The effects of telerehabilitation in adults with complex biventricular congenital heart conditions: protocol for a multi-centre, randomised controlled trial-CH-FIT.

BACKGROUND: Accumulated evidence suggests that exercise training exerts beneficial effects on people with congenital heart conditions. These findings are predominantly derived from small, single-centre exercise trials conducted in outpatient rehabilitation facilities. In recent years, the delivery of exercise interventions remotely has increased through digital communications technology (telerehabilitation). However, very little research to date has been conducted into the efficacy of telerehabilitation in people with a congenital heart condition. AIMS: To evaluate the effects of a telehealth-delivered exercise intervention in people with a history of a surgical biventricular repair due to a congenital heart condition. METHODS: One hundred eligible adolescent (≥ 16 years) and adult participants living with a complex biventricular congenital heart condition will be recruited from four Australian sites and randomised to either (1) a 16-week telehealth-delivered combined (aerobic and resistance) exercise training programme of moderate-to-vigorous intensity or (2) usual care (control group), in a 1:1 allocation, with an 8-month follow-up. OUTCOMES OF INTEREST: The primary outcome will be the change in aerobic capacity expressed as peak oxygen uptake (VO2peak). Secondary outcomes will include changes in vascular function, muscle oxygenation, metabolic profile, body composition and musculoskeletal fitness, neurohormonal activation, neurocognitive function, physical activity levels, dietary and nutritional status, and quality of life. Outcomes will be assessed at baseline, 16 weeks, and 12 months (to determine longer-term maintenance potential). DISCUSSION: If found to be efficacious, telerehabilitation may be an alternative option for delivering exercise, improving health outcomes, and increasing accessibility to exercise programmes. Efficacy data is required to quantify the clinical significance of this delivery mode of exercise. TRIAL REGISTRATION: ACTRN12622000050752 Trial registration date: 17 January 2022 Trial registration URL: https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=382635&showOriginal=true&isReview=true Trial registry name: Australian and New Zealand Clinical Trials Registry.

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.

Effects of Age and Playing Position on Field-Based Physical Fitness Measures in Adolescent Female Netball Players.

This cross-sectional study investigated the impact of age and playing position, controlling for maturity, on physical fitness indicators in 303 adolescent female netball players aged 12.0 to 15.9 years. Assessments included estimated maximal oxygen uptake (VO2max) via the 20 m shuttle run test, 10 m and 20 m sprints, change of direction speed (CODS) using the 505 test, and muscle power via the medicine ball chest throw (MBCT) and countermovement vertical jump (CMJ). Participants were grouped by age (12 to 15 years) and playing position (non-circle and circle players), with age at peak height velocity as a covariate for maturity. Results revealed that, at 15 years, CMJ height was greater than at 12 years and 13 years (p < 0.05, partial η2 = 0.048). MBCT distance increased across age groups (p < 0.01, partial η2 = 0.323). Age had no impact on sprints, VO2max, or CODS. Non-circle players outperformed circle players in the 10 m sprint (p = 0.042, partial η2 = 0.016) and 20 m sprints (p = 0.010, partial η2 = 0.025) and displayed higher VO2max (p < 0.001, partial η2 = 0.036). Circle players were taller (p = 0.046, partial η2 = 0.014) and heavier (p < 0.001, partial η2 = 0.040) than non-circle players. Playing positions showed no differences in CMJ and MBCT. In adolescent female netball players, only muscle power is influenced by age, while non-circle players exhibit superior aerobic fitness and speed compared to circle players. Coaches may be able to utilize the distinct age and playing position traits of adolescent netballers to inform player selection and design targeted training programs.

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.

Acute Effects of Resistance Exercise on Intraocular Pressure in Healthy Adults: A Systematic Review.

