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Millimeter-sized plastics are numerically abundant and widespread across the world's ocean surface. These buoyant macroscopic particles can be mixed within the upper water column due to turbulent transport. Models indicate that the largest decrease in their concentration occurs within the first few meters of water, where subsurface observations are very scarce. By using a new type of multi-level trawl at 12 sites within the North Atlantic accumulation zone, we measured concentrations and physical properties of plastics from the air-seawater interface to a depth of 5m, at 0.5m intervals. Our results show that plastic concentrations drop exponentially with water depth, but decay rates decrease with increasing Beaufort scale. Furthermore, smaller pieces presented lower rise velocities and were more susceptible to vertical transport. This resulted in higher depth decays of plastic mass concentration (mgm-3) than numerical concentration (piecesm-3). Further multi-level sampling of plastics will improve our ability to predict at-sea plastic load, size distribution, drifting pattern, and impact on marine species and habitats. [ABSTRACT FROM AUTHOR]

The major aim of this study was to evaluate and develop artificial bioremediation systems to reduce or remove metal pollutants from contaminated river water and to decrease the chemical oxygen demand (COD) in distillery effluent. Metals were extracted using the nitric acid digestion method, and the concentrations determined using inductively coupled plasma-atomic emission spectrometry. A decrease in metal concentrations was observed for most of the metals analysed in the river water after being pumped through the bioreactor system for approximately two weeks, e.g. Al concentration decreased from 0.75 mg.l-1 to 0.18 mg.l-1 and for Ni, from 0.19 mg.l-1 to 0 mg.l-1. In addition, the COD counts decreased from 2255 mg.l-1 to a final value of < 150 mg.l-1 in the distillery effluent. It could thus be concluded that the bioreactor system decreased the COD and metal concentrations in the distillery effluent as well as the river water, respectively. A bioreactor is being evaluated on-site at a wine cellar to reduce COD and will be constructed at a site along the Plankenbrug River. [ABSTRACT FROM AUTHOR]

Abstract. The monitoring of vegetation in Southern Africa with satellite data has become increasingly important over the past decade because it is linked to variation in agricultural production and climate change with implications for wildlife management and tourism. This study shows how maps of vegetation status were produced in near real time from NOAA images acquired from the local receiving stations in Etosha National Park, Namibia and in Zambia. Map products based on the NDVI were put into historical context and stratified to remove effects of the main vegetation types in order to assess vegetation status. The historical data were extracted from the FAO ARTEMIS NDVI archive and processed to obtain a statistical distribution of the NDVI for each 10-day period of the year and vegetation type by applying techniques commonly used in hydrology for the prediction of extreme events. The quintile probability ranges were used to define five classes of a Vegetation Productivity Indicator (VPI). LAC NDVI images obtained in real-time from the receiving station were processed to derive a VPI map for each 10-day period. In Etosha National Park and in Zambia, the VPI was strongly related to the rainfall and the VPI maps provided improved information on the spatial variations. The weighted average VPI for the main agricultural region of Zambia was significantly correlated with maize production. [ABSTRACT FROM AUTHOR]

The effect of ozonation on wine distillery wastewater was investigated firstly by monitoring the effect of ozonation on the composition of the wastewater and, secondly, by investigating its effect on the biodegradability of the wastewater. An average COD reduction of 271 mg COD.g O3 -1 was found for wastewater from a distillery pond system. Stable microbial populations, which are found in upflow anaerobic sludge blanket (UASB) granules, were used to determine the toxic effect of wastewater on microbial activity. Granule activity was measured in terms of the rate of biogas and methane production, as well as cumulative biogas volume. Low ozone doses (200 to 400 mg O3.L-1) increased granule activity in terms of biogas, methane production, and cumulative gas volumes. Distillery wastewater reduced the activity of granules, most likely due to the presence of polyphenols and other recalcitrant compounds in the distillery wastewater. [ABSTRACT FROM AUTHOR]

