A Comparison of Ductal Stenting and Surgical Shunts for Infants with Duct-Dependent Pulmonary Blood Flow: The Impact of Single Versus Biventricular Repair Pathways on Outcomes.

Ductal stenting (DS) is an alternative to the Blalock-Taussig-Thomas Shunt (BTTS) as initial palliation for congenital heart disease with duct-dependent pulmonary blood flow (DDBPF). We sought to analyze the impact of intended single ventricle (SV) and biventricular (BiV) repair pathways on the outcome of DS and BTTS in infants with DDPBF. A single-center, retrospective comparison of infants with DDPBF who underwent either DS (2012-2022) or BTTS procedures (2013-2017). Primary outcomes included all-cause mortality and risk of unplanned re-intervention. Participants were divided into four groups: 1.SV with DS, 2.SV with BTTS, 3.BiV with DS, and 4.BiV with BTTS. Fifty-one DS (SV 45%) and 86 BTTS (SV 49%) procedures were undertaken. For those who had DS, mortality was lower in the BiV compared to SV patients (BiV: 0/28, versus SV: 4/23, p = 0.04). Compared to BiV DS, BiV BTTS had a higher risk of combined death or unplanned re-intervention (HR 4.28; CI 1.25-14.60; p = 0.02). In SV participants, there was no difference for either primary outcome based on procedure type. DS was associated with shorter intensive care length of stay for SV participants (mean difference 5 days, p = 0.01) and shorter intensive care and hospital stay for BiV participants (mean difference 11 days for both outcomes, p = 0.001). There is a survival benefit for DS in BiV participants compared with DS in SV and BTTS in BiV participants. Ductal stenting is associated with a shorter intensive care and hospital length of stay.

Cognitive Functioning and Psychosocial Outcomes in Adults with Complex Congenital Heart Disease: A Cross-sectional Pilot Study.

Adults with complex congenital heart disease (CHD) are at risk for cognitive dysfunction. However, associations between cognitive dysfunction and psychosocial outcomes are poorly defined. Between June and November 2022, we prospectively recruited 39 adults with complex CHD who completed a computerized cognitive assessment (Cogstate) and validated psychosocial scales measuring psychological distress, health-related quality of life (HRQOL), and resilience. Participants had a mean age of 36.4 ± 11.2 years. Over half (62%) were women, most (79%) had complex biventricular CHD, and 21% had Fontan physiology. Prevalence of cognitive dysfunction was greatest in the domains of attention (29%), working memory (25%), and psychomotor speed (21%). Adjusting for age and sex, Pearson partial correlations between Cogstate z-scores and self-reported cognitive problems were small. Participants who lived in the most disadvantaged areas and those with a below-average annual household income had lower global cognitive z-scores (p = 0.02 and p = 0.03, respectively). Two-thirds (64%) reported elevated symptoms of depression, anxiety, and/or stress. Small correlations were observed between psychological distress and cognitive performance. Greater resilience was associated with lower psychological distress (r ≥ -0.5, p < 0.001) and higher HRQOL (r = 0.33, p = 0.02). Our findings demonstrate that adults with complex CHD have a high risk of cognitive dysfunction, though may not recognize or report their cognitive challenges. Lower socioeconomic status may be an indicator for those at risk of poorer cognitive functioning. Psychological distress is common though may not be a strong correlate of performance-based cognitive functioning. Formal cognitive evaluation in this patient population is essential. Optimizing resilience may be a protective strategy to minimize psychological distress and bolster HRQOL.

Nutritional considerations for people living with a Fontan circulation: a narrative review.

The population of people living with a Fontan circulation are highly heterogenous, including both children and adults, who have complex health issues and comorbidities associated with their unique physiology throughout life. Research focused on nutritional considerations and interventions in the Fontan population is extremely limited beyond childhood. This review article discusses the current literature examining nutritional considerations in the setting of Fontan physiology and provides an overview of the available evidence to support nutritional management strategies and future research directions. Protein-losing enteropathy, growth deficits, bone mineral loss, and malabsorption are well-recognised nutritional concerns within this population, but increased adiposity, altered glucose metabolism, and skeletal muscle deficiency are also more recently identified issues. Emergencing evidence suggets that abnormal body composition is associated with poor circulatory function and health outcomes. Many nutrition-related issues, including the impact of congenital heart disease on nutritional status, factors contributing to altered body composition and comorbidities, as well as the role of the microbiome and metabolomics, remain poodly understood.

Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Prolonged Enteral Tube Feeding in Infants With a Functional Single Ventricle Is Associated With Adverse Outcomes After Fontan Completion.

OBJECTIVES: To define the baseline characteristics of long-term tube-fed (TF) single ventricle patients, investigate associations between long-term enteral tube feeding and growth, and determine associations with long-term outcomes after Fontan procedure. STUDY DESIGN: We performed a retrospective cohort study of patients in the Australia and New Zealand Fontan Registry undergoing treatment at the Royal Children's Hospital, the Children's Hospital at Westmead, Royal Melbourne Hospital, and Royal Prince Alfred Hospital from 1981 to 2018. Patients were defined as TF or non-tube-fed (NTF) based on enteral tube feeding at the age of 90 days. Feeding groups were compared regarding body mass index (BMI) trajectory, BMI at last follow-up, and long-term incidence of severe Fontan failure. RESULTS: Of 390 patients (56 [14%] TF, 334 [86%] NTF), TF was associated with right ventricular dominance, hypoplastic left heart syndrome, Norwood procedure, increased procedures prior to Fontan, extracardiac conduit Fontan, Fontan fenestration, and atrioventricular valve repair/replacement. TF patients were less likely to be in the higher compared with lowest 0-6 month BMI trajectory (P < .01; P = .03), had lower 6 month weight-for-age z-scores (P < .01) and length-for-age z-scores (P = .01). TF were less likely to be overweight/obese at pediatric follow-up (hazard ratio [HR] = 0.31, 95% CI: 0.12-0.80; P = .02) and more likely to be underweight at adult follow-up (HR = 16.51; 5% CI: 2.70-101.10; P < .01). TF compared with NTF was associated with increased risk of severe Fontan failure (HR = 4.13; 95% CI = 1.65-10.31; P < .01). CONCLUSIONS: Prolonged infant enteral tube feeding is an independent marker of poor growth and adverse clinical outcomes extending long-term post-Fontan procedure.

Investigating the scope and costs of dental treatment provided under general anaesthesia among children with congenital heart disease.

AIM: To identify the types of dental treatment provided under general anaesthesia for children diagnosed with congenital heart disease (CHD), quantify the costs within a publicly funded tertiary paediatric hospital setting and identify factors which affect the cost. METHODS: A retrospective analysis of dental records (July 2015 to June 2019) was conducted for children with CHD who had undergone a dental general anaesthetic procedure at The Children's Hospital at Westmead, Australia. Patient and treatment-related information were collected, and a costing analysis was performed on 89 dental general anaesthetic procedures. RESULTS: Mean age at the time of the general anaesthetic was 8.15 years. About 27% of children with CHD had a history of dental infection. Dental extractions and restorations comprised the majority of treatments provided, with extractions performed in 86% of procedures. The mean number of days in hospital was 1.43 and the mean cost was $4395.14. The cost was significantly greater when children presented with a facial swelling compared to any other reason. CONCLUSIONS: Dental extractions are performed in the majority of general anaesthetics. Not only is there an economic burden to the public health system in providing dental treatment under general anaesthesia for children with CHD, the health impacts also appear to be substantial. A considerable proportion required overnight hospitalisation and days in hospital was strongly related to the cost of the dental general anaesthetic. Systematic referral pathways for accessing dental care are an important consideration for children with CHD.

Neurocognitive Dysfunction and Smaller Brain Volumes in Adolescents and Adults With a Fontan Circulation.

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.

Safety and efficacy of exercise training in children and adolescents with congenital heart disease: A systematic review and descriptive analysis.

