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Body composition in patients with Fontan physiology: a systematic review

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EUROPEAN JOURNAL OF PEDIATRICS
卷 -, 期 -, 页码 -

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SPRINGER
DOI: 10.1007/s00431-023-05100-2

关键词

Fontan procedure; Body composition; Muscle mass; Fat mass; Single ventricle

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Fontan circulation is an abnormal circulatory state that affects organ systems. This review examines body composition and related outcomes in Fontan patients. The review included 18 studies with a total of 774 Fontan patients. The results showed increased fat mass and decreased muscle or lean mass in Fontan patients, despite having a normal BMI. This unfavorable body composition was associated with adverse outcomes such as decreased exercise capacity and worse cardiac function.
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.

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