Background: The international education sector is important not only to Australian society, but also to the national economy. There are growing concerns about the potential wellbeing challenges facing international students in their host country, owing to acculturative stress; including loneliness, isolation and experiences of racism. Risks include poor mental health and decreased likelihood to access support due to stigma, language and cultural barriers, not knowing where to seek help, and poor mental health knowledge., Methods: This study explored students' perceptions of their accommodation, subjective wellbeing (through the Personal Wellbeing Index, ['PWI']), mental health help-seeking and individual engagement with evidence-based everyday health promotion actions (informed by the '5 Ways to Wellbeing' model) through an online survey (N = 375) and three online focus groups (N = 19). A mixed-methods approach using descriptive statistics, ANOVA, regression analysis and thematic analysis, were used., Results: The PWI of international students in the survey was observed to be substantially lower (M = 60.7) than that reported for the Australian population (M = 77.5). Accommodation impacted on wellbeing (loneliness, belonging, connectedness) in a number of different ways including through location, safety, and shared accommodation. In terms of help-seeking, international students noted a number of barriers to accessing support for mental health: cost of accessing support, language and cultural barriers, lack of information on where to find support and stigma., Conclusions: In the discussion, implications of the findings are considered, including that more could be done to shape policy and practice in service and facility provision around wellbeing, connectedness, and help-seeking for mental health support of international students., (© 2024. The Author(s).)

Aim: The study sought to develop an understanding of Australian first-year university residential college students' alcohol consumption, their experience of alcohol-related harms and their alcohol knowledge. Method: Students were surveyed during Orientation Week in 2015 ( N  = 84, men 36%) and again in 2017 ( N  = 97, men = 45%) using the Alcohol Use Disorders Identification Test (AUDIT) to measure alcohol consumption, and purpose-designed measures of alcohol-related harms and alcohol knowledge. Results: The mean AUDIT score across the two cohorts was 10.79, placing these first-year college residents at much riskier consumption levels than their Australian undergraduate and international peers. Three-quarters were consuming alcohol at hazardous/harmful levels. They reported frequent occurrence of alcohol-related harms and, given the higher levels of drinking, these were for the most part more pronounced than in other studies: vomiting (73%), memory loss (55%), regretting their actions when drinking (41%), not having enough money because of money spent on alcohol (31%), doing something dangerous just for fun (29%), being injured (27%), poor performance at work (22%), poor physical health (21%), loss of consciousness (20%), and having sexual encounters they later regretted (19%). Poor knowledge of standard drink measures, particularly in relation to blood alcohol concentration, was also indicated. Conclusions: The study highlights the worrying occurrence of hazardous/harmful drinking in Australian first-year university residential college students and high levels of alcohol-related harms experienced by these residents. It also highlights poor alcohol knowledge and the need for early intervention prior to and within university college residences to minimise harm., Competing Interests: Declaration of conflicting interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2022.)

Introduction: It is unknown if the Fontan circulation has an impact on sexual health in men. This study assessed self-reported sexual health and fertility in men with a Fontan circulation. Aims: In this prospective, cross-sectional study, Australian men ≥18 years enrolled in the Fontan Registry of Australia and New Zealand were invited to complete the International Index of Erectile Function (IIEF), alongside questions assessing fertility. These data were compared to historical, age-matched controls. Results: Of 227 eligible men, 54 completed the survey; of those 37 were sexually active and included in the final analysis. Mean age was 28 ± 3 years, age at Fontan was 5 ± 3 years. Fontan type was extra-cardiac conduit in 15 (41%), lateral tunnel in 12 (32%), and atriopulmonary connection (APC) in 10 (27%). Ventricular function was normal in 24 (83%), and all were New York Heart Association Class I (23 patients, 79%) and II (six patients, 21%). Nine participants (24%) had erectile dysfunction (IIEF-EF score ≤25). The severity was mild (IIEF 22-24) in six (16%), mild-moderate (IIEF 17-21) in two (5%), and moderate (IIEF 11-16) in one (3%). Baseline characteristics and current medication usage were similar in those with and without erectile dysfunction. Compared with historical control values, erectile function was not significantly impaired in the Fontan population ( p =0.76). Men with a Fontan circulation had decreased levels of sexual desire and overall satisfaction ( p < 0.001). There was no correlation between the presence of erectile dysfunction and any assessed parameter. Eleven (30%) of the cohort reported a pregnancy with a prior partner. Conclusion: In our cohort, overall erectile function was comparable between men with a Fontan circulation and historical controls, however sexual desire and overall satisfaction were reduced. There was no correlation between study parameters and the presence of erectile dysfunction. The proportion of the cohort who had a prior pregnancy was congruent with population data., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Rubenis, Tran, Bullock, Wijesekera, Baker, d'Udekem, du Plessis, Katz, Lowy, Zentner, Celermajer and Cordina.)

