Dataset Information

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

ABSTRACT: 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] ? coefficient: 0.53; 95% CI, 0.34-0.73) and slight in the setting of muscle mass deficiency (weighted ? coefficient: 0.32; 95% CI, 0.13-0.50). Appendicular lean mass was independently associated with absolute peak VO2 (?=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.

SUBMITTER: Tran D

PROVIDER: S-EPMC7428525 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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

Tran Derek D D'Ambrosio Paolo P Verrall Charlotte E CE Attard Chantal C Briody Julie J D'Souza Mario M Fiatarone Singh Maria M Ayer Julian J d'Udekem Yves Y Twigg Stephen S Davis Glen M GM Celermajer David S DS Cordina Rachael R

Journal of the American Heart Association 20200415 8

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 app ...[more]

PMID: 32290749

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Project description:Abstract Objectives To examine the association between meal habits (i.e., eating breakfast every day, usually ≥80% fullness at breakfast, and not eating snacks at night), body composition and the subsequent changes in mood status in young adults. Methods This is a longitudinal follow-up study based on a randomized trial (ClinicalTrials.gov Identifier: NCT03828812). Young adults were recruited based in a university campus in Taipei City, Taiwan. In total, 125 participants were recruited and 103 completed the follow-ups. Measured body composition (TANITA BC-418) and moods (Depression Anxiety Stress Scale, DASS) were assessed at baseline, the third month and 15th month from baseline. Baseline meal habits were based on the participants’ recall of their diets in the month before interview. We used mixed-effect model to test the association of baseline total body fat percentage and trunk body fat percentage and moods during follow-ups, adjusting for age, sex, baseline body mass index (BMI), food security status. Results After controlling for sex, age, BMI, and food security status at baseline, having breakfast every day at baseline was associated with 2.04 (se = 0.78, P = 0.0098) lower anxiety score and with 2.58 (se = 1.26, P = 0.0409) lower stress score at baseline. Meal habits were not associated with the subsequent changes in mood status. As for body composition, one % of trunk body fat replacing one % of non-trunk body fat at baseline was associated with 1.19 (se = 0.57, P = 0.0381) higher depression score, 1.02 (se = 0.47, P = 0.0327) higher anxiety score, 2.06 (se = 0.76, P = 0.0075) higher anxiety score at baseline. One % of non-trunk body fat percentage was associated with 2.60 (se = 1.02, P = 0.0116) lower anxiety score, as trunk body fat % is controlled. Baseline body fat composition parameters were not associated with the temporal changes in mood scores. The significance patterns were similar after adjusting for baseline physical activity level, assessed by International Physical Activity Questionnaire. Conclusions Breakfast habits and body composition, especially trunk body fat, was associated with university-based young adults’ emotional health status. Funding Sources Ministry of Science and Technology, Taiwan.

Project description:BackgroundThe molecular mechanisms underlying Fontan associated liver disease (FALD) remain largely unknown. We aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes.MethodsThis retrospective cohort study included adults with the Fontan circulation at the Ahmanson/UCLA Adult Congenital Heart Disease Center. Clinical, laboratory, imaging and hemodynamic data prior to the liver biopsy were extracted from medical records. Patients were classified into early (F1-F2) or advanced fibrosis (F3-F4). RNA was isolated from formalin-fixed paraffin embedded liver biopsy samples; RNA libraries were constructed using rRNA depletion method and sequencing was performed on Illumina Novaseq 6000. Differential gene expression and gene ontology analyses were carried out using DESeq2 and Metascape. Medical records were comprehensively reviewed for a composite clinical outcome which included decompensated cirrhosis, hepatocellular carcinoma, liver transplantation, protein-losing enteropathy, chronic kidney disease stage 4 or higher, or death.ResultsPatients with advanced fibrosis had higher serum BNP levels and Fontan, mean pulmonary artery and capillary wedge pressures. The composite clinical outcome was present in 23 patients (22%) and was predicted by age at Fontan, right ventricular morphology and presence of aortopulmonary collaterals on multivariable analysis. Samples with advanced fibrosis had 228 up-regulated genes compared to early fibrosis. Samples with the composite clinical outcome had 894 up-regulated genes compared to those without it. A total of 136 up-regulated genes were identified in both comparisons and these genes were enriched in cellular response to cytokine stimulus, response to oxidative stress, VEGFA-VEGFR2 signaling pathway, TGF-beta signaling pathway, and vasculature development.ConclusionsPatients with FALD and advanced liver fibrosis or the composite clinical outcome exhibit up-regulated genes including pathways related to inflammation, congestion, and angiogenesis. This adds further insight into FALD pathophysiology.

Project description:Aims: Patients with a congenital heart condition palliated with a Fontan circulation generally present with decreased exercise capacity due to impaired cardiopulmonary function. Recently, a study in patients with a Fontan circulation reported evidence for abnormal vascular endothelial function in leg muscle. We investigated if abnormal skeletal muscle hemodynamics during exercise play a role in the limited exercise tolerance of Fontan patients. If so, abnormalities in intramuscular energy metabolism would be expected both during exercise as well as during post-exercise metabolic recovery. Methods: In a young patient with a Fontan circulation and his healthy twin brother we studied the in vivo dynamics of energy- and pH-balance in quadriceps muscle during and after a maximal in-magnet bicycling exercise challenge using 31-phosphorus magnetic resonance spectroscopy. An unrelated age-matched boy was also included as independent control. Results: Quadriceps phosphocreatine (PCr) depletion during progressive exercise was more extensive in the Fontan patient than in both controls (95% vs. 80%, respectively). Importantly, it failed to reach an intermittent plateau phase observed in both controls. Quadriceps pH during exercise in the Fontan patient fell 0.3 units at low to moderate workloads, dropping to pH 6.6 at exhaustion. In both controls quadriceps acidification during exercise was absent but for the maximal workload in the twin brother (pH 6.8). Post-exercise, the rate of metabolic recovery in the Fontan patient and both controls was identical (time constant of PCr recovery 32 ± 4, 31 ± 2, and 28 ± 4 s, respectively). Conclusion: Homeostasis of quadriceps energy- and pH-balance during a maximal exercise test failed in the Fontan patient in comparison to his healthy twin brother and an age-matched independent control. Post-exercise metabolic recovery was normal which does not support the contribution of significant endothelial dysfunction affecting adequate delivery of oxidative substrates to the muscle to the lower exercise capacity in this particular Fontan patient. These results suggest that mitochondrial ATP synthetic capacity of the quadriceps muscle was intact but cardiac output to the leg muscles during exercise was insufficient to meet the muscular demand for oxygen. Therefore, improving cardiac output remains the main therapeutic target to improve exercise capacity in patients with a Fontan circulation.

Dataset Information

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

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

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