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Objective: To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes., Study Design: Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures. Clinical data were provided by the Australia and New Zealand Fontan Registry., Results: Participants (mean age: 11.5 ± 2.6 years, 62% male) reported lower overall HRQOL (P < .001), and lower scores across all HRQOL domains (all P < .0001), compared with normative data. Median global QOL score was 7.0 (IQR 5.8-8.0), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of congenital heart disease explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes., Conclusions: Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population., Competing Interests: Declaration of Competing Interest This work was supported by HeartKids Australia and Australian National Health and Medical Research Council (NHMRC) Project (APP1065794) and Partnership (APP1076849) Grants. The authors have no conflicts of interest to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: To characterize global and health-related quality of life (QOL) among adults with Fontan physiology enrolled in the Australian and New Zealand Fontan Registry (ANZFR), and identify sociodemographic, clinical, psychological, and relational factors associated with outcomes., Methods and Results: Using a cross-sectional survey design, 66 adults with Fontan physiology (58% women; mean age, 29.6±7.7 years; range, 18-50 years) completed validated self-report measures. Health-related QOL was assessed using the Pediatric Quality of Life Inventory, and global QOL was assessed using a visual analog scale (0-10). Participants reported lower total health-related QOL ( P <0.001), as well as lower physical ( P <0.001) and social ( P =0.002) functioning compared with normative data. Median global QOL was 7.0 (interquartile range: 5.0-8.0) and most participants (71%) rated their QOL ≥6. For health-related QOL, age, sex, university education, and length of hospital stay in the past 12 months explained 27% of the variance in scores, while general psychological stress, medical traumatic stress, communication problems, and access to emotional support explained a further 44% of variance (final model: 71% of variance explained). For global QOL, sociodemographic and clinical factors explained 20% of the variance in scores, while psychological stress and sense of coherence explained a further 24% (final model: 44% of variance explained)., Conclusions: Adults with Fontan physiology reported lower overall health-related QOL compared with community-based norms. Variance in QOL outcomes were predominantly attributable to psychological and relational factors. Tailored screening and assessment to identify Fontan patients at greatest risk of lower QOL, and a proactive approach to supportive care, are needed.

In recent issues of the Journal of the Society for Cardiovascular Angiography and Interventions and the Journal of the American College of Cardiology: Cardiovascular Interventions , Holzer and colleagues presented an Expert Consensus Document titled: " PICS / AEPC / APPCS / CSANZ / SCAI / SOLACI: Expert consensus statement on cardiac catheterization for pediatric patients and adults with congenital heart disease. " This Expert Consensus Document is a massively important contribution to the community of paediatric and congenital cardiac care. This document was developed as an Expert Consensus Document by the Pediatric and Congenital Interventional Cardiovascular Society, the Association for European Paediatric and Congenital Cardiology, the Asia-Pacific Pediatric Cardiac Society, the Cardiac Society of Australia and New Zealand, the Society for Cardiovascular Angiography and Interventions, and the Latin American Society of Interventional Cardiology, as well as the Congenital Cardiac Anesthesia Society and the American Association of Physicists in Medicine.As perfectly stated in the Preamble of this Expert Consensus Document, "This expert consensus document is intended to inform practitioners, payors, hospital administrators and other parties as to the opinion of the aforementioned societies about best practices for cardiac catheterisation and transcatheter management of paediatric and adult patients with congenital heart disease, with added accommodations for resource-limited environments." And, the fact that the authorship of this Expert Consensus Document includes global representation is notable, commendable, and important.This Expert Consensus Document has the potential to fill an important gap for this patient population. National guideline documents for specific aspects of interventions in patients with paediatric heart disease, including training guidelines, do exist. However, this current Expert Consensus Document authored by Holzer and colleagues provides truly globally applicable standards on cardiac catheterisation for both paediatric patients and adults with congenital heart disease (CHD).Our current Editorial provides different regional perspectives from senior physicians dedicated to paediatric and congenital cardiac care who are practicing in Europe, the Asia-Pacific region, Latin America, Australia/New Zealand, and North America. Establishing worldwide standards for cardiac catheterisation laboratories for children and adults with CHD is a significant stride towards improving the quality and consistency of care. These standards should not only reflect the current state of medical knowledge but should also be adaptable to future advancements, ultimately fostering better outcomes and enhancing the lives of individuals affected by CHD worldwide.Ensuring that these standards are accessible and adaptable across different healthcare settings globally is a critical step. Given the variability in resources and infrastructure globally, the need exists for flexibility and tailoring to implement recommendations.The potential impact of the Expert Consensus Document and its recommendations is likely significant, but heterogeneity of healthcare systems will pose continuing challenges on healthcare professionals. Indeed, this heterogeneity of healthcare systems will challenge healthcare professionals to finally close the gap between acceptable and ideal in the catheterisation of patients with paediatric and/or congenital heart disease.