African savannah elephants ( Loxodonta africana ) are well-known as ecosystem engineers with the ability to modify vegetation structure. The present study aimed to examine how male elephant foraging behaviour is affected across (a) season (wet versus dry); (b) time of day (before or after noon); (c) presence or absence of other elephants; and (d) reproductive state (musth versus no musth). Six radio-collared adult elephant bulls were observed twice per week from June 2007-June 2008 in Kruger National Park (KNP), South Africa. Using generalized linear mixed effect modeling, results indicate that elephant bulls graze more during the wet season and browse more during the dry season. To potentially offset the costs associated with thermoregulation during the heat of the day, KNP elephants spent more time foraging during the morning, and more time resting during the afternoon. Male elephants also foraged significantly less when they were associated with females compared to when they were alone or with other males. This is likely due to male-female associations formed mainly for reproductive purposes, thus impeding on male foraging behaviours. In contrast, the condition of musth, defined by the presence of related physical signs, had no significant effect on foraging behaviour.

Background: In people with a Fontan circulation, serial cardiopulmonary exercise testing (CPET) to evaluate change in peak exercise capacity has been increasingly recognised as a useful prognostic tool; a decline is associated with adverse clinical outcomes. The aim of this study is to describe the "natural" history of exercise capacity in the Australian and New Zealand (ANZ) Fontan cohort and to identify factors associated with a decline., Methods: The ANZ Fontan registry was retrospectively reviewed for adolescent and adult patients (≥16 years) with serial CPET results performed on a cycle ergometer ≥6 months apart. Patients were excluded if they underwent a surgical procedure or fenestration closure in-between tests or if the tests were considered as submaximal effort. Exercise capacity trajectory was defined as the change in percentage of predicted peak oxygen uptake (% pred VO 2peak ) points per year., Results: Thirty-seven (37) patients (59.5% male, mean age 24±7 years) were eligible. Average duration between CPET was 5.3±3.9 years. At baseline, % pred VO 2peak was 61.3±14.5%. Thirteen (13) (35%) had a systemic right ventricle, and 14 (38%) had an atriopulmonary type Fontan circulation. Average change in % pred VO 2peak overall was +1.3±6.4 percentage points per year. Sixteen (16) had a negative exercise capacity trajectory, and the average decline in that group was -2.7±3.4 percentage points per year. There was no association between exercise capacity trajectory and clinical characteristics. Of the 18 patients with physical activity levels recorded, 12 (67%) were physically active and % pred VO 2peak in that group increased by 2.7±4.0 percentage points per year compared with the physically inactive group who fell by 0.5±0.8 percentage points per year., Conclusions: In this ANZ series of Fontan patients, over half of our cohort had stable, or an increase, in peak exercise capacity. Regular participation in physical activity was common in patients with a positive exercise capacity trajectory. Clinical characteristics were not associated with exercise capacity trajectory., (Copyright © 2021 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: Fontan-associated liver disease is accompanied by a hypercoagulable state. While hepatic dysfunction in Fontan patients is common, its relationship with haemostatic changes and clinical outcomes in this patient population remains unclear., Objective: To correlate liver dysfunction and haemostatic profiles with clinical outcomes in the Fontan population., Patients/methods: Patients were enrolled in a multicentre, cross-sectional study in Australia and New Zealand. Hepatic structure and function were assessed using serum-based calculations (Fibrotest and model for end-stage liver disease excluding