BACKGROUND: While exercise training is beneficial in the prevention and management of many chronic diseases, the role of exercise training in children and adolescents with congenital heart disease is less understood. We sought to determine the safety and efficacy of exercise training in children and adolescents with congenital heart disease. METHODS: We conducted a systematic search of the following databases: PubMed, CINAHL, EMBASE, Web of Science and SportDiscus. We included randomised controlled trials that incorporated an exercise intervention compared with a non-exercising comparator group and examined safety and efficacy in children and adolescents with congenital heart disease. A descriptive analysis of the included trials was then conducted. RESULTS: A total of 9 articles from 6 trials (642 participants with varying conditions and disease severity) were included. Significant variability of study participants and outcomes were observed across the trials. No adverse events linked to the exercise interventions were stated. The articles reported numerous positive changes to clinically relevant fitness measures. Exercise capacity improved with exercise training in 3 of 4 trials in which it was measured. Cardiorespiratory fitness showed improvements in 3 of 4 trials. Neuromuscular fitness increased in 1 of 2 trials. Physiological and metabolic parameters were improved, and negative changes were not observed to several clinically important measures (e.g. muscular oxygenation, cardiac measures) in 2 of 2 trials. Physical activity increased in 1 of 3 trials. No articles reported on changes in measures of body composition. Outcomes are varied with little consensus on measurements or assessment methods. CONCLUSIONS: Exercise training appears to be safe and efficacious for improving physical fitness in children and adolescents with congenital heart disease who have been appropriately screened by their medical team. However, the certainty of the evidence for these findings is low to moderate.

The Australia and New Zealand Cardio-Oncology Registry: evaluation of chemotherapy-related cardiotoxicity in a national cohort of paediatric cancer patients.

Cancer therapy related cardiac dysfunction (CTRCD) is an area of increasing focus, particularly during the survivorship period, for paediatric, adolescent and adult cancer survivors. With the advent of immunotherapy and targeted therapy, there is a new set of mechanisms from which paediatric and young adult patients with cancer may suffer cardiovascular injury. Furthermore, cardiovascular disease is the leading cause of morbidity and mortality in the survivorship period. The recently established Australian Cardio-Oncology Registry is the largest and only population-based cardiotoxicity database of paediatric and adolescent and young adult oncology patients in the world, and the first paediatric registry that will document cardiotoxicity caused by chemotherapy and novel targeted therapies using a prospective approach. The database is designed for comprehensive data collection and evaluation of the Australian practice in terms of diagnosis and management of CTRCD. Using the Australian Cardio-Oncology Registry critical clinical information will be collected regarding predisposing factors for the development of CTRCD, the rate of subclinical left ventricular dysfunction and transition to overt heart failure, further research into protectant molecules against cardiac dysfunction and aid in the discovery of which genetic variants predispose to CTRCD. A health economic arm of the study will assess the cost/benefit of both the registry and cardio-oncology clinical implementation. Finally, an imaging arm will establish if exercise cardiac magnetic resonance imaging and VO2 max testing is a more sensitive predictor of cardiac reserve in paediatric and adolescent and young adult oncology patients exposed to cardiac toxic therapies.

CSANZ Position Statement on COVID-19 From the Paediatric and Congenital Council✰.

At the time of writing (25 May 2020), there have been nearly 4.4 million infections and 300,000 deaths worldwide related to COVID-19, an infection caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Australia (currently 6,900 infections and 98 deaths) and New Zealand (1,500 infections and 21 deaths) have thus far been less affected than other regions. Risk factors for more severe disease include older age and pre-existing cardiovascular disease. The purposes of this document from the Paediatric and Congenital Council of the Cardiac Society of Australia and New Zealand (CSANZ) are to: 1) To review the mechanisms for cardiac involvement in COVID-19, specifically as they may impact patients with childhood and adult congenital heart disease (CHD); 2) To review the impact of SARS-CoV-2 infection in the paediatric population; 3) To review available data on the risks related to COVID-19 for childhood heart disease and adult CHD; 4) To provide guidance for childhood heart disease and adult CHD units in our Australasian region to re-organise services during the pandemic, so as to protect a highly specialised workforce and yet continue to provide an essential service; and 5) To review risk reduction strategies for acquiring COVID-19 for patients with childhood heart disease or adult CHD. Eleven (11) recommendations relevant to the care of children with heart disease and adults with CHD to mitigate the impact of COVID-19 are highlighted through the document.

Living With, and Caring for, Congenital Heart Disease in Australia: Insights From the Congenital Heart Alliance of Australia and New Zealand Online Survey.