ABSTRACT: Hackett, DA, Li, J, Wang, B, Way, KL, Cross, T, and Tran, DL. Acute effects of resistance exercise on intraocular pressure in healthy adults: A systematic review. J Strength Cond Res 38(2): 394-404, 2024-Intraocular pressure (IOP) tends to fluctuate during a resistance exercise (RE). This systematic review examines the acute effects of RE on IOP in healthy adults and factors that influence changes in IOP. Five electronic databases were searched using terms related to RE and IOP. A strict inclusion criterion was applied, which included being 55 years or younger with no medical conditions and RE intensity needing to be quantifiable (e.g., based on a maximal effort). Thirty-four studies met the inclusion criteria for this review. Isometric and isotonic contractions produced similar changes in IOP during RE up to 28.7 mm Hg. Exercises that involved larger muscle mass, such as squats and leg press, were found to produce changes in IOP during exercise ranging from 3.1 to 28.7 mm Hg. Smaller changes in IOP during RE were found for exercises engaging less muscle mass (e.g., handgrip and bicep curls). Intraocular pressure was found to increase during RE when lifting heavier loads and with longer exercise durations (e.g., greater repetitions). The Valsalva maneuver (VM) and breath-hold during RE accentuated the change in IOP, with more extreme changes observed with the VM. However, most studies showed that postexercise IOP returned to baseline after approximately 1 minute of recovery. An acute increase in IOP is observed during RE in healthy adults with fluctuations of varying magnitude. Factors that independently increase IOP during RE include exercises involving larger muscle mass, heavy loads, greater set duration, and when the VM or breath-hold is performed.

Cluster Sets to Prescribe Interval Resistance Training: A Potential Method to Optimise Resistance Training Safety, Feasibility and Efficacy in Cardiac Patients.

The integration of resistance training for cardiac patients leads to important health outcomes that are not optimally obtained with aerobic exercise; these include an increase in muscle mass, maintenance of bone mineral density, and improvements in muscular fitness parameters. Despite the proliferation of evidence supporting resistance exercise in recent decades, the implementation of resistance training is underutilised, and prescription is often sub-optimal in cardiac patients. This is frequently associated with safety concerns and inadequate methods of practical exercise prescription. This review discusses the potential application of cluster sets to prescribe interval resistance training in cardiac populations. The addition of planned, regular passive intra-set rest periods (cluster sets) in resistance training (i.e., interval resistance training) may be a practical solution for reducing the magnitude of haemodynamic responses observed with traditional resistance training. This interval resistance training approach may be a more suitable option for cardiac patients. Additionally, many cardiac patients present with impaired exercise tolerance; this model of interval resistance training may be a more suitable option to reduce fatigue, increase patient tolerance and enhance performance to these workloads. Practical strategies to implement interval resistance training for cardiac patients are also discussed. Preliminary evidence suggests that interval resistance training may lead to safer acute haemodynamic responses in cardiac patients. Future research is needed to determine the efficacy and feasibility of interval resistance training for health outcomes in this population.

Exercise as therapy for neurodevelopmental and cognitive dysfunction in people with a Fontan circulation: A narrative review.

People with a Fontan circulation are at risk of neurodevelopmental delay and disability, and cognitive dysfunction, that has significant implications for academic and occupational attainment, psychosocial functioning, and overall quality of life. Interventions for improving these outcomes are lacking. This review article discusses current intervention practices and explores the evidence supporting exercise as a potential intervention for improving cognitive functioning in people living with a Fontan circulation. Proposed pathophysiological mechanisms underpinning these associations are discussed in the context of Fontan physiology and avenues for future research are recommended.

Effect of resistance training on local muscle endurance in middle-aged and older adults: A systematic review with meta-analysis and meta-regression.

BACKGROUND: Local muscle endurance (LME) is a significant indicator of muscle health and function in middle-aged and older adults. However, resistance training (RT) practices which optimise performance in this population are currently unclear. This study examined: 1) the effect of RT on LME; and 2) the impact of acute resistance exercise program variables on LME in middle-aged and older adults. METHODS: Five electronic databases were searched using terms related to RT, LME, and older adults. Random effects (Hedges' g) meta-analyses were undertaken to estimate the effect of RT on upper and lower body LME assessed via maximal repetitions during an isotonic test. The impact of resistance exercise program variables on LME effects was explored using meta-regression analyses. RESULTS: Fifteen studies met the inclusion criteria for this review. Upon sensitivity analysis, one study was removed. Large effects favoured RT for LME of the upper body (g = 1.10, p < 0.001) and lower body (g = 1.18, p < 0.001). Large effects on LME from RT were found irrespective of training intensity or other resistance exercise program variables. Moderate heterogeneity and publication bias were found in most analyses. DISCUSSION: RT is an effective means for improving LME in middle-aged and older adults. Optimal training characteristics have not been defined by this review, as improvement in LME was unrelated to RT volume or loading intensity. Caution is warranted when interpreting the findings due to heterogeneity and bias present in existing literature. Additional studies are needed with direct comparisons of various training techniques.