international normalised ratio scores). Haemostatic profiles were assessed by Thrombin Generation. Platelet function was assessed via Platelet Factor 4 (PF4) and P-selectin (P-SEL). Clinical outcomes were obtained from the Australian and New Zealand Fontan Registry., Results: Seventy-three patients participated in the study (mean age 18.9±8.5 years with a mean of 13.5±6.9 years post-Fontan). The Endogenous Thrombin Potential (ETP) for patients who suffered thrombotic events (TE) (1366.4±66.2 nM/min) was higher compared with patients with major bleeding events (1011.1±138.4 nM/min) (p=0.03). Except for a negative correlation between Fibrotest-score and PF4 (p=0.045), PF4 and P-SEL concentrations did not correlate with markers of hepatic dysfunction or structural abnormality., Conclusions: Increased ETP is associated with TE during clinical follow-up after Fontan. This study reinforces that hepatic dysfunction may contribute to the derangement of coagulation factors, impacting the individual risk of haemostatic complications for the Fontan population., Competing Interests: Competing interests: YD is consultant for MSD and Actelion. The remaining authors have no conflicts of interest to declare., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Neurocognitive outcomes beyond childhood in people with a Fontan circulation are not well defined. This study aimed to investigate neurocognitive functioning in adolescents and adults with a Fontan circulation and associations with structural brain injury, brain volumetry, and postnatal clinical factors., Methods: In a binational study, participants with a Fontan circulation without a preexisting major neurological disability were prospectively recruited from the Australia and New Zealand Fontan Registry. Neurocognitive function was assessed by using Cogstate software in 107 participants with a Fontan circulation and compared with control groups with transposition of the great arteries (n=50) and a normal circulation (n=41). Brain MRI with volumetric analysis was performed in the participants with a Fontan circulation and compared with healthy control data from the ABIDE I and II (Autism Brain Imaging Data Exchange) and PING (Pediatric Imaging, Neurocognition, and Genetics) data repositories. Clinical data were retrospectively collected., Results: Of the participants with a Fontan circulation who had a neurocognitive assessment, 55% were male and the mean age was 22.6 years (SD 7.8). Participants with a Fontan circulation performed worse in several areas of neurocognitive function compared with those with transposition of the great arteries and healthy controls ( P <0.05). Clinical factors associated with worse neurocognitive outcomes included more inpatient days during childhood, younger age at Fontan surgery, and longer time since Fontan procedure ( P <0.05). Adults with a Fontan circulation had more marked neurocognitive dysfunction than adolescents with a Fontan circulation in 2 domains (psychomotor function, P =0.01 and working memory, P =0.02). Structural brain injury was present in the entire Fontan cohort; the presence of white matter injury was associated with worse paired associate learning ( P <0.001), but neither the presence nor severity of infarct, subcortical gray matter injury, and microhemorrhage was associated with neurocognitive outcomes. Compared with healthy controls, people with a Fontan circulation had smaller global brain volumes ( P <0.001 in all regions) and smaller regional brain volumes in most cerebral cortical regions ( P <0.05). Smaller global brain volumes were associated with worse neurocognitive functioning in several domains ( P <0.05). A significant positive association was also identified between global brain volumes and resting oxygen saturations ( P ≤0.04)., Conclusions: Neurocognitive impairment is common in adolescents and adults with a Fontan circulation and is associated with smaller gray and white matter brain volume. Understanding modifiable factors that contribute to brain injury to optimize neurocognitive function is paramount.