Aim: To identify and map evidence describing components of neurodevelopmental follow-up care for children with congenital heart disease (CHD)., Method: This was a scoping review of studies reporting components of neurodevelopmental follow-up programmes/pathways for children with CHD. Eligible publications were identified through database searches, citation tracking, and expert recommendations. Two independent reviewers screened studies and extracted data. An evidence matrix was developed to visualize common characteristics of care pathways. Qualitative content analysis identified implementation barriers and enablers., Results: The review included 33 studies. Twenty-one described individual care pathways across the USA (n = 14), Canada (n = 4), Australia (n = 2), and France (n = 1). The remainder reported surveys of clinical practice across multiple geographical regions. While heterogeneity in care existed across studies, common attributes included enrolment of children at high-risk of neurodevelopmental delay; centralized clinics in children's hospitals; referral before discharge; periodic follow-up at fixed ages; standardized developmental assessment; and involvement of multidisciplinary teams. Implementation barriers included service cost/resourcing, patient burden, and lack of knowledge/awareness. Multi-level stakeholder engagement and integration with other services were key drivers of success., Interpretation: Defining components of effective neurodevelopmental follow-up programmes and care pathways, along with enhancing and expanding guideline-based care across regions and into new contexts, should continue to be priorities., What This Paper Adds: Twenty-two different neurodevelopmental follow-up care pathways/programmes were published, originating from four countries. Twelve additional publications described broad practices for neurodevelopmental follow-up across regions Common attributes across eligibility, service structure, assessment processes, and care providers were noted. Studies reported programme acceptability, uptake, cost, and effectiveness. Implementation barriers included service cost/resourcing, patient burden, and lack of knowledge/awareness., (© 2023 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.)

Background: There is a paucity of contemporary information regarding the prevalence and related health care expenditure of the most common cardiac conditions in Australian children, including congenital heart disease (CHD)., Methods: The 10 most common cardiac conditions managed by Queensland Paediatric Cardiac Service during 2014-2015 were identified from an electronic database: ventricular septal defect (VSD), pulmonary stenosis, aortic stenosis, tetralogy of Fallot, atrioventricular septal defect, transposition, Ebstein's anomaly, long QT syndrome, dilated cardiomyopathy, and rheumatic carditis. Demographic data, clinic attendance, investigational procedures, and therapeutic interventions were extracted from the electronic health records to derive indicative population estimates and direct health care expenditure relating to CHD., Results: A total of 2,519 patients diagnosed with the 10 target conditions were being actively managed, including 456 (18.1%) new-born and 787 prevalent cases (2.5/1,000 population) aged <5 years. A total of 12,180 (4.8/case) investigations were performed (6,169 echocardiographic and 279 cardiac catheterisation procedures) costing $2.25 million/annum. A further 5,326 clinic visits (2.1/case, 22% regional) were conducted at a cost of $550,000/annum. A combined total of 804 catheter-based interventions and surgical procedures were performed in 300 cases (11.9%) at a cost of $13.6 million/annum. VSD (38.6% of cases) was the single greatest contributor ($5.1 million/annum) to total combined direct health care costs of $13.6 million/annum for the 2,519 patients., Conclusions: These pilot data indicate a significant patient population and health care burden imposed by CHD in Queensland. Future initiatives to better quantify this burden, from an individual to health system perspective, are urgently needed., (Copyright © 2020 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.)