Effect of Blood Flow Restriction during the Rest Periods of Squats on Accuracy of Estimated Repetitions to Failure.

This study investigated the impact of resistance training with blood flow restriction during rest (BFRrest) on the accuracy of estimated repetitions to failure (ERF). It also explored associations between error in ERF and mean concentric velocity (MCV) along with physiological responses. In a randomised cross-over study, 18 male trainers (23.4 ± 2.7 years) performed three sets of squats at 70% of their one-repetition maximum until failure. One session integrated BFRrest, while another employed traditional passive inter-set rest (TRAD) during the 3 min inter-set rest intervals. Cardiorespiratory and metabolic measures were taken in the inter-set recovery periods. The results revealed no significant differences between BFRrest and TRAD in terms of ERF and error in ERF. A notable set effect for ERF was observed, with a greater ERF during set 1 compared to sets 2 and 3 (p < 0.001). Additionally, a lower error in ERF was observed during sets 2 and 3 compared to set 1 (p < 0.001). Error in ERF were strongly associated with the respiratory exchange ratio, and moderately associated with end-tidal carbon dioxide partial pressure, carbon dioxide output, and MCV variables. Notably, the precision of ERF seems to be predominantly influenced by indicators of physiological stress rather than the incorporation of BFRrest.

Scaling Peak Oxygen Consumption for Body Size and Composition in People With a Fontan Circulation.

Background Peak oxygen consumption (peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$) is traditionally divided ("ratio-scaled") by body mass (BM) for clinical interpretation. Yet, it is unknown whether ratio-scaling to BM can produce a valid size-independent expression of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ in people with a Fontan circulation. Furthermore, people with a Fontan circulation have deficits in lean mass, and it is unexplored whether using different measures of body composition may improve scaling validity. The objective was to assess the validity of different scaling denominators (BM, stature, body surface area, fat-free mass, lean mass, and appendicular lean mass using ratio and allometric scaling). Methods and Results Eighty-nine participants (age: 23.3±6.7 years; 53% female) with a Fontan circulation had their cardiorespiratory fitness and body composition measured by cardiopulmonary exercise testing and dual-energy x-ray absorptiometry. Ratio and allometric (log-linear regression) scaling was performed and Pearson correlations assessed scaling validity. Scaling denominators BM (r=-0.25, P=0.02), stature (r=0.46, P<0.001), and body surface area (0.23, P=0.03) were significantly correlated with their respective ratio-scaled expressions of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$, but fat-free mass, lean mass, or appendicular lean mass were not (r≤0.11; R2=1%). Allometrically expressed peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ resulted in no significant correlation with any scaling denominator (r=≤0.23; R2=≤4%). Conclusions The traditional and accepted method of ratio-scaling to BM is invalid because it fails to create a size-independent expression of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ in people with a Fontan circulation. However, ratio-scaling to measures of body composition (fat-free mass, lean mass, and appendicular lean mass) and allometric techniques can produce size-independent expressions of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ in people with a Fontan circulation.

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.

Sexual Function in Men Living With a Fontan Circulation.

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.

The "Super-Fontan" Phenotype: Characterizing Factors Associated With High Physical Performance.