Objective: Fontan takedown remains an option for the management of Fontan failure. We sought to evaluate early and late outcomes after Fontan takedown., Methods: The Australia and New Zealand Fontan Registry was interrogated to identify all patients who had a Fontan takedown., Results: Over a 43-year study period (1975-2018), 36 of 1540 (2.3%) had a Fontan takedown. The median age at takedown was 5.1 years (interquartile range [IQR], 3.7, 7.0). Nine (25%) patients had a takedown within 48 hours, 6 (16%) between 2 days and 3 weeks, 14 (39%) between 3 weeks and 6 months, whereas 7 (19%) had a late takedown (>6 months). Median interval to takedown was 26 days (IQR, 1.5, 127.5). Sixteen (44%) patients died at a median of 57.5 days (IQR, 21.8, 76.8). The greatest mortality occurred between 3 weeks and 6 months (<2 days: 1/9, 11%; 2 days to 3 weeks: 2/6, 33%; 3 weeks to 6 months: 11/14, 79%; >6 months: 2/7, 28%; P = .007). At median follow-up of 9.4 years (IQR, 4.5, 15.3), 11 (31%) patients were alive with an intermediate circulation (10 in New York Heart Association class I/II). Five (14%) patients underwent a successful second Fontan. Freedom from death/transplant after Fontan takedown was 59%, 56%, and 52% at 1, 5, and 10 years, respectively., Conclusions: The incidence of Fontan takedown is low, but mortality is high. The majority of takedowns occurred within 6 months. Mortality was lowest when takedown occurred <2 days and highest between 3 weeks and 6 months. A second Fontan is possible in a small proportion of survivors., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Advances in omics and specifically genomic technologies are increasingly transforming rare disease diagnosis. However, the benefits of these advances are disproportionately experienced within and between populations, with Indigenous populations frequently experiencing diagnostic and therapeutic inequities. The International Rare Disease Research Consortium (IRDiRC) multi-stakeholder partnership has been advancing toward the vision of all people living with a rare disease receiving an accurate diagnosis, care, and available therapy within 1 year of coming to medical attention. In order to further progress toward this vision, IRDiRC has created a taskforce to explore the access barriers to diagnosis of rare genetic diseases faced by Indigenous peoples, with a view of developing recommendations to overcome them. Herein, we provide an overview of the state of play of current barriers and considerations identified by the taskforce, to further stimulate awareness of these issues and the passage toward solutions. We focus on analyzing barriers to accessing genetic services, participating in genomic research, and other aspects such as concerns about data sharing, the handling of biospecimens, and the importance of capacity building., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2020 D'Angelo, Hermes, McMaster, Prichep, Richer, van der Westhuizen, Repetto, Mengchun, Malherbe, Reichardt, Arbour, Hudson, du Plessis, Haendel, Wilcox, Lynch, Rind, Easteal, Estivill, Thomas and Baynam.)

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.)

