Prevalence and risk factors associated with renal dysfunction in patients with single ventricle congenital heart disease after Fontan palliation

Sheetal R. Patel*, David M. Kwiatkowski, Adin Cristian Andrei, Ankita Devareddy, Hangzhi Shi, Catherine D. Krawczeski, Natalie Ebert, Barbara J. Deal, Craig B. Langman, Bradley S. Marino

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)181-195
Number of pages15
JournalCongenital Heart Disease
Volume15
Issue number4
DOIs
StatePublished - 2020

Keywords

  • Congenital heart disease
  • Fontan
  • Renal dysfunction
  • Risk factors
  • Single ventricle

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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