BACKGROUND: There is a paucity of data describing the day-to-day experiences of adult Australians personally living with or caring for a child born with congenital heart disease (CHD). Such data would be of great practical importance to inform health care initiatives to improve outcomes. METHODS: 588 men (38.3 ± 11.9 years) and women (39.6 ± 12.6 years, 78% of respondent patients) living with CHD and 1,091 adult carers (93% mothers) of children with CHD (median age 7.3 [IQR 3.5-13.3 years], 54% male), representing all Australian states and territories, responded to a comprehensive online survey designed and hosted by the Congenital Heart Alliance of Australia and New Zealand. Data on demographic factors, the nature of underlying CHD, interactions with health care services, psychological wellbeing and wider impacts of CHD were collected. RESULTS: Most respondents were able to identify the type of CHD they (29% with a simple lesion such atrial septal defect, 17% tetralogy of Fallot) or their child had (21% with a simple lesion, 15% tetralogy of Fallot), whilst 90% cases of CHD had undergone cardiac surgery. Patients with CHD were mostly employed (70%) or studying (8.8%), whilst 9.1% were receiving disability benefits. In terms of transition care, 52% of adult patients had been referred by a paediatric to adult cardiologist with 84% still actively managed by a specialist. Overall, 31% of patients with CHD sought emergency care and required >10 days sick leave in the past 12 months. Moreover, 71% and 55% of patients, respectively, reported recent feelings of anxiety/worry or depressive thoughts related to their CHD (61% sought professional assistance). Consistent with high levels of disruption to daily living, 59% of carer respondents (24%>10 days) had taken carer's leave in the past 12 months. CONCLUSIONS: These contemporary, self-reported, Australian data reveal the burden of living and caring for CHD from an adult's perspective. Survey respondents highlighted the potential disconnect between paediatric and adult CHD services and suggest an important, unmet need for dedicated health services/community care to cost-effectively manage high levels of health care utilisation coupled with associated psychological distress.

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement.

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

Weight Gain Trajectories from Birth to Adolescence and Cardiometabolic Status in Adolescence.

OBJECTIVE: To assess the influence of the trajectory of weight gain from birth to adolescence on cardiovascular and metabolic risk. We studied childhood body mass index (BMI) trajectories from birth to age 14 years and cardiometabolic risk factors at age 14 years. STUDY DESIGN: In total, 410 children with weight and height measurements were assessed from birth throughout childhood, from the Childhood Asthma Prevention Study, a prospective community-based cohort. BMI trajectory groups were determined by latent basis growth mixture models. Of these subjects, 190 had detailed cardiometabolic risk factors assessed at age 14 years. RESULTS: Three BMI trajectory groups were identified; normal BMI, "early rising" excess BMI from 2 years, and "late rising" excess BMI from 5 years. Differences were found between normal and excess BMI in children at 14 years of age. In addition, children with an early rising BMI trajectory had statistically significantly higher central adiposity and a more atherogenic lipoprotein profile at age 14 years than children with a late rising BMI trajectory (P < .05). No differences between BMI trajectory groups in vascular structure or function was identified at age 14 years. CONCLUSIONS: Earlier onset of an elevated BMI trajectory persisting from birth to age 14 years results in an unfavorable cardiometabolic risk profile at age 14 years, including central adiposity and more atherogenic lipoproteins, independent of achieved BMI.

Intermittent Energy Restriction Is a Feasible, Effective, and Acceptable Intervention to Treat Adolescents with Obesity.