Objectives: The mechanisms of attrition of the Fontan population have been poorly characterised and it is unclear whether some of the deaths are potentially preventable. We analysed the circumstances of late death in patients with a Fontan circulation, with a special focus on identifying lesions amenable to intervention that may have contributed to the decline of their circulation., Methods: Between 1975 and 2018, a total of 105 patients from a Bi-National Registry died beyond 1 year after Fontan completion, at a median age of 18.6 (IQR 13.8-26.0) years old, 12.7 (IQR 6.0-19.3) years after Fontan completion., Results: A total of 105 patients died-63 patients (60%) with an atriopulmonary (AP) Fontan, 21 patients (20%) with a lateral tunnel (LT) and 21 patients (20%) with an extracardiac conduit (ECC). 72 patients (69%) were reviewed within 2 years preceding death, with 32% (23/72) deemed to be clinically well. Fontan circulatory failure was the most common cause of death in 42 patients (45%). Other causes of death included sudden death/arrhythmia (19%), perioperative death (12%), neurological complication (7%) and thromboembolism (7%). All patients with an LT or ECC who died from Fontan failure had at least one surgical defect that was amenable to intervention at time of death., Conclusions: Conventional clinical surveillance has been insensitive in detecting a significant proportion of patients at risk of late death. Fontan circulatory failure contributes to half of the late deaths. Patients with an LT or ECC Fontan who died with a clinical picture of circulation failure may have potentially correctable lesions., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Heterotaxy is considered a risk factor for poor outcomes after the Fontan operation. However, long-term data to support this notion are lacking. The aims of this study were to ascertain the long-term outcomes of patients with heterotaxy after hospital discharge after Fontan completion and to compare these outcomes with those of a contemporary nonheterotaxy cohort., Methods: A binational Fontan registry (n = 1540) was analyzed to identify patients with heterotaxy and compare them with patients without heterotaxy. The primary composite end point was Fontan failure, encompassing death, heart transplantation, Fontan takedown or conversion, protein-losing enteropathy, plastic bronchitis, or New York Heart Association functional class III or IV., Results: A total of 109 patients with heterotaxy were identified and they were compared with 1431 nonheterotaxy patients after Fontan completion. There was no difference in unadjusted 15-year freedom from Fontan failure (heterotaxy, 78% vs nonheterotaxy, 85%; P = .2). Patients in the heterotaxy group had a significantly higher cumulative incidence of post-Fontan arrhythmias (P < .001). Propensity-score matching for confounders yielded 73 patients with heterotaxy matched with 439 patients without heterotaxy, in whom 15-year freedom from Fontan failure was also not different (heterotaxy, 76% vs nonheterotaxy, 81%; P = .2). There was no difference in 15-year freedom from Fontan failure in patients with right vs left isomerism (right isomerism, 80% vs left isomerism, 76%; P = .7)., Conclusions: Although heterotaxy may complicate the pre-Fontan course, once the Fontan procedure is successfully completed, heterotaxy does not appear to be an important risk factor for Fontan failure. Patients with heterotaxy are at a higher risk of post-Fontan arrhythmias compared with patients without heterotaxy., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients., Methods: A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival., Results: Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%., Conclusions: A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Moderate to severe neurodevelopmental impairments are recognized as a significant comorbidity in one-fourth to one-third of single-ventricle patients. This report reviews the nature of neurodevelopment across the lifespan, contributing factors, and interventions., Methods: A focused review was performed of all articles in English that address the topic from 1980 to 2018., Results: Innate patient factors, rather than intraoperative factors, affect the development of neurodevelopmental outcomes, yet a large portion of causality factors remains unexplained. There are also limits to how we currently measure neurodevelopmental outcomes. Limited studies in adulthood and a lack of longitudinal studies hamper our full understanding of how neurodevelopment progresses across time, but estimates are that adults with neurodevelopmental impairments will be affected by abnormal brain aging and early-onset dementia. Intervention services and their efficacy have not been researched in depth and have not been researched across the lifespan., Conclusions: Further research into neurodevelopmental testing that accurately predicts future functioning is needed. The complexity of implementing systematic and effective neurodevelopmental interventions in the busy lives of patients and families, who often have multiple other clinical demands, needs a complex and thoughtful solution within the context of a multidisciplinary care team approach. The role of targeted psychological and educational interventions that decrease maternal worry and improve family functioning should also continue to be explored in patients with a Fontan circulation., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Few patients with total