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

  • Finn Holler (a1), Tobias Hannes (a1), Ingo Germund (a1), Mathias Emmel (a1), Heike Hoyer-Kuhn (a2), Markus Khalil (a3), Narayanswami Sreeram (a1) and Floris E. A. Udink ten Cate (a1)...



Limited data exist on the vitamin D status in Fontan patients. We determined the prevalence and potential risk factors of vitamin D deficiency in this patient subset.

Methods and results

Data were collected from 27 Fontan patients (55.6% male, mean age 8.1±5.3 years). Protein-losing enteropathy was diagnosed in six patients (22.2%). Vitamin D deficiency was defined as a serum 25-hydroxyvitamin D level of <20 ng/ml. The neutrophil-to-lymphocyte ratio, a marker of systemic inflammation, was calculated. Associations between laboratory measurements and patient characteristics were explored. Mean serum 25-hydroxyvitamin D level was 14.1±10.4 ng/ml. Vitamin D deficiency was found in 19/27 patients (70.3%). Only skin type was associated with vitamin D deficiency (p=0.04). Hyperparathyroidism was present in 5/21 (23.8%) patients, and was more prevalent in patients with protein-losing enteropathy (p<0.001). Parathyroid hormone levels correlated with parameters of systemic inflammation (neutrophil-to-lymphocyte ratio: r=0.484, p=0.026; relative lymphocyte count: r=−0.635, p=0.002). Vitamin D supplementation significantly increased serum 25-hydroxyvitamin D levels (p<0.0001), and was accompanied by a reduction in parathyroid hormone concentrations (p=0.032).


A high prevalence of vitamin D deficiency was found among Fontan patients, independent of age, time after Fontan procedure, ventricular morphology, and presence of protein-losing enteropathy. A potentially important link between parathyroid hormone levels and systemic inflammation is suggested.


Corresponding author

Correspondence to: Dr F. E. A. Udink ten Cate, Department of Paediatric Cardiology, Heart Centre Cologne, University Hospital of Cologne, Kerpenerstrasse 62, 50973 Cologne, Germany. Tel: +492 214 783 2330; Fax: +492 214 783 2646; E-mail:


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Both authors contributed equally.



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