Low serum 25-hydroxyvitamin D levels and secondary hyperparathyroidism in Fontan patients

Holler, Finn; Hannes, Tobias; Germund, Ingo; Emmel, Mathias; Hoyer‐Kuhn, Heike; Khalil, Markus; Sreeram, Narayanswami; Cate, Floris E.A. Udink ten

doi:10.1017/s1047951115001444

Cited by 19 publications

(15 citation statements)

References 41 publications

(134 reference statements)

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“…56 Positive correlations also existed between increased arterial stiffness and inflammation, which might be explained by disturbed vitamin D/parathormone interactions frequent in this patient group. 56,57 Vascular stiffening also occurs in cerebral arteries in young Fontan patients, suggesting altered (arterial) vascular properties in general or at least in vessels neighbouring the heart. 58 Local assessment of arterial stiffness would be desirable.…”

Section: Discussionmentioning

confidence: 99%

Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

Michel

Dubowy

Zlamy

et al. 2020

Therapeutic Advances in Chronic Disease

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Background: Patients with a Fontan circulation have altered cholesterol and lipoprotein values. We analysed small organic molecules in extended phopsholipid and acylcarnitine metabolic pathways (‘metabolomes’) in adult Fontan patients with a dominant left ventricle, seeking differences between profiles in baseline and Fontan circulations. Methods: In an observational matched cross-sectional study, we compared phosphatidylcholine (PC), sphingomyelin (SM), and acylcarnitine metabolomes (105 analytes; AbsoluteIDQ® p180 kit (Biocrates Life Sciences AG, Innsbruck, Austria) in 20 adult Fontan patients having a dominant left ventricle with those in 20 age- and sex-matched healthy controls. Results: Serum levels of total PC ( q-value 0.01), total SM ( q-value 0.0002) were significantly lower, and total acylcarnitines ( q-value 0.02) were significantly higher in patients than in controls. After normalisation of data, serum levels of 12 PC and 1 SM Fontan patients were significantly lower ( q-values <0.05), and concentrations of 3 acylcarnitines were significantly higher than those in controls ( q-values <0.05). Conclusion: Metabolomic profiling can use small specimens to identify biomarker patterns that track derangement in multiple metabolic pathways. The striking alterations in the phospholipid and acylcarnitine metabolome that we found in Fontan patients may reflect altered cell signalling and metabolism as found in heart failure in biventricular patients, chronic low-level inflammation, and alteration of functional or structural properties of lymphatic or blood vessels. Trial registration number: ClinicalTrials.gov Identifier NCT03886935

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Section: Discussionmentioning

confidence: 99%

Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

Michel

Dubowy

Zlamy

et al. 2020

Therapeutic Advances in Chronic Disease

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“…22 Vitamin D, an important component of health for both bone and the vascular system, is remarkably low in our Fontan population. 23 Parathyroid hormone levels are elevated, 24 which may reflect a compensatory attempt to maintain serum calcium at the expense of bone calcium stores, thereby leading to bone density abnormalities on dual energy x-ray absorptiometry (DXA) scanning. Significant renal insufficiency is uncommon early.…”

Section: Comprehensive Monitoring and Evaluation After Fontan Operatimentioning

confidence: 99%

The Relentless Effects of the Fontan Paradox

Rychik¹

2016

Seminars in Thoracic and Cardiovascular Surgery: Pediatric Card

143

139

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The Fontan operation is the anticipated palliative strategy for children born with single-ventricle type of congenital heart disease. As a result of important circulatory limitations, a series of end-organ complications are now increasingly recognized. Elevated central venous pressure and impaired cardiac output are the hallmarks of cavo-pulmonary flow, which result in a cascade of pathophysiological consequences. The Fontan circulation likely impacts all organ systems in an indolent and relentless manner, with progressive decline in functionality likely to occur in many. Liver fibrosis, altered bone density, decreased muscle mass, renal dysfunction, lymphatic insufficiency, and a host of other conditions are present. Standardized screening and evaluation of survivors as they grow through childhood and beyond is indicated and can be facilitated through dedicated multidisciplinary clinical programs. Invasive assessment at specific milestones can provide important actionable information to optimize individual status. More detailed characterization and understanding of these end-organ complications is necessary to contribute to the goal of achieving a normal duration and quality of life for these unique individuals.

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“…13 Low levels of serum 25-hydroxyvitamin D appear to be equally common in the Fontan population as it is amongst healthy children. 14 Vitamin D is essential for calcium and skeletal homeostasis, and it is important in other tissues, including skeletal muscle. 15,16 Vitamin D is stored in adipose tissue and requires 25hydroxylation in the liver to become biologically active.…”

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confidence: 99%

Vitamin D, liver-related biomarkers, and distribution of fat and lean mass in young patients with Fontan circulation

et al. 2021

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Introduction/aim: Young patients with Fontan circulation may have low serum 25-hydroxyvitamin D levels, an affected liver, and unhealthy body compositions. This study aimed to explore the association between vitamin D intake/levels, liver biomarkers, and body composition in young Fontan patients. Method: We collected prospective data in 2017 to 2018, obtained with food-frequency questionnaires, biochemical analyses of liver biomarkers, and dual-energy X-ray absorptiometry scans in 44 children with Fontan circulation. Body compositions were compared to matched controls (n = 38). Linear regression analyses were used to investigate associations of biomarkers, leg pain, and lean mass on serum levels of 25-hydroxyvitamin D. Biomarkers were converted to z scores and differences were evaluated within the Fontan patients. Results: Our Fontan patients had a daily mean vitamin D intake of 9.9 µg and a mean serum 25-hydroxyvitamin D of 56 nmol/L. These factors were not associated with fat or lean mass, leg pain, or biomarkers of liver status. The Fontan patients had significantly less lean mass, but higher fat mass than controls. Male adolescents with Fontan circulation had a greater mean abdominal fat mass than male controls and higher cholesterol levels than females with Fontan circulation. Conclusion: Vitamin D intake and serum levels were not associated with body composition or liver biomarkers in the Fontan group, but the Fontan group had lower lean mass and higher fat mass than controls. The more pronounced abdominal fat mass in male adolescents with Fontan circulation might increase metabolic risks later in life.

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Low serum 25-hydroxyvitamin D levels and secondary hyperparathyroidism in Fontan patients

Cited by 19 publications

References 41 publications

Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

The Relentless Effects of the Fontan Paradox

Vitamin D, liver-related biomarkers, and distribution of fat and lean mass in young patients with Fontan circulation

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