Background: People with a Fontan circulation usually have moderately impaired exercise performance, although a subset have high physical performance ("Super-Fontan"), which may represent a low-risk phenotype. Methods: People with a "Super-Fontan" phenotype were defined as achieving normal exercise performance [≥80% predicted peak oxygen uptake (VO2) and work rate] during cardiopulmonary exercise testing (CPET) and were identified from the Australian and New Zealand Fontan Registry. A Fontan control group that included people with impaired exercise performance (<80% predicted VO2 or work rate) was also identified based on a 1:3 allocation ratio. A subset of participants were prospectively recruited and completed a series of physical activity, exercise self-efficacy, and health-related quality of life questionnaires. Results: Sixty CPETs ("Super-Fontan", n = 15; control, n = 45) were included. A subset ("Super-Fontan", n = 10; control, n = 13) completed a series of questionnaires. Average age was 29 ± 8 years; 48% were males. Exercise capacity reflected by percent predicted VO2 was 67 ± 17% in the entire cohort. Compared to the "Super-Fontan" phenotype, age at Fontan completion was higher in controls (4.0 ± 2.9 vs. 7.2 ± 5.3 years, p = 0.002). Only one (7%) person in the "Super-Fontan" group had a dominant right ventricle compared to 15 (33%) controls (p = 0.043). None of those in the "Super-Fontan" group were obese, while almost a quarter (22%) of controls were obese based on body mass index (p = 0.046). Lung function abnormalities were less prevalent in the "Super-Fontan" group (20 vs. 70%, p = 0.006). Exercise self-efficacy was greater in the "Super-Fontan" group (34.2 ± 3.6 vs. 27.9 ± 7.2, p = 0.02). Self-reported sports participation and physical activity levels during childhood and early adulthood were higher in the "Super-Fontan" group (p < 0.05). The total average time spent participating in structured sports and physical activity was 4.3 ± 2.6 h/wk in the "Super-Fontan" group compared to 2.0 ± 3.0 h/wk in controls, p = 0.003. There were no differences in self-reported current total physical activity score or health-related quality of life between groups (p ≥ 0.05). Conclusions: The "Super-Fontan" phenotype is associated with a healthy weight, lower age at Fontan completion, better exercise self-efficacy, and higher overall levels of sport and physical activity participation during physical development.

Decline Is Not Inevitable: Exercise Capacity Trajectory in an Australian and New Zealand Fontan Cohort.

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 VO2peak) 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 VO2peak 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 VO2peak 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 VO2peak 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.

Chronic Effects of Altering Resistance Training Set Configurations Using Cluster Sets: A Systematic Review and Meta-Analysis.

BACKGROUND: The acute responses to cluster set resistance training (RT) have been demonstrated. However, as compared to traditional sets, the effect of cluster sets on muscular and neuromuscular adaptations remains unclear. OBJECTIVE: To compare the effects of RT programs implementing cluster and traditional set configurations on muscular and neuromuscular adaptations. METHODS: Systematic searches of Embase, Scopus, Medline and SPORTDiscus were conducted. Inclusion criteria were: (1) randomized or non-randomized comparative studies; (2) publication in English; (3) participants of all age groups; (4) participants free of any medical condition or injury; (5) cluster set intervention; (6) comparison intervention utilizing a traditional set configuration; (7) intervention length ≥ three weeks and (8) at least one measure of changes in strength/force/torque, power, velocity, hypertrophy or muscular endurance. Raw data (mean ± SD or range) were extracted from included studies. Hedges' g effect sizes (ES) ± standard error of the mean (SEM) and 95% confidence intervals (95% CI) were calculated. RESULTS: Twenty-nine studies were included in the meta-analysis. No differences between cluster and traditional set configurations were found for strength (ES = - 0.05 ± 0.10, 95% CI - 0.21 to 0.11, p = 0.56), power output (ES = 0.02 ± 0.10, 95% CI - 0.17 to 0.20, p = 0.86), velocity (ES = 0.15 ± 0.13, 95% CI - 0.10 to 0.41, p = 0.24), hypertrophy (ES = - 0.05 ± 0.14, 95% CI - 0.32 to 0.23, p = 0.73) or endurance (ES = - 0.07 ± 0.18, 95% CI - 0.43 to 0.29, p = 0.70) adaptations. Moreover, no differences were observed when training volume, cluster set model, training status, body parts trained or exercise type were considered. CONCLUSION: Collectively, both cluster and traditional set configurations demonstrate equal effectiveness to positively induce muscular and neuromuscular adaptation(s). However, cluster set configurations may achieve such adaptations with less fatigue development during RT which may be an important consideration across various exercise settings and stages of periodized RT programs.

