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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: [email protected]
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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.

References

Fontan, F, Baudet, E.Surgical repair of tricuspid atresia. Thorax. 1971; 26: 240–248.CrossRefGoogle ScholarPubMed
Jonas, RA.The intra/extracardiac conduit fenestrated fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011; 14: 11–18.CrossRefGoogle ScholarPubMed
Harahsheh, A, Sade, R.Active and passive euthanasia in the context of severe congenital heart disease. In: Mavroudis, C., Cook, J., Mavroudis, C. (eds). Bioethical Controversies in Pediatric Cardiology and Cardiac Surgery. Springer, Cham, 2020: 251–259. doi: 10.1007/978-3-030-35660-6_19CrossRefGoogle Scholar
Sinha, P, Zurakowski, D, He, D, et al.Intra/extracardiac fenestrated modification leads to lower incidence of arrhythmias after the Fontan operation. J Thorac Cardiovasc Surg 2013; 145: 678–682.CrossRefGoogle ScholarPubMed
Ooi, YK, Sinha, P, Gierdalski, M, Harahsheh, A.High-risk single ventricle palliation in children with Down syndrome: single institution experience. Cardiol Young 2015; 25: 539–543.CrossRefGoogle ScholarPubMed
Silvilairat, S, Cabalka, AK, Cetta, F, Grogan, M, Hagler, DJ, O’Leary, PW.Protein-losing enteropathy after the Fontan operation: associations and predictors of clinical outcome. Congenit Heart Dis 2008; 3: 262–268.CrossRefGoogle ScholarPubMed
Feldt, RH, Driscoll, DJ, Offord, KP, et al.Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 1996; 112: 672–680.CrossRefGoogle ScholarPubMed
Mertens, L, Hagler, DJ, Sauer, U, Somerville, J, Gewillig, M.Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998; 115: 1063–1073.CrossRefGoogle ScholarPubMed
Yu, JJ, Yun, TJ, Yun, SC, et al.Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy. Int J Cardiol 2013; 165: 454–457.CrossRefGoogle ScholarPubMed
Ruth, A, McCracken, CE, Fortenberry, JD, Hebbar, KB.Extracorporeal therapies in pediatric severe sepsis: findings from the pediatric health-care information system. Crit Care 2015; 19: 397.CrossRefGoogle ScholarPubMed
Harahsheh, AS, O’Byrne, ML, Pastor, B, Graham, DA, Fulton, DR.Pediatric chest pain-low-probability referral: a multi-institutional analysis from standardized clinical assessment and management plans (SCAMPs®), the pediatric health information systems database, and the national ambulatory medical care survey. Clin Pediatr (Phila) 2017; 56: 1201–1208.CrossRefGoogle Scholar
Allen, KY, Downing, TE, Glatz, AC, et al.Effect of Fontan-associated morbidities on survival with intact Fontan circulation. Am J Cardiol 2017; 119: 1866–1871.CrossRefGoogle ScholarPubMed
John, AS, Johnson, JA, Khan, M, Driscoll, DJ, Warnes, CA, Cetta, F.Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol 2014; 64: 54–62.CrossRefGoogle ScholarPubMed
Harahsheh, AS, Hom, LA, Clauss, SB, et al.The impact of a designated cardiology team involving telemedicine home monitoring on the care of children with single-ventricle physiology after norwood palliation. Pediatr Cardiol 2016; 37: 899–912.CrossRefGoogle ScholarPubMed
Rychik, J, Kim, Y.The adolescent and adult with a Fontan circulation: “Unnatural” selection and survival of the fittest. J Am Coll Cardiol 2018; 71: 1018–1020.CrossRefGoogle ScholarPubMed
Davis, JAM, Miller-Tate, H, Texter, KM.Launching a new strategy for multidisciplinary management of single-ventricle heart defects. Crit Care Nurse 2018; 38: 60–71.CrossRefGoogle ScholarPubMed
Oster, ME, Kelleman, M, McCracken, C, Ohye, RG, Mahle, WT.Association of digoxin with interstage mortality: Results from the pediatric heart network single ventricle reconstruction trial public use dataset. J Am Heart Assoc 2016; 5: e002566.CrossRefGoogle ScholarPubMed
Brown, DW, Mangeot, C, Anderson, JB, et al.Digoxin use is associated with reduced interstage mortality in patients with no history of arrhythmia after stage I palliation for single ventricle heart disease. J Am Heart Assoc 2016; 5: e002376.CrossRefGoogle Scholar
Truong, DT, Menon, SC, Lambert, LM, et al.Digoxin use in infants with single ventricle physiology: secondary analysis of the pediatric heart network infant single ventricle trial public use dataset. Pediatr Cardiol 2018; 39: 1200–1209.CrossRefGoogle ScholarPubMed
Goldberg, DJ, Dodds, K, Avitabile, CM, et al.Children with protein-losing enteropathy after the Fontan operation are at risk for abnormal bone mineral density. Pediatr Cardiol 2012; 33: 1264–1268.CrossRefGoogle ScholarPubMed
Chernow, B, Zaloga, G, McFadden, E, et al.Hypocalcemia in critically ill patients. Crit Care Med 1982; 10: 848–851.CrossRefGoogle ScholarPubMed
Averin, K, Villa, C, Krawczeski, CD, et al.Initial observations of the effects of calcium chloride infusions in pediatric patients with low cardiac output. Pediatr Cardiol 2016; 37: 610–617.CrossRefGoogle ScholarPubMed
Kimura, S, Iwasaki, T, Oe, K, et al.High ionized calcium concentration is associated with prolonged length of stay in the intensive care unit for post-operative pediatric cardiac patients. J Cardiothorac Vasc Anesth 2018; 32: 1667–1675.CrossRefGoogle ScholarPubMed
Dean, PN, Hillman, DG, McHugh, KE, Gutgesell, HP.Inpatient costs and charges for surgical treatment of hypoplastic left heart syndrome. Pediatrics 2011; 128: e1181–e1186.CrossRefGoogle ScholarPubMed
Hinkle, KA, Wilkes, J, McFadden, M, Williams, RV, Minich, LL, Menon, SC.A multi-institutional study of factors affecting resource utilisation following the Fontan operation. Cardiol Young 2017; 27: 739–746.CrossRefGoogle ScholarPubMed
Huang, L, Schilling, C, Dalziel, KM, et al.Hospital inpatient costs for single ventricle patients surviving the Fontan procedure. Am J Cardiol 2017; 120: 467–472.CrossRefGoogle ScholarPubMed
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