BACKGROUND: Intermittent energy restriction (IER) is an effective obesity management strategy in adults. OBJECTIVE: The aim of this study was to investigate the feasibility, effectiveness, and acceptability of IER in adolescents (aged 12-17 y) with obesity [adult equivalent body mass index (BMI; kg/m2) ≥30]. METHODS: During weeks 1-12 participants followed an IER dietary plan consisting of a very-low-energy diet (VLED) 3 d/wk (500-600 kcal/d) and an eating plan consistent with national dietary guidelines 4 d/wk. For weeks 13-26 participants chose to continue with 1-3 VLED d/wk or follow a prescriptive eating plan. Primary outcomes were feasibility and change in BMI expressed as a percentage of the 95th percentile (BMI %95th percentile) at 12 wk. Secondary outcomes were diet acceptability, body composition, cardiometabolic risk, vascular structure and function, quality of life (Pediatric Quality of Life Inventory), and eating behaviors [Dutch Eating Behavior Questionnaire (DEBQ-C)]. Linear mixed models were used to assess change in outcome measures. RESULTS: Of 45 adolescents invited to participate, 30 adolescents (mean ± SD age: 14.5 ± 1.4 y, female n = 25) with a median BMI of 34.9 (range: 27.7-52.4) were recruited. At 12 wk, 23 participants chose to continue with the VLED 2-3 d/wk, and 21 completed the study, indicating the feasibility of IER. Consistent with intention-to-treat analysis, BMI %95th percentile was reduced at 12 wk (difference in estimated marginal means ± SEMs: -5.6 ± 1.1, P < 0.001) and 26 wk (-5.1 ± 1.9, P = 0.013) compared with baseline. Plasma triglycerides were reduced at 26 wk from baseline (-0.33 ± 0.12 mmol/L, P = 0.03). Body fat percentage reduced between 12 and 26 wk (-1.57% ± 0.76%, P = 0.05). Carotid intima-media thickness (CIMT) (-0.06 ± 0.01 mm, P < 0.001) and flow-mediated dilation (absolute increase 0.44% ± 0.11%, P = 0.001) improved between baseline and 12 wk, with reduced CIMT maintained at 26 wk (P < 0.001). DEBQ-C and Pediatric Quality of Life Inventory scores improved throughout the intervention. Nineteen adolescents completed an acceptability interview, rating IER as easy and pleasant to follow (mean ± SD: +2.1 ± 1.2; +1.9 ± 1.2, respectively) on a Likert scale from -4 to +4. CONCLUSION: IER is a feasible, effective, and acceptable intervention in adolescents with obesity achieving reductions in BMI and cardiovascular disease risk. This trial was registered at www.anzctr.org.au as ACTRN12618000200280.

The Degree of Left Ventricular Hypoplasia Is Associated with Tricuspid Regurgitation Severity in Infants with Hypoplastic Left Heart Syndrome.

Tricuspid valve regurgitation (TR) increases adverse outcomes in children with hypoplastic left heart syndrome (HLHS). Changes in tricuspid valve (TV) annulus and leaflet geometry have been described but the underlying causes for TR in HLHS remain uncertain. We aimed to examine the association between left ventricular (LV) size and TR in infants with HLHS as right ventricular (RV)-LV interactions may be important in TR development. Echocardiograms of 50 infants with HLHS were reviewed. LV size, RV function, TR grade, TV annulus z-score, and aortic arch obstruction were examined at birth and 1 year of age (or the latest study post-bidirectional Glenn anastomosis if the patient was < 1 year of age). 24/50 (48%) had severe LV hypoplasia and 26/50 (52%) had mild/moderate LV hypoplasia. At 1 year, 10/24 (42%) with severe LV hypoplasia had moderate/severe TR versus 0/26 in the mild/moderate LV hypoplasia group (p = 0.0002). TR progressed ( ≥ 1 grade) in 14/24(58%) with severe LV hypoplasia versus 5/26 (19%) with mild/moderate LV hypoplasia (p = 0.008). In this cohort, no association was found between the degree of TR and either RV function, TV annular z-score, or arch obstruction; or between the degree of LV hypoplasia and either RV function or TV annular z-score. In infants with HLHS, the severity and progression of TR is associated with the severity of LV hypoplasia. The mechanism for this association needs further exploration but suggests a role for RV-LV interactions in the development of TR.

Management errors in adults with congenital heart disease: prevalence, sources, and consequences.

Aims: Improved survival has resulted in increasing numbers and complexity of adults with congenital heart disease (ACHD). International guidelines recommend specialized care but many patients are still not managed at dedicated ACHD centres. This study analysed referral sources and appropriateness of management for patients referred to our tertiary ACHD Centre over the past 3 years. Methods and results: We compared differences in care between patients referred from paediatric/ACHD-trained vs. general adult cardiologists, according to Adherence (A) or Non-Adherence (NA) with published guidelines. Non-Adherent cases were graded according to the severity of adverse outcome or risk of adverse outcome. Of 309 consecutively referred patients (28 ± 14 years, 51% male), 134 (43%) were from general cardiologists (19% highly complex CHD) and 115 (37%) were from paediatric cardiology or ACHD specialists (33% highly complex CHD). Sixty referrals (20%) were from other medical teams and of those, 31 had been lost to follow-up. Guideline deviations were more common in referrals from general compared to CHD-trained cardiologists (P < 0.001). Of general cardiology referrals, 49 (37%) were NA; 18 had catastrophic or major complications (n = 2, 16 respectively). In contrast, only 12 (10%) of the paediatric/ACHD referrals were NA, but none of these were catastrophic and only 3 were major. Simple, moderate, and highly complex CHD patients were at increased risk of adverse outcome when not under specialized CHD cardiology care (P = 0.04, 0.009, and 0.002, respectively). Conclusion: Non-adherence with guidelines was common in the ACHD population, and this frequently resulted in important adverse clinical consequences. These problems were more likely in patients who had not been receiving specialized CHD care. Configuring healthcare systems to optimize 'whole of life' care for this growing population is essential.