Impact of adiposity on clinical outcomes in people living with a Fontan circulation.

BACKGROUND: To assess the association between body composition and the risk of adverse outcomes in Fontan patients. METHODS: Participants from the Australian and New Zealand Fontan Registry with dual-energy X-ray absorptiometry scans were included. Appendicular lean mass (ALM), appendicular lean mass index (ALM divided by height squared; ALMI) and total body fat mass percentage (%BF) were calculated. ALMI and %BF z-scores were derived using age- and sex-matched reference ranges. The primary outcome was Fontan failure (death, transplantation, New York Heart Association functional class III/IV, protein-losing enteropathy, and plastic bronchitis) or moderate-or-severe ventricular dysfunction. RESULTS: 144 patients were included. Mean %BF was 29% (SD 10) with 50% having increased adiposity. Mean ALMI z-score was -1.4 (SD 1.1); one third of patients had skeletal muscle deficiency (ALMI z-score < -1 and -2) and another third had Fontan-associated myopaenia (ALMI z-score < -2). Age and %BF were associated with the risk of the endpoint in univariable regression (age: HR 1.09 per year, 95% CI 1.02-1.17, p = 0.01; %BF: HR 1.08, 95% CI 1.01-1.17, p = 0.03). On multivariable regression, every 1% increase in %BF was associated with a 10% increased risk of reaching the clinical endpoint (HR 1.10, 95% CI 1.01-1.19; p = 0.03). ALM was not associated with the endpoint (HR 1.02 per kg, 95% CI 0.88-1.20, p = 0.77). CONCLUSIONS: Increased adiposity is associated with higher risk for adverse outcomes. Prospective studies to assess lifestyle interventions to optimise body composition should be prioritised.

Exercise Testing and Training in Adults With Congenital Heart Disease: A Surgical Perspective.

In the current era, the majority of children born with congenital heart disease (CHD) will survive well into adulthood because of major advances in surgical techniques, as well as in critical and medical care. However, reoperation and palliative surgical interventions are increasingly common in the adults with CHD. Tools to risk stratify patients effectively and therapies to improve outcomes are required to optimize the management of adult patients with CHD during the preoperative and postoperative periods and beyond. Exercise testing is an invaluable tool to guide risk stratification. In addition, exercise training in patients with CHD may decrease postoperative complications by enhancing physiological reserve and also has an important role in physical rehabilitation. This review aims to provide individualized recommendations on exercise prescription in patients with CHD in the preoperative and postoperative settings. The response to exercise testing and prognostic implications is also discussed.

Exercise Intolerance, Benefits, and Prescription for People Living With a Fontan Circulation: The Fontan Fitness Intervention Trial (F-FIT)-Rationale and Design.

Background: Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to optimize the circulation have not been established. Exercise intolerance affects the majority of the population and is associated with worse prognosis. Historically, people living with a Fontan circulation were advised to avoid physical activity, but a small number of heterogenous, predominantly uncontrolled studies have shown that exercise training is safe-and for unique reasons, may even be of heightened importance in the setting of Fontan physiology. The mechanisms underlying improvements in aerobic exercise capacity and the effects of exercise training on circulatory and end-organ function remain incompletely understood. Furthermore, the optimal methods of exercise prescription are poorly characterized. This highlights the need for large, well-designed, multi-center, randomized, controlled trials. Aims and Methods: The Fontan Fitness Intervention Trial (F-FIT)-a phase III clinical trial-aims to optimize exercise prescription and delivery in people with a Fontan circulation. In this multi-center, randomized, controlled study, eligible Fontan participants will be randomized to either a 4-month supervised aerobic and resistance exercise training program of moderate-to-vigorous intensity followed by an 8-month maintenance phase; or usual care (control group). Adolescent and adult (≥16 years) Fontan participants will be randomized to either traditional face-to-face exercise training, telehealth exercise training, or usual care in a three-arm trial with an allocation of 2:2:1 (traditional:telehealth:control). Children (<16 years) will be randomized to either a physical activity and exercise program of moderate-to-vigorous intensity or usual care in a two-arm trial with a 1:1 allocation. The primary outcome is a change in aerobic exercise capacity (peak oxygen uptake) at 4-months. Secondary outcomes include safety, and changes in cardiopulmonary exercise testing measures, peripheral venous pressure, respiratory muscle and lung function, body composition, liver stiffness, neuropsychological and neurocognitive function, physical activity levels, dietary and nutritional status, vascular function, neurohormonal activation, metabolites, cardiac function, quality of life, musculoskeletal fitness, and health care utilization. Outcome measures will be assessed at baseline, 4-months, and 12-months. This manuscript will describe the pathophysiology of exercise intolerance in the Fontan circulation and the rationale and protocol for the F-FIT.