Children who undergo open-heart surgery in the first year of life for congenital heart disease (CHD) are at high-risk for impaired development across multiple domains. International recommendations include systematic periodic developmental surveillance into adolescence and the establishment of long-term follow-up programmes. This article describes the establishment and evolution of the Queensland Paediatric Cardiac Service neurodevelopmental follow-up programme - CHD LIFE (Long-term Improvement in Functional hEalth). Contextualising best practice recommendations to ensure a family-centred and sustainable approach to understand and support the long-term functional health needs of high-risk children with CHD as standard care was needed. We describe the transition from a centralised pilot Programme to the implementation of an integrated statewide approach aimed at delivering consistent high-level standards of care and a platform to evaluate therapeutic interventions., (© 2020 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Background: The purpose of this study was to assess the performance of more than 500 tissue-engineered bovine pericardial implants (CardioCel; Admedus, Toowong, Queensland, Australia) used for the repair of congenital heart defects., Methods: Clinical data were collected for all patients who received a CardioCel implant at three centers (Brisbane, Australia; and Leicester and Bristol, United Kingdom). During this period, 501 CardioCel patches were implanted in 377 patients. The primary endpoint was CardioCel-related surgical or catheter intervention. Secondary endpoints included implant-related thromboembolism, residual shunt, infection, calcification leading to loss of function, and hemodynamic compromise. Mortality or reintervention was considered early if the event occurred within 30 days of implantation., Results: The median follow-up was 31 months (range, 1 to 60). There were 11 deaths (2.9%), 1 related to CardioCel. There was no echocardiographic or radiologic evidence of patch calcification in any patient. Overall freedom from reintervention 3 and 5 years after implantation was 96% (95% confidence interval, 93% to 98%). Fourteen implants (2.8%) required 18 reinterventions (3.6%) at the site of implantation (9 catheter based and 9 operative). There was no difference in the incidence of reintervention in the pulmonary arterial or systemic circulations (P = .18). There was no difference in performance of CardioCel in neonates (aged 0 to 28 days), infants (aged 29 to 365 days), or children aged more than 1 year (P = .22)., Conclusions: CardioCel has good durability when used for the repair of congenital heart defects. It performs comparably in the systemic and pulmonary circulations in neonates, infants, and older children., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Atrioventricular valve failure (moderate or greater regurgitation, or valve operation) is a risk factor for adverse outcomes in patients undergoing Fontan palliation., Objectives: This study determined the incidence of atrioventricular valve failure and its clinical impact on patients undergoing Fontan palliation., Methods: A retrospective cohort longitudinal study was conducted using patient data extracted from an existing bi-national, population-based registry., Results: A total of 1,468 patients who underwent Fontan palliation were identified; complete follow-up data were available for 1,199 patients. Six hundred eighty-six patients had 2 atrioventricular valves, 286 had a single mitral valve, 130 had a common atrioventricular valve, and 97 had a single tricuspid valve. A total of 132 repairs were performed in 110 patients, and 15 replacements were performed in 13 patients. The cumulative incidence of atrioventricular valve failure at 25 years of age for patients with a common atrioventricular, single tricuspid, single mitral, and 2 atrioventricular valves was 56% (95% confidence interval [CI]: 46% to 67%), 46% (95% CI: 31% to 61%), 8% (95% CI: 4% to 12%), and 26% (95% CI: 21% to 30%), respectively. In patients without valve failure, freedom from Fontan failure at 10 and 20 years post-Fontan palliation was 91% (95% CI: 89% to 93%) and 77% (95% CI: 73% to 81%), respectively, compared with 77% (95% CI: 69% to 85%) and 54% (95% CI: 42% to 68%), respectively, in patients with valve failure (hazard ratio: 2.43; 95% CI: 1.74 to 3.39; p < 0.001)., Conclusions: Atrioventricular valve failure occurs frequently in patients undergoing Fontan palliation. Patients with valve failure are twice as likely to have their Fontan circulation fail than those without valve failure., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study aims to assess the performance of tissue-engineered bovine pericardium (CardioCel®) at 24 months and beyond when used for the repair of congenital heart defects., Methods: Between October 2012 and November 2014, CardioCel was implanted in 135 patients (140 procedures and 195 implants). Applications included the closure of septal defects (98, 50.3%), repair of pulmonary arteries (63, 32.3%), intra-atrial/intraventricular baffles (10, 5.1%), repair of systemic arteries (15, 7.7%), valve repair (5, 2.6%), repair of systemic veins (3, 1.5%) and Fontan operation (1, 0.5%). There were 19 (13.6%) procedures performed in neonates, 77 (55%) in infants and 44 (31.4%) in children older than 365 days., Results: Eight patients (n = 135, 5.9%) required reintervention in 12 instances (n = 195, 6.2%, 6 catheters and 6 surgical). There was no echocardiographic or radiological evidence of calcification in any patient. Ten of the reinterventions (83%) occurred within the first 12 months. All the reinterventions occurred within 36 months. Freedom from reintervention at both 12 and 24 months was 95% [confidence interval (CI) 91-97] and at 36 months was 94% (CI 89-97). There was no statistical difference in freedom from reintervention when stratified by age or patch position., Conclusions: At 24 months and beyond the follow-up, the performance of CardioCel remains acceptable with good haemodynamic performance. There was no echocardiographic or radiological evidence of calcification in any patient. CardioCel performs comparably in systemic and pulmonary circulations.