Children's experiences of congenital heart disease: a systematic review of qualitative studies.

We aimed to describe the experiences of children and adolescents with congenital heart disease (CHD). Electronic databases were searched until August 2016. Qualitative studies of children's perspectives on CHD were included. Data was extracted using thematic synthesis. From 44 studies from 12 countries involving 995 children, we identified 6 themes: disrupting normality (denying the diagnosis, oscillating between sickness and health, destabilizing the family dynamic), powerlessness in deteriorating health (preoccupation with impending mortality, vulnerability to catastrophic complications, exhaustion from medical testing), enduring medical ordeals (traumatized by invasive procedures, disappointed by treatment failure, displaced by transition, valuing empathy and continuity in care, overcoming uncertainty with information), warring with the body (losing stamina, distressing inability to participate in sport, distorted body image, testing the limits), hampering potential and goals (feeling disabled, unfair judgment and exclusion, difficulties with academic achievement, limiting attainment and maintenance of life milestones), and establishing one's own pace (demarcating disease from life, determination to survive, taking limitations in their stride, embracing the positives, finding personal enrichment, relying on social or spiritual support). CONCLUSION: Children with CHD feel vulnerable and burdened by debilitating physical symptoms, unpredictable complications, and discrimination. Clinicians may support patients by sharing recognition of these profound psychosocial consequences. What is Known: • CHD is associated with difficulties in learning and attention, school absenteeism, decreased endurance, poor body image, and peer socialization • What is lesser known is how young patients cope with the symptoms, prognostic uncertainty, and treatment burden What is New: • We found that children are challenged by lifestyle restrictions, fear of invasive procedures, impaired body image, discrimination, and uncertainty about the future. Feelings of disempowerment are intensified by the unpredictability of disease progression • Thus, strategies to improve outcomes include improved patient education on disease and lifestyle management and partnership with school teachers and counselors for unique psychosocial support.

Reproducibility of Cardiac Magnetic Resonance Imaging (CMRI)-Derived Right Ventricular Parameters in Repaired Tetralogy of Fallot (ToF).

BACKGROUND: Quantification of right ventricular (RV) volumes is challenging owing to variable reproducibility and is especially so in congenital heart disease. Cardiac magnetic resonance (CMR) has the ability to more comprehensively survey the entire right ventricle and is currently considered the gold standard. AIMS: We aimed to determine the inter-observer reproducibility of CMR-derived RV volumes generated by two independent and experienced (SCMR Level III) observers in Tetralogy of Fallot (ToF) patients with varying degrees of RV dilatation. METHODS: We performed a retrospective analysis of 120 consecutive patients with repaired ToF who underwent CMR. Two blinded observers calculated RV volumes in each oblique short axis slice independently. Bland-Altman analysis and inter-observer correlation coefficients (ICC) were assessed. RESULTS: The coefficients of variation for RV parameters were: 2.9%, 8% and 3.4% for right ventricular end diastolic volume (RVEDV), right ventricular end systolic volume (RVESV) and right ventricular ejection fraction (RVEF) respectively. For RVEDV the interobserver correlation was 0.992 demonstrating excellent volumetric correlation between observers. The mean difference between the observers for right ventricular end diastolic volume index (RVEDVi) was 2.5ml/m2 (95% limits of agreement -7.3 to 12.2ml/m2). For patients with mild-moderate RV dilatation (RVEDVi <150ml/m2) the mean difference of RVEDVi was 1.8ml/m2 (95% limits of agreement -5.7 to 9.3ml/m2). For patients with severe RV dilatation (RVEDVi≥150ml/m2) the mean difference was -3.4ml/m2 (95% limits of agreement -8.6 to 15.4ml/m2). CONCLUSIONS: In patients with repaired ToF and variable degrees of RV dilatation, CMR assessment of RV volumes and function has high inter-observer reproducibility. This allows for optimal timing of pulmonary valve replacement, based on progression of RV dilatation over time.