Involvement of patients and parents in research undertaken by the Australian and New Zealand Fontan Registry.

Research that is closely connected with the population it is studying and in which the translational value to healthcare is high is a laudable goal, but it is not often achieved. The Australian and New Zealand Fontan Registry has developed a model for involving patients and parents of children with a Fontan circulation in its research. The model involves consumer participation in the overarching Steering Committee, and has set in place multiple channels of communication allowing the early dissemination of research findings before peer-reviewing, and consumer feedback at all levels of the research. Our focus was not only to provide information but also to give a voice to this community and include them as researchers. These communication channels are a part of a larger network involving the practitioner community, support groups, funding agencies, and health authorities. This close connection with the target research population has multiple benefits: safeguarding the project; controlling and adjusting both the messages conveyed and the investigations; building a community; raising new ideas for research; increasing our research participation rate; increasing the weight of our endeavours; and, above all, increasing our own satisfaction in our research undertakings. In conclusion, the interactions with patients and their families within Australia and New Zealand provide one potential model for the involvement of patients and parents that may result in research that is more relevant, focussed, and practically applicable in a healthcare setting.

Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand.

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.

Exploring Australian high-performance athletes' perceptions and experiences of sport participation during pregnancy and post-pregnancy: Development and test-retest reliability of the Mum-Alete Survey.

OBJECTIVES: To develop and assess the test-retest reliability of a survey exploring high-performance athletes' perceptions and experiences during and post-pregnancy. DESIGN: Cross-sectional mixed-methods survey. METHODS: A three-phase approach was employed to develop the Mum-Alete survey. Relevant domains and questions were identified through a review of the literature and gap analysis (Phase 1). The face and content validity were assessed during Phase 2. The survey was modified, and the final survey included 113 questions. The test-retest reliability was assessed during Phase 3. Seven athletes aged ≥18 years who were currently pregnant and/or given birth since 1 July 2016 were recruited. The survey was administered via Qualtrics and completed on two occasions. Intraclass correlation coefficient (ICC) were determined to assess test-retest reliability (excellent, good, moderate, and poor). RESULTS: The average ICC of all items was 0.962 (95% CI 0.957-0.966) demonstrating excellent test-retest reliability. The test-retest reliability was excellent for the demographic and general questions domain (ICC = 0.967 95% CI 0.955-0.977) and good for the exercise (ICC 0.762 95% CI 0.707-0.811), physical health (ICC 0.841 95% CI 0.810-0.868) and well-being (ICC 0.827 95% CI 0.784-0.865) domains. CONCLUSIONS: The high test-retest reliability of the survey indicates excellent consistency of measures between the two time-points.

Medical and surgical management of pediatric perianal crohn's disease: A systematic review.

BACKGROUND: The timely management of pediatric Crohn's disease (CD), and specifically perianal CD, is important owing to the possible adverse effects on growth, development, and quality of life. Perianal involvement is increasingly common, with up to 62% of pediatric CD patients affected. Presently, literature addressing the management of perianal CD has focused primarily on adults, with findings that cannot always be extrapolated to the pediatric population. We aimed to review the rates of healing, recurrence, and need for surgical intervention in perianal CD to provide evidence-based recommendations for the ideal management in children. METHOD: We conducted a systematic review of CENTRAL, PubMed, Medline, and EMBASE databases (January 1997-December 2017) in accordance with PRISMA. Two independent reviewers performed data extraction. RESULT: Ten studies met the inclusion criteria with a combined total of 538 patients. Median study population size was 17 (range 7-276), with a median age at intervention of 13.9 years (range 1-18). Seton placement allowed complete healing in 28.6% of children. Similar results (28.5%) were seen in children undergoing fecal diversion. One study demonstrated complete resolution of fistulizing disease in 70% of children treated with infliximab (IFX). One quarter of patients treated with IFX required further surgical intervention for disease control. Recurrence occurred most frequently in children undergoing Seton placement alone (5/14, 35.7%), compared with IFX (46/197, 23.4%) and combination therapy (12/276, 4.3%). CONCLUSION: In the pediatric population, a combination of medical and surgical treatment is required to control perianal CD, with fewer side effects. LEVEL OF EVIDENCE: Level II.

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

BACKGROUND: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed. METHODS: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions. 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.

Long-term outcomes after first-onset arrhythmia in Fontan physiology.

OBJECTIVES: Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias. METHODS: Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed. We evaluated the incidence and predictors of developing arrhythmias and their prognostic impact on late outcomes. RESULTS: Arrhythmia developed in 195 patients. Tachyarrhythmia was present in 162 patients, bradyarrhythmia was present in 74 patients, and both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia, and bradyarrhythmia was 66% (95% confidence interval [CI], 59-72), 69% (95% CI, 62-75), and 85% (95% CI, 80-90), respectively. On multivariable analyses, patients with an extracardiac Fontan (hazard ratio [HR], 0.23; 95% CI, 0.10-0.51; P < .001) were less likely to develop an arrhythmia, whereas those with left atrial (HR, 3.18; 95% CI, 1.45-6.95; P = .004) and right atrial (HR, 4.00; 95% CI, 2.41-6.61; P < .001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachyarrhythmia or bradyarrhythmia), 10- and 15-year survivals were 74% (65%-83%) and 70% (60%-80%), respectively, and freedom from Fontan failure was 55% (44%-64%) and 44% (32%-56%), respectively. The development of any arrhythmia (HR, 2.20; 95% CI, 1-44-3.34; P < .001), tachyarrhythmia (HR, 2.56; 95% CI, 1.60-4.11; P < .001), and bradyarrhythmia (HR, 1.85; 95% CI, 1.16-2.95; P = .01) were all independent predictors of late Fontan failure on multivariable analyses. CONCLUSIONS: The development of an arrhythmia is associated with a heightened risk of subsequent failure of the Fontan circulation.

Unsatisfactory Early and Late Outcomes After Fontan Surgery Delayed to Adolescence and Adulthood.

The ideal age to perform the Fontan procedure is still unknown. The aim of this study is to determine outcomes after Fontan surgery delayed to adolescence and adulthood in Australia and New Zealand. Patients who had undergone a Fontan procedure at 15 years of age or older were identified in the 1133 patients registered in the Australia and New Zealand Fontan Registry until December 2012. A total of 45 patients underwent the following Fontan procedure at a median age of 18.3 years (16-21 years): 24 atriopulmonary connections, 10 lateral tunnel, and 11 extracardiac conduits. Hospital mortality was 13% (6 of 45). After a mean follow-up of 15.5 ± 9 years, there were 8 late deaths. Survival rates after 10, 20, and 25 years were 79% (95% CI: 64-89), 70% (95% CI: 51-83), and 70% (95% CI: 51-83), respectively. Freedom from Fontan failure (death, heart transplantation, Fontan takedown, protein-losing enteropathy, and poor functional status) after 10 and 20 years was 63% (95% CI: 47-76) and 35% (95% CI: 19-52), respectively. Patients with a single left ventricle had a lower risk of failure (hazard ratio = 0.25, 95% CI: 0.10-0.59; P = 0.002). Arrhythmias developed in 29 patients after a median of 0.1 years (0-9.3 years) and 10 required a permanent pacemaker. Freedom from all adverse events at 10 years was 30% (95% CI: 16-45). Outcomes of the Fontan procedure in adolescents and adults are poor, with disproportionately high hospital mortality and late adverse events. The Fontan procedure should not be delayed to adolescence and adulthood and should be performed electively in childhood.