anomalous pulmonary venous drainage (TAPVD) and a univentricular circulation survive to Fontan completion. Hence, we sought to determine the long-term outcomes of the Fontan operation in patients with TAPVD., Methods: Patients with TAPVD who underwent the Fontan operation and survived to hospital discharge in Australia and New Zealand between 1985 to 2017 were identified (n = 54) from a binational Fontan registry., Results: Thirty-two patients (60%) underwent repair of TAPVD at a median age of 0.8 (interquartile range: 0.3-1.6) years. Thirty-seven patients (69%) had heterotaxy. The median age at time of Fontan operation was 5.7 years. There were 4 late deaths and 3 patients required cardiac transplantation for a failing Fontan circulation. On univariate analysis, the concomitant diagnosis of pulmonary stenosis and right ventricular dominance was associated with late death or transplantation (P = .04). Freedom from late death or transplantation at 15 years after the Fontan operation was 88% ± 7% (95% confidence interval [CI], 67%-96%) for the repaired TAPVD group and 90% ± 6% (95% CI, 67%-98%) for the unrepaired TAPVD group (P = .47). Median follow-up after the Fontan procedure was 10.8 (interquartile range, 6.7-16.2) years. The majority of survivors (94%) were in New York Heart Association functional class I or II. The 15-year freedom from death or transplantation was similar for patients with TAPVD (89% ± 5%; 95% CI, 76%-95%) compared with patients without TAPVD in the Fontan registry (n = 1446; 92% ± 1%; 95% CI, 90%-93%) (P = .12)., Conclusions: Long-term survival of patients with TAPVD who undergo the Fontan operation and survived to hospital discharge is comparable to Fontan survivors without TAPVD., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The need for permanent pacing has been identified as a predictor of poor outcomes in the late survivors of Fontan surgery. However, it is not clear if the need for a pacemaker is a surrogate marker of a declining Fontan state, or if pacing is deleterious to the Fontan circulation., Objectives: We sought to compare the long-term outcomes of propensity-matched Fontan patients with and without a permanent pacemaker., Methods: Patients who have survived Fontan completion with a documented history of cardiac arrhythmia were identified from the Australia and New Zealand Fontan Registry. Pacemaker insertion details, cardiac function and electrophysiological data were obtained for the patients with a permanent pacemaker. Survival analysis was performed with propensity score matching to compare late survival and outcomes in patients with versus without a pacemaker., Results: There was a total of 310 patients with a history of cardiac arrhythmia, of which 126 (41%) had a permanent pacemaker. After propensity-score matching, 99 pairs were generated (n = 198). Patients with a permanent pacemaker had a higher risk of death (HR 3.32 95% CI 1.60-6.90, p = 0.001) and death or transplantation (HR 3.55 95% CI 1.87-6.73, p < 0.001). Patients who were only paced atrially were not at a significantly increased risk of death or transplantation. However, patients who were ventricular paced >50% of the time were much more likely to encounter late death or transplantation (HR 3.82 95% CI 1.64-8.95, p = 0.002)., Conclusions: Having a permanent pacemaker and needing ventricular pacing is likely associated with an increased risk of death and transplantation in patients with a Fontan circulation., (Crown Copyright © 2019. Published by Elsevier B.V. All rights reserved.)

Background: A subset of patients who underwent Fontan operations has two adequate-sized ventricles, but an anatomic biventricular circulation cannot be achieved because of complex morphology or for technical reasons. This study sought to determine whether these patients with two-ventricle Fontan circulation had superior outcomes compared with those with a single ventricle., Methods: A binational Fontan Registry of patients (n = 1,377) was analyzed to identify those patients with two adequate ventricles. This cohort was compared with patients with single-ventricle Fontan circulation. The primary end point was a composite end point called "Fontan failure" encompassing death, heart transplantation, Fontan takedown or conversion, protein-losing enteropathy, plastic bronchitis, or New York Heart Association functional class III or IV., Results: A total of 79 Fontan patients with two adequate ventricles (2V) were compared with 1,291 single ventricle (1V) Fontan patients. Median follow-up for the entire cohort was 11.5 years (interquartile range, 5.1 to 18.8 years). There was no difference in unadjusted 15-year freedom from Fontan failure (2V: 81% [95% confidence interval (CI), 69% to 94%] vs 1V: 86% [95% CI, 83% to 88%], p = 0.4). Propensity-score matching for potential confounding factors yielded 75 two-ventricle Fontan patients matched with 604 single-ventricle Fontan patients, in which 15-year freedom from Fontan failure was also not different (2V: 79% [95% CI, 67% to 94%] vs 1V: 87% [95% CI, 84% to 91%], p = 0.3)., Conclusions: The two-ventricle Fontan circulation does not have better outcomes compared with the single-ventricle Fontan circulation. Late outcomes may depend more on other characteristics of the Fontan circulation. This finding is relevant when the Fontan procedure is being considered as an alternative to anatomic repair in patients with complex two-ventricle morphologies., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.)

