Measures: Duration of diarrhoeal illness; re-admission for diarrhoeal illness within 120 days., Results: Our study involved 392 Aboriginal children with 436 episodes of diarrhoea. Supplementation with zinc, vitamin A, or combined zinc and vitamin A had no significant effect on duration of diarrhoea or rate of re-admission compared with placebo. Median diarrhoea duration after starting supplementation was 3.0 days for the vitamin A and zinc supplemented and placebo groups (P values 0.25 and 0.69, respectively). The number of re-admissions did not differ significantly between those receiving vitamin A or zinc and the relevant placebo groups (relative risk [95% CI], 1.2 [0.7-2.1] and 1.3 [0.8-2.1], respectively)., Conclusion: Vitamin A and zinc supplementation may not be indicated for in-hospital management of acute diarrhoeal disease in Aboriginal children living in remote areas. This finding may not apply to children with malnutrition, for whom other studies suggest a benefit. Larger trials incorporating more comprehensive data on the vitamin A and zinc status as well as nutritional status of study populations might help to explain the different results in different populations.