@Article{CHD.2020.012097,
AUTHOR = {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},
TITLE = {Prevalence and Risk Factors Associated with Renal Dysfunction in Patients with Single Ventricle Congenital Heart Disease after Fontan Palliation},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {15},
YEAR = {2020},
NUMBER = {4},
PAGES = {181--195},
URL = {http://www.techscience.com/schd/v15n4/40123},
ISSN = {3071-1738},
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.},
DOI = {10.32604/CHD.2020.012097}
}