Open Access

ARTICLE

Prevalence and Risk Factors Associated with Renal Dysfunction in Patients with Single Ventricle Congenital Heart Disease after Fontan Palliation

Sheetal R. Patel^1,2,*, David M. Kwiatkowski³, Adin-Cristian Andrei², Ankita Devareddy², Hangzhi Shi², Catherine D. Krawczeski⁴, Natalie Ebert⁵, Barbara J. Deal^1,2, Craig B. Langman^2,6, Bradley S. Marino^1,2

1 Division of Pediatric Cardiology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 60611, USA
2 Northwestern University Feinberg School of Medicine, Chicago, IL, 60611, USA
3 Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, 94304, USA
4 Division of Pediatric Cardiology, Nationwide Children’s Hospital, Columbus, OH, 43205, USA
5 Institute of Public Health, Charité–Universitaetsmedizin Berlin, Berlin, 10177, Germany
6 Division of Kidney Diseases, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 60611, USA

* Corresponding Author: Sheetal R. Patel. Email:

Congenital Heart Disease 2020, 15(4), 181-195. https://doi.org/10.32604/CHD.2020.012097

Received 17 June 2020; Accepted 30 July 2020; Issue published 07 September 2020

Abstract

Objectives: The Fontan operation has increased survival in patients with single-ventricle congenital heart defects. However, Fontan survivors are at risk of other organ dysfunctions, such as renal dysfunction (RD). The objectives of this study are to assess the prevalence of and potential risk factors for RD among Fontan survivors. Design, setting, and patients: We performed a two-center, cross-sectional study that included Fontan survivors evaluated in outpatient-clinics for routine follow up between 01/08-12/16. Outcome measures: The primary outcome was the presence of RD defined by estimated glomerular filtration rate (eGFR) of <90 ml/min/1.73 m² derived using the serum creatinine-based Full Age Spectrum equation. Chi-square and t-tests were used to compare groups with and without RD. A multivariable logistic regression model was derived to identify risk factors associated with the presence of RD using stepwise variable selection methods. Additionally, using eGFR as a continuous variable, a linear regression model was derived to evaluate risk factors that negatively correlate with eGFR. Results: We included 402 Fontan survivors; 61% male; median age 13.7 (2.3–49.9) years; median time since initial Fontan 9.8 (0.1–36.9) years. RD was present in 27.4% (110/402) of patients. Risk factors for RD included single ventricle with right ventricular morphology [odds ratio 2.04; 95% CI (1.26,3.3)], ascites [2.99 (1.04,8.59)] and sildenafil therapy [2.22 (1.05,4.67)]. Risk factors that negatively correlate with eGFR included history of Stage 1 Norwood palliation (−7.6 ml/min/ 1.73 m²; p = 0.003); “failing Fontan physiology” defined by ascites, protein-losing enteropathy and/or plastic bronchitis (−8.9 ml/min/1.73 m²; p = 0.01) and moderate or greater ventricular dysfunction (−16.7 ml/min/1.73 m²; p = 0.02). Conclusions: One-fourth of Fontan survivors demonstrate RD within ten years after Fontan. Risk factors for RD included right ventricular morphology of the single ventricle, history of Stage 1 Norwood palliation, “failing Fontan physiology,” or ventricular dysfunction. Therefore, comprehensive screening for RD in Fontan survivors is needed, particularly in those identified at a higher risk for RD.

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