CardioCel is a bovine pericardium that is subjected to a novel anticalcification tissue-engineering process. We present the histopathologic findings of human explants of CardioCel that were used in operations for congenital heart disease in children. Six explants were identified from 140 patients undergoing CardioCel implants from October 2012 to March 2015. CardioCel explants were evaluated histologically using hematoxylin and eosin, Masson trichrome, and immunohistochemical staining. A variable inflammatory response was seen in the surrounding native tissue, but not within the CardioCel graft in any of the explants. A neointimal layer of varying thickness developed on the visceral surface of 5 CardioCel explants with endothelialization of the longest duration explant. A granulation tissue layer developed on the parietal surface of the graft (consistently thicker than the neointima). Maintained collagen fiber architecture (laminated) and variable fibroblastic invasion (which increased with the age of the implant) were identified in all 6 cases. Scattered capillary vessels were noted in the majority of the explants with new collagen fibers in one, suggesting early remodeling. Calcium was seen in 1 explant at the interface of the graft and inflammatory response on its parietal surface. Evidence of graft remodeling was noted in the majority of the explants without inflammatory cells or calcification within the explanted graft material. A noticeable feature was the differential thickness of the host reaction to the parietal compared with the visceral surface of the graft. We will continue to evaluate CardioCel as a cardiovascular substitute for extracardiac and intracardiac reconstructions., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD)., Methods: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI)., Results: Among 1770 subjects from 22 institutions, assessed at age 14.5 ± 3.7 months, PDIs and MDIs (77.6 ± 18.8 and 88.2 ± 16.7, respectively) were lower than normative means (each P < .001). Later calendar year of birth was associated with an increased proportion of high-risk infants (complexity of CHD and prevalence of genetic/extracardiac anomalies). After adjustment for center and type of CHD, later year of birth was not significantly associated with better PDI or MDI. Risk factors for lower PDI were lower birth weight, white race, and presence of a genetic/extracardiac anomaly (all P ≤ .01). After adjustment for these factors, PDIs improved over time (0.39 points/year, 95% confidence interval 0.01 to 0.78; P = .045). Risk factors for lower MDI were lower birth weight, male gender, less maternal education, and presence of a genetic/extracardiac anomaly (all P < .001). After adjustment for these factors, MDIs improved over time (0.38 points/year, 95% confidence interval 0.05 to 0.71; P = .02)., Conclusions: Early neurodevelopmental outcomes for survivors of cardiac surgery in infancy have improved modestly over time, but only after adjustment for innate patient risk factors. As more high-risk CHD infants undergo cardiac surgery and survive, a growing population will require significant societal resources., (Copyright © 2015 by the American Academy of Pediatrics.)

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.)

Venovenous anomalous vessels are a common complication seen in the bidirectional cavopulmonary circulation. In this report, we describe the use of the Amplatzer Vascular Plug II and subsequent demonstration of nitinol device memory with significant device reconfiguration at 5 months follow-up., (Copyright 2010 Wiley-Liss, Inc.)