Background: Exercise training has been shown to increase exercise capacity in survivors of Fontan surgery. The geographic distribution of the Fontan population has been a barrier to hospital-based exercise training programmes. The objective of this study was to establish whether a home exercise training programme could achieve similar improvements to a hospital programme., Methods: Adolescents with a Fontan circulation aged 12-19 years were prospectively recruited in a hospital or home exercise training programme. Patients underwent cardiopulmonary exercise testing and completed the Paediatric Quality of Life Inventory at initial assessment and after completion of an 8-week programme. Both groups performed two 1-hour training sessions per week. Patients in the home training programme had their first session in the hospital, and then progressed independently with one phone consult per week and one home visit by a physiotherapist., Results: In total, 17 patients, with a mean age of 15±3 years, completed the training programme (six hospital). Characteristics and baseline performance of patients were similar in both groups. Oxygen consumption at anaerobic threshold increased from 19.3±3.8 to 21.6±6.0 ml/kg/minute (p=0.02) and peak oxygen pulse increased from 8.8±2.5 to 9.5±2.7 ml/beat (p=0.049). Total quality of life scale improved from 68 to 74% (p=0.01) and psychosocial health improved from 67 to 74% (p=0.02). No patient experienced training-related complications., Conclusions: Exercise training is beneficial and most likely safe after Fontan, resulting in improved exercise capacity and self-reported quality of life. Home exercise training programmes are probably as effective as hospital programmes. Home exercise training programmes should be integrated in the follow-up care of patients undergoing Fontan surgery.

Objectives: This study sought to describe atrial arrhythmia mechanisms, acute outcomes, and long-term arrhythmia burdens following catheter ablation in adult atriopulmonary (AP) Fontan patients., Background: Atrial arrhythmias are a significant cause of morbidity and mortality in the AP Fontan population., Methods: Sixty consecutive atrial arrhythmia ablations were reviewed in 42 AP Fontan patients (31 ± 8 years of age), performed between 1998 and 2017. The number of induced and ablated tachycardias was recorded for each case, as well as the ability to ablate the suspected clinical tachycardia. Longer-term arrhythmia burden was assessed by using a 12-point clinical arrhythmia severity score., Results: Intra-atrial re-entrant tachycardia (IART) was induced in 93% of cases (n = 56), atrioventricular re-entrant tachycardia in 2 (3%) and atrioventricular nodal re-entrant tachycardia in a single case. The mean number of tachycardias induced per case was 2.3. The critical isthmus for IART was mapped to the lateral (n = 10), inferolateral (n = 8), posterior/posterolateral (n = 16), or septal (n = 10) systemic venous atrium, or to the pulmonary venous atrium (n = 4). Ablation of all inducible tachycardias was achieved in 62%, ablation of at least one (but not all) inducible tachycardias in 25%, with failure to ablate any tachycardias in 13%. The suspected clinical arrhythmia was ablated in 50 cases (83%). Catheter ablation resulted in a significant reduction in arrhythmia score at 3 to 6, 12, and 24 months, irrespective of whether all inducible tachycardias were ablated, or the suspected clinical arrhythmia only. Twelve patients (29%) underwent at least one repeat ablation procedure, with a mean time between ablations of 2.7 ± 3.0 years. There were no cases of periprocedural death, stroke or cardiac tamponade., Conclusions: Catheter ablation can be a safe and effective intervention that will significantly reduce arrhythmia burden in the AP Fontan patient., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)