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Focal segmental glomerulosclerosis in patients after Fontan operation: is this a Fontan-associated renal disease?

Published online by Cambridge University Press:  15 September 2021

Takashi Furuta Open the ORCID record for Takashi Furuta [Opens in a new window] ,
Jun Muneuchi Open the ORCID record for Jun Muneuchi [Opens in a new window] ,
Yuichiro Sugitani  and
Miwa Yoshino
Takashi Furuta
Affiliation:
Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan
Jun Muneuchi*
Affiliation:
Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan
Yuichiro Sugitani
Affiliation:
Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan
Miwa Yoshino
Affiliation:
Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan
*
Author for correspondence: Jun Muneuchi, MD, Department of Pediatrics, Kyushu Hospital Japan Community Healthcare Organization, 1-8-2, Kishionura, Yahatanishi-ku, Kitakyushu, Fukuoka 806-8507, Japan. Tel: +81 93 641 5111; Fax: +81 93 642 1868. E-mail: jmune@msn.com
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Abstract

Despite acceptable survival for Fontan operation, there are concerns about late complications affecting the major organs. We herein present two cases of adults after Fontan operation who developed focal segmental glomerulosclerosis. These cases suggest that focal segmental glomerulosclerosis is owing to haemodynamic incompetence associated with Fontan operation, including congestion, hypoxia, and hyperviscosity, which may be called Fontan-associated renal disease.

Keywords

CHDFontan operationfocal segmental glomerulosclerosismesangial proliferative glomerulonephritis
Type
Brief Report
Information
Cardiology in the Young , Volume 32 , Issue 5 , May 2022 , pp. 837 - 839
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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References

Ohuchi, H. Adult patients with Fontan circulation: what we know and how to manage adults with Fontan circulation? J Cardiol 2016; 68: 181–189.CrossRefGoogle ScholarPubMed
Han, M-H, Kim, Y-J. Practical application of columbia classification for focal segmental glomerulosclerosis. Biomed Res Int 2016; 2016: 9375753.10.1155/2016/9375753CrossRefGoogle ScholarPubMed
Campbell, KN, Tumlin, JA. Protecting podocytes: a key target for therapy of focal segmental glomerulosclerosis. Am J Nephrol 2018; 47: 14–29.10.1159/000481634CrossRefGoogle ScholarPubMed
Rangaswami, J, Bhalla, V, Blair, JEA, et al. Cardiorenal syndrome: classification, pathophysiology, diagnosis, and treatment strategies: a scientific statement from the American Heart Association. Circulation 2019; 139: e840–e878.10.1161/CIR.0000000000000664CrossRefGoogle ScholarPubMed
Khuong, JN, Wilson, TG, Grigg, LE, et al. Fontan-associated nephropathy: predictors and outcomes. Int J Cardiol 2020; 306: 73–77.10.1016/j.ijcard.2020.01.014CrossRefGoogle ScholarPubMed
Dimopoulos, K, Diller, GP, Koltsida, E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation 2008; 117: 2320–2328.10.1161/CIRCULATIONAHA.107.734921CrossRefGoogle ScholarPubMed
Ohuchi, H, Negishi, J, Hayama, Y, Miyazaki, A, Shiraishi, I, Ichikawa, H. Renal resistive index reflects Fontan pathophysiology and predicts mortality. Heart 2017; 103: 1631–1637. DOI 10.1007/s11255-013-0528-6..10.1136/heartjnl-2016-310812CrossRefGoogle ScholarPubMed
Gupte, PA, Vaideeswar, P, Kandalkar, BM. Cyanotic nephropathy-a morphometric analysis. Congenit Heart Dis 2014; 9: 280–285.10.1111/chd.12121CrossRefGoogle ScholarPubMed
Broda, CR, Sriraman, H, Wadhwa, D, et al. Renal dysfunction is associated with higher central venous pressures in patients with Fontan circulation. Congenit Heart Dis 2018; 13: 602–607.10.1111/chd.12617CrossRefGoogle ScholarPubMed
Sharma, SG, Bomback, AS, Radhakrishnan, J, et al. The modern spectrum of renal biopsy findings in patients with diabetes. Clin J Am Soc Nephrol 2013; 8: 1718–1724.10.2215/CJN.02510213CrossRefGoogle ScholarPubMed