Body Composition Abnormalities in Adults with a Fontan Circulation

Tran, Derek; D’Ambrosio, Paolo; Verrall, Charlotte E; Attard, Chantal; Briody, Julie; D’Souza, Mario; Singh, M. Fiatarone; Ayer, Julian; d’Udekem, Yves; Twigg, Stephen M.; Davis, Glen M.; Celermajer, David S.; Cordina, Rachael

doi:10.1016/j.hlc.2019.06.038

Cited by 3 publications

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“…Compliance was high, with a median training session attendance of 33 (32)(33)(34) out of 36 sessions. In total, 27 patients completed the exercise intervention.…”

Section: Compliance and Drop-outsmentioning

confidence: 99%

A 12-week lifestyle intervention: effects on fatigue, fear, and nutritional status in children with a Fontan circulation

Scheffers,

Helbing,

Pereira

et al. 2023

Front. Pediatr.

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IntroductionChildren and adolescents with a Fontan circulation are less physically active compared to healthy peers. In the current study, effects of a 12-week lifestyle intervention on fatigue, fears regarding exercise, caloric intake, rest energy expenditure (REE), and body composition were measured in children with a Fontan circulation.MethodsThis study was a semi-cross-over randomized controlled trial. The lifestyle intervention consisted of a 12-week high-weight resistance training (three supervised training sessions a week) supported by high-protein diet (>2 g/kg) and tailored recommended caloric intake. Fatigue (measured by the validated PedsQol Multidimensional Fatigue Scale), fears regarding exercise (measured on a fear thermometer), REE (measured using indirect calorimetry), caloric intake and body composition using air displacement plethysmography, and four-skinfold method were measured before and after the intervention and control period.ResultsTwenty-seven pediatric Fontan patients, median age 12.9 years (IQR: 10.5–16.2), of the included 28 patients successfully completed the program. Before training, both child- and parent-reported levels of fatigue were significantly worse on all domains (general, sleep/rest, and cognitive fatigue) compared to healthy peers. After training, parent-reported fatigue significantly improved on the general and cognitive fatigue domains [effect size +16 points (7–25), p < 0.001, and +10 points (2–17), p = 0.015, compared to the control period]. Before training, fear regarding exercise scored on the fear thermometer was low for both children and parents (median score 1 and 2, respectively, on a scale of 0–8). After training, child-reported fear decreased further compared to the control period [effect size −1.4 points (−2.3 to −0.6), p = 0.001]. At baseline, children had increased REE +12% compared to reference values, which did not change after exercise. Children ate an average of 637 calories below recommended intake based on REE, caloric deficit became smaller after the intervention, and protein intake increased compared to the control period [−388 calories (−674 to −102), p = 0.008, and +15 g (0.4–30), p = 0.044]. Body fat percentage did not change significantly.ConclusionA 12-week lifestyle intervention improved parent-reported fatigue symptoms in the children, further decreased child-reported fears, and increased caloric and protein intake.

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“…Compliance was high, with a median training session attendance of 33 (32)(33)(34) out of 36 sessions. In total, 27 patients completed the exercise intervention.…”

Section: Compliance and Drop-outsmentioning

confidence: 99%

A 12-week lifestyle intervention: effects on fatigue, fear, and nutritional status in children with a Fontan circulation

Scheffers,

Helbing,

Pereira

et al. 2023

Front. Pediatr.

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A Multi-system Disorder: Extracardiac Complications

Cordina,

Celermajer

2023

Univentricular Congenital Heart Defects and the Fontan Circulation

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Body composition in patients with Fontan physiology: a systematic review

van den Berg,

Pos,

Scheffers

et al. 2023

Eur J Pediatr

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Fontan circulation is a highly abnormal circulatory state that may affect various organ systems. The effect on body composition is an important factor to assess the condition of the patient. This systematic review assesses body composition and possibly related adverse outcomes in patients with a Fontan circulation, to provide an overview of current insights. Studies evaluating body composition by compartment (either fat mass or lean/muscle mass) in Fontan patients published up to April 2023 were included in this systematic review. Of 1392 potential studies, 18 studies met the inclusion criteria. In total, body composition measurements of 774 Fontan patients were included. Body composition was measured using dual-energy X-ray absorptiometry (DXA) (n = 12), bioelectrical impedance analysis (BIA) (n = 5), computer tomography (CT) (n = 1), or magnetic resonance imaging (MRI) (n = 1). All studies reported a normal body mass index (BMI) in Fontan patients, compared to controls. Five out of nine studies reported significantly higher body fat values, and twelve out of fifteen studies reported significantly lower muscle or lean mass values in the Fontan population compared to the healthy population. Unfavorable body composition in Fontan patients was associated with decreased exercise capacity, worse cardiac function, and adverse outcomes including hospital admissions and death. Conclusions: Despite having a normal BMI, Fontan patients have an increased fat mass and decreased muscle mass or lean mass compared to the healthy population. This unfavorable body composition was associated with various adverse outcomes, including a decreased exercise capacity and worse cardiac function. What is Known:• Patients with a Fontan circulation have a decreased exercise capacity compared to healthy peers, an unfavorable body composition might be a contributor to their impaired exercise capacity. What is New:• Fontan patients are predisposed to an unfavorable body composition, characterized by increased fat mass and decreased muscle mass accompanied by a normal BMI compared to the healthy population.• Among others, unfavorable body composition was associated with decreased exercise capacity, cardiac function, and increased morbidity in patients with a Fontan circulation.

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Body Composition Abnormalities in Adults with a Fontan Circulation

Cited by 3 publications

References 0 publications

A 12-week lifestyle intervention: effects on fatigue, fear, and nutritional status in children with a Fontan circulation

A 12-week lifestyle intervention: effects on fatigue, fear, and nutritional status in children with a Fontan circulation

A Multi-system Disorder: Extracardiac Complications

Body composition in patients with Fontan physiology: a systematic review

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