Background: Post-operative junctional ectopic tachycardia is a transient, but potentially life threatening, rapid automatic tachyarrhythmia that requires urgent and adequate treatment. In our study, we review retrospectively the use and efficacy of amiodarone for this arrhythmia over an 8-year period in our institution., Methods and Patients: Retrospective review revealed 15 patients who were administered amiodarone for post-operative junctional ectopic tachycardia during the period. The median age was 2.6 months, with a range from 8 days to 8.1 months. The median weight was 4.6 kilograms, with a range from 2.6 to 8.2 kilograms., Results: The median heart rate at diagnosis of the tachycardia was 192 beats per minute, and the range was 182 to 229 beats per minute. The biochemistry was essentially normal. The median length of time until the tachycardia was controlled was 4.5 hours, and the range was from 1 to 19.5 hours, with 13 of the 15 patients controlled within 12 hours. The median dose of amiodarone received by this time was 5.9 milligrams per kilogram, with a range from 1.0 to 25.0 milligrams per kilogram. Hypotension or bradycardia within 4 hours of commencing amiodarone were noted in 2 patients., Conclusion: Experience in our institution, and a review of the literature, suggests that the most rapid control of post-operative junctional ectopic tachycardia will be obtained by a bolus of amiodarone followed by an intravenous infusion. Intravenous amiodarone is generally safe, with few side effects. Reported life threatening arrhythmias, however, suggest that intravenous amiodarone should be restricted to a setting where invasive monitoring and external cardiac pacing are available.

Objective: To determine the diagnostic accuracy of prenatal fetal echocardiography., Methods: The study was a retrospective chart review of 190 consecutive patients over a 3-year period from November 1998 to February 2002 of all women referred to the Maternal Fetal Medicine unit, Mater Mothers Hospital, for fetal echocardiography. The prenatal diagnosis was compared with the postnatal diagnosis made by postnatal echocardiography, surgical findings or post-mortem. The accuracy of prenatal diagnosis was described on a predetermined 4-point scale., Results: Of the 89 patients, for whom complete diagnostic follow-up was available, there was complete agreement between the prenatal and postnatal diagnosis in 63 cases, minor discrepancies in 25 cases and major disagreement in 1 case., Conclusions: In experienced hands, fetal echocardiography is accurate and allows medical staff and patients information in order manage a pregnancy appropriately., (Copyright (c) 2004 John Wiley & Sons, Ltd.)

Postoperative junctional ectopic tachycardia (JET) is a potential life-threatening tachycardia that mainly occurs after surgical correction of congenital heart defects. The arrhythmia itself or the related treatment has significant clinical impact on the postoperative course and intensive care stay. In general, JET is a self-limiting disorder that usually resolves within one week. However, JET occurs usually within the first 24 to 48 hours after corrective surgery, when systolic and diastolic function of the heart is impaired. Thus, the rapid heart rate leads to an acute further deterioration of cardiac output that requires adequate treatment. The diagnosis of JET is made by the typical ECG-appearance with narrow QRS-configuration at a rate of 170 to 260 bpm and AV-dissociation. A variety of different therapeutic strategies have been tested in postoperative and congenital/spontaneous JET. Treatment success is usually defined as a stable decrease in the ventricular rate below 140-150/min, the possibility of atrial pacing and thereby the improvement of cardiac output. Optimal success is the reinstitution of sinus rhythm. Many of the treatment strategies reported are based on specific institutional treatment protocols. These include conventional supportive treatment, specific medical antiarrhythmic therapy, specific forms of pacing and surface cooling. Today, the administration of high doses of amiodarone usually leads to adequate control of the rate and enables pacing. Surgical intervention or catheter ablation of the HIS-bundle is rarely necessary. This article reviews the literature about JET over the past years and offers a specific treatment protocol.

Videoconferencing at 384 kbit/s for the transmission of echocardiograms has proved useful for the assessment of children with suspected cardiac disease, in regional areas of Queensland. A retrospective review of patient and management outcomes was conducted on cardiac teleconsultations performed at two regional hospitals during the period November 2000 to February 2004, inclusive. There were 106 echo studies. A subset of 72 cardiac teleconsultations performed between May 2001 and February 2004 was reviewed in detail. The median age of patients at the time of consultation was 3 months (range 1 day-17 years). Sixteen per cent of teleconsultations were classified as urgent and were conducted on the same day as referral. Following the videoconference, 90% of patients could be managed locally and reviewed by the paediatrician or visiting paediatric cardiologist during an outreach clinic. Six children (8%) had significant cardiac lesions that were initially managed locally, with subsequent elective transfer at the appropriate time for treatment. Only one child (1%) required urgent transfer to the tertiary centre for specialist care and surgery. Telecardiology was effective in accurately identifying congenital heart disease. Paediatric telecardiology is an evolving modality of assessment and communication, and is likely to result in continued improvements in patient care, patient outcomes and parental satisfaction, in provincial centres removed from the tertiary cardiac centre.