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Review
. 2022 Sep 29;14(19):4055.
doi: 10.3390/nu14194055.

Nutritional Management of Patients with Fontan Circulation: A Potential for Improved Outcomes from Birth to Adulthood

Letizia Baldini  1   2 , Katia Librandi  1 , Chiara D'Eusebio  3 , Antonella Lezo  3
Affiliations
Review

Nutritional Management of Patients with Fontan Circulation: A Potential for Improved Outcomes from Birth to Adulthood

Letizia Baldini et al. Nutrients. .

Abstract

Fontan circulation (FC) is a surgically achieved palliation state offered to patients affected by a wide variety of congenital heart defects (CHDs) that are grouped under the name of univentricular heart. The procedure includes three different surgical stages. Malnutrition is a matter of concern in any phase of life for these children, often leading to longer hospital stays, higher mortality rates, and a higher risk of adverse neurodevelopmental and growth outcomes. Notwithstanding the relevance of proper nutrition for this subset of patients, specific guidelines on the matter are lacking. In this review, we aim to analyze the role of an adequate form of nutritional support in patients with FC throughout the different stages of their lives, in order to provide a practical approach to appropriate nutritional management. Firstly, the burden of faltering growth in patients with univentricular heart is analyzed, focusing on the pathogenesis of malnutrition, its detection and evaluation. Secondly, we summarize the nutritional issues of each life phase of a Fontan patient from birth to adulthood. Finally, we highlight the challenges of nutritional management in patients with failing Fontan.

Keywords: Fontan circulation; congenital heart defects; faltering growth; nutrition; univentricular heart.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
PRISMA-chart summarizing search and selection process.
Figure 2
Figure 2
Scheme of more common surgical stages ending with Fontan procedure. Abbreviations: BDG, bidirectional Glenn; BT, Blalock–Taussig; CHD, congenital heart defects; HLHS, hypoplastic left heart syndrome; SV, single ventricle.
See this image and copyright information in PMC

References

    1. Driscoll D.J., Offord K.P., Feldt R.H., Schaff H.V., Puga F.J., Danielson G.K. Five- to Fifteen-Year Follow-up after Fontan Operation. Circulation. 1992;85:469–496. doi: 10.1161/01.CIR.85.2.469. - DOI - PubMed
    1. Ungerleider R.M., Meliones J.N., McMillan K.N., Cooper D.S., Jacobs J.P. Critical Heart Disease in Infants and Children. Elsevier; Amsterdam, The Netherlands: 2018. Critical Heart Disease in Infants and Children; pp. 747–757.e2.
    1. Sadineni R., Kumar B.S., Chander N., Boppana D. Prenatal Sonographic Diagnosis of Hypoplastic Left Heart Syndrome. Int. J. Appl. Basic Med. Res. 2017;7:213. doi: 10.4103/ijabmr.IJABMR_389_16. - DOI - PMC - PubMed
    1. Atz A.M., Travison T.G., Williams I.A., Pearson G.D., Laussen P.C., Mahle W.T., Cook A.L., Kirsh J.A., Sklansky M., Khaikin S., et al. Prenatal Diagnosis and Risk Factors for Preoperative Death in Neonates with Single Right Ventricle and Systemic Outflow Obstruction: Screening Data from the Pediatric Heart Network Single Ventricle Reconstruction Trial. J. Thorac. Cardiovasc. Surg. 2010;140:1245–1250. doi: 10.1016/j.jtcvs.2010.05.022. - DOI - PMC - PubMed
    1. Van Velzen C.L., Clur S.A., Rijlaarsdam M.E.B., Bax C.J., Pajkrt E., Heymans M.W., Bekker M.N., Hruda J., De Groot C.J.M., Blom N.A., et al. Prenatal Detection of Congenital Heart Disease--Results of a National Screening Programme. BJOG. 2016;123:400–407. doi: 10.1111/1471-0528.13274. - DOI - PubMed