Impaired knee extension muscle strength in adolescents but not in children with Fontan circulation

Sandberg, Camilla; Frisk, Emelie; Hansson, Lotta; Isberg, Annika; Hedlund, Eva Rylander; Sjöberg, Gunnar; Rydberg, Annika

doi:10.1017/s1047951120001675

Cited by 8 publications

(22 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Handgrip strength, dynamic muscular strength, and muscular endurance have all been reported to be lower in people with a Fontan circulation than in healthy, age-matched controls and are associated with reduced skeletal muscle mass (85,(87)(88)(89). In a series with a heterogenous sample of CHD lesions (30% Fontan), when strength was indexed to lean mass, there was no difference in isometric strength compared with healthy controls (90).…”

Section: Skeletal Muscle Functionmentioning

confidence: 99%

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

et al. 2022

View full text Add to dashboard Cite

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.

show abstract

Section: Skeletal Muscle Functionmentioning

confidence: 99%

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

et al. 2022

View full text Add to dashboard Cite

show abstract

“…Lean muscle mass Z-scores were not related to age for Fontan patients ( 62 ). However, isometric knee extension muscle strength is impaired for adolescent Fontan patients, but not during childhood, which suggests that muscular impairment increases with age ( 67 ). Markers of neurohormonal activation, sex hormones, and inflammatory mediators did not relate to lean muscle mass, implying the role of these factors is limited in the pathogenesis in myopenia ( 63 ).…”

Section: Resultsmentioning

confidence: 99%

Fontan Circulation Associated Organ Abnormalities Beyond the Heart, Lungs, Liver, and Gut: A Systematic Review

Ritmeester

Veger

Ven

et al. 2022

Front. Cardiovasc. Med.

View full text Add to dashboard Cite

IntroductionPatients with a Fontan circulation are at risk for sequelae of Fontan physiology during follow-up. Fontan physiology affects all organ systems and an overview of end-organ damage is needed.MethodsWe performed a systematic review of abnormalities in multiple organ systems for patients with a longstanding Fontan circulation. We searched online databases for articles describing abnormalities in multiple organ systems. Cardio-pulmonary abnormalities, protein losing enteropathy, and Fontan associated liver disease have already extensively been described and were excluded from this systematic review.ResultsOur search returned 5,704 unique articles. After screening, we found 111 articles relating to multiple organ systems. We found abnormalities in, among others, the nervous system, pituitary, kidneys, and musculoskeletal system. Pituitary edema—relating to the unique pituitary vasculature- may affect the thyroid axis. Renal dysfunction is common. Creatinine based renal function estimates may be inappropriate due to myopenia. Both lean muscle mass and bone mineral density are decreased. These abnormalities in multiple organ systems may be related to Fontan physiology, cyanosis, iatrogenic factors, or lifestyle.ConclusionsHealth care providers should be vigilant for hypothyroidism, visual or hearing deficits, and sleep disordered breathing in Fontan patients. We recommend including cystatin C for assessment of renal function. This review may aid health care providers and guide future research.Systematic Review Registration:https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021232461, PROSPERO, identifier: CRD42021232461.

show abstract

“…Table 1 presents the selected articles and their main characteristics. Twelve articles assessed peripheral muscle strength 18,[26][27][28][29][30][31][32][33][34][35][36] and three assessed respiratory muscle strength. 5,8,37 The total sample size of the included studies was 1769.…”

Section: Resultsmentioning

confidence: 99%

“…5,8,37 The total sample size of the included studies was 1769. 5,8,18,[26][27][28][29][30][31][32][33][34][35][36][37] The studies comparing patients with CHD and healthy controls included 1202 participants in total, 27,33,34,36 and the studies only included patients with CHD had a total number of 567 participants. 5,8,18,26,[28][29][30][31][32]35,37 Regarding the studies with evaluation only of patients with CHD, the majority had undergone at least one surgical procedure and submitted it to cardiac training or rehabilitation.…”

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Peripheral and respiratory muscle strength in children and adolescents with CHD: systematic review and meta-analysis

et al. 2022

View full text Add to dashboard Cite

Patients with CHD are less active if compared with controls and have limited functional capacity, related to muscle weakness and fatigue. The aim of this study was to evaluate the peripheral and respiratory muscle strength of children and adolescents with CHD with systematic review and meta-analysis. The review included observational and randomised control trial studies which evaluated peripheral and respiratory muscle strength in children and adolescents with CHD under 18 years old. The peripheral muscle strength was evaluated through dynamometry and respiratory muscle strength through manovacuometry. In studies that compared patients with CHD and respective control groups, it was possible to perform a meta-analysis. A total of 5634 articles met the criteria of eligibility, 15 were included in the systematic review, and 4 were included in the meta-analysis. Twelve studies assessed peripheral muscle strength with a reduction in patients with CHD. In the meta-analysis, patients with CHD had lower muscle strength than controls (−34.07 nm; 95% CI, −67.46 to −0.68; I2 47%; p for heterogeneity = 0.05), and the meta-analysis of the handgrip muscle strength showed no significant difference between patients with CHD and controls (0.08 nm; 95% CI, −6.39 to 6.55; I2 98%; p for heterogeneity <0.00001). The meta-analysis in the present study showed lower limb muscle strength in patients with CHD in comparison to controls. In contrast, no difference was found regarding hand grip strength. Also, the review showed lower respiratory muscle strength in patients with CHD, yet no meta-analysis was possible to perform.

show abstract

Impaired knee extension muscle strength in adolescents but not in children with Fontan circulation

Cited by 8 publications

References 24 publications

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

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

Fontan Circulation Associated Organ Abnormalities Beyond the Heart, Lungs, Liver, and Gut: A Systematic Review

Peripheral and respiratory muscle strength in children and adolescents with CHD: systematic review and meta-analysis

Contact Info

Product

Resources

About