Inspiratory Muscle Training Improves Inspiratory Muscle Strength and Functional Exercise Capacity in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: A Pilot Randomised Controlled Study.

BACKGROUND: Exercise intolerance is present even in the early stages of pulmonary arterial hypertension (PAH) and is associated with poorer prognosis. Respiratory muscle dysfunction is common and may contribute to exercise limitation. We sought to investigate the effects of inspiratory muscle training (IMT) to improve exercise capacity in PAH. METHODS: Adults with PAH were prospectively recruited and randomly assigned to either IMT or a control group. At baseline and after 8 weeks, assessment of respiratory muscle function, pulmonary function, neurohormonal activation, 6-minute walk distance and cardiopulmonary exercise testing variables were conducted. Inspiratory muscle strength was assessed by maximal static inspiratory pressure (PImax). The IMT group performed two cycles of 30 breaths at 30-40% of their PImax 5 days a week for 8 weeks. RESULTS: Twelve (12) PAH patients (60±14 years, 10 females) were recruited and randomised (six in the IMT group and six in the control group). After 8 weeks, the IMT group improved PImax by 31 cmH2O compared with 10 cmH2O in controls, p=0.02. Following IMT, 6-minute walk distance improved by 24.5 m in the IMT group and declined by 12 m in the controls (mean difference 36.5 m, 95% CI 3.5-69.5, p=0.03). There was no difference in peak oxygen uptake between-groups (mean difference 0.4 mL/kg/min, 95% CI -2.6 to 3.4, p=0.77). There was no difference in the mean change between-groups in neurohormonal activation or pulmonary function. CONCLUSION: In this pilot randomised controlled study, IMT improved PImax and 6-minute walk distance in PAH patients.

Body Composition in Young Adults Living With a Fontan Circulation: The Myopenic Profile.

Background We sought to characterize body composition abnormalities in young patients living with a Fontan circulation and explore potential pathophysiologic associations. Methods and Results Twenty-eight patients with a Fontan circulation were prospectively recruited in this cross-sectional study. Participants underwent cardiopulmonary exercise testing, dual-energy X-ray absorptiometry, echocardiography, and biochemical assessment. Mean age was 26±7 years. Skeletal muscle mass, estimated by appendicular lean mass index Z score, was reduced compared with reference data (-1.49±1.10, P<0.001). Percentage body fat Z score overall was within normal range (0.23±1.26, P=0.35), although 46% had elevated adiposity. Those with reduced skeletal muscle mass (appendicular lean mass index Z score of -1 or lower) had lower percent predicted oxygen pulse (55±15 versus 76±16%, P=0.002). Overall agreement between body mass index and dual-energy X-ray absorptiometry to assess adiposity was fair only (weighted [linear] κ coefficient: 0.53; 95% CI, 0.34-0.73) and slight in the setting of muscle mass deficiency (weighted κ coefficient: 0.32; 95% CI, 0.13-0.50). Appendicular lean mass was independently associated with absolute peak VO2 (ß=70.6 mL/min, P=0.001). Appendicular lean mass index Z score was inversely associated with hemoglobin (r=-0.4, P=0.04), and the degree of muscle deficit was associated with ventricular systolic impairment. Conclusions Young patients with a Fontan circulation have a body composition characterized by reduced skeletal muscle mass, which is associated with peak exercise capacity. Increased adiposity is common despite a normal body mass index. Low skeletal muscle mass is associated with systolic dysfunction and compensatory erythrocytosis.