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Impact of protein-losing enteropathy in children who underwent the Fontan operation

Published online by Cambridge University Press:  20 July 2020

Baneen Alkofair ,
Abdulmajeed Alruwaili ,
Jiaxiang Gai  and
Ashraf S. Harahsheh Open the ORCID record for Ashraf S. Harahsheh [Opens in a new window]
Baneen Alkofair
Affiliation:
Department of Pediatrics, George Washington University School of Medicine, Washington, DC, USA
Abdulmajeed Alruwaili
Affiliation:
Department of Pediatrics, George Washington University School of Medicine, Washington, DC, USA
Jiaxiang Gai
Affiliation:
Division of Biostatistics and Study Methodology, Children’s National Hospital, Washington, DC, USA
Ashraf S. Harahsheh*
Affiliation:
Department of Pediatrics, George Washington University School of Medicine, Washington, DC, USA Division of Cardiology, Children’s National Hospital, Washington, DC, USA
*
Author for correspondence: Ashraf S. Harahsheh, MD, FACC, FAAP, Division of Cardiology, Children’s National Hospital, 111 Michigan Ave, NW, Washington, DC20010, USA. Tel: +1 2024762020; Fax: +1 2024765700. E-mail: aharahsh@childrensnational.org
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Abstract

Previous reports have identified risk factors associated with development of post-Fontan protein-losing enteropathy. Less is known about the economic impact and resource utilisation required for post-Fontan protein-losing enteropathy in the current era. We conducted a single-centre retrospective study to assess the impact of post-Fontan protein-losing enteropathy on transplant-free survival. We also described resource utilisation and treatment variations among post-Fontan protein-losing enteropathy patients. Children who received care at our centre between 2009 and 2017 after the Fontan surgery were eligible. Initial admissions for the Fontan operative procedure were excluded. Demographics, hospital admissions, resource utilisation, medications and charges were reviewed. Patients were divided into two groups based on the presence of post-Fontan protein-losing enteropathy. Of the 343 patients screened, 147 met the eligibility criteria. Of these, 28 (19%) developed protein-losing enteropathy. After adjusting for follow-up duration, the protein-losing enteropathy group had higher number of encounters (2.15 ± 2.16 versus 1.47 ± 2.56, p 0.002), hospital length of stay (days) (25 ± 51.3 versus 11.4 ± 41.7, p < 0.0001) and total charges (2018US$) (388,489 ± 759,859 versus 202,725 ± 1,076,625, p < 0.0001). Encounters for patients with protein-losing enteropathy utilised more therapies. Among those with protein-losing enteropathy, use of digoxin was associated with slightly decreased odds for mortality and/or transplant (0.95, confidence interval 0.90–0.99, p 0.021). The 10-year transplant-free survival for patients with/without protein-losing enteropathy was 65.7/97.3% (p 0.002), respectively. Post-Fontan protein-losing enteropathy is associated with reduced 10-year transplant-free survival, higher resource utilisation, charges and medication use compared with the non-protein-losing enteropathy group. Practice variation among post-Fontan protein-losing-enteropathy patients is common. Further larger studies are needed to assess the impact of standardisation on the well-being of children with post-Fontan protein-losing enteropathy.

Keywords

Post-Fontan protein-losing enteropathyresource utilisationfunctionally single ventricle anatomyhealth economicscongenital heart surgery
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 9 , September 2020 , pp. 1273 - 1280
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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Footnotes

a

Dr. Abdulmajeed Alruwaili is now a paediatrics resident at Nicklaus Children’s Hospital.

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