Assessment of Kidney Function in Survivors Following Fontan Palliation

Sheena Sharma, Rebecca Ruebner, Susan L. Furth, Kathryn M. Dodds, Jack Rychik, David J. Goldberg

Research output: Contribution to journalArticle

Abstract

Objective: The Fontan operation is a palliative procedure for congenital single ventricle heart disease. Long-term kidney function in this cohort is not well-known. Our aim was to evaluate renal function in long-term survivors post-Fontan palliation, and we hypothesize that this cohort will have a higher prevalence of chronic kidney disease (CKD) compared to controls. Design: We performed a retrospective cohort study of 68 subjects evaluated through the Single Ventricle Survivorship Program at the Children's Hospital of Philadelphia between July 2010 and December 2014 compared to 70 healthy children similar in age and sex. Primary outcome was CKD, defined as estimated glomerular filtration rate (eGFR) <90 mL/min/1.73 m2 using creatinine and cystatin C–based estimating equations. Secondary outcomes included proteinuria and elevated intact parathyroid hormone. Results: The Fontan cohort included 68 subjects with median age 13 years (IQR 9.0, 17.3) who were median 11.1 years (IQR 6.5, 15.7) post-Fontan palliation. This cohort was compared to 70 healthy individuals (median age 15.5 years (IQR 12.5, 18.3). Although median eGFRs were comparable: 102.6 vs. 101.9 mL/min/1.73 m2 (P =.89) in Fontan vs. healthy subjects <18 years of age (Full CKiD equation), and 128.5 vs. 129.7 mL/min/1.73 m2 (P =.56) in Fontan vs. healthy subjects ≥18 years of age (CKD-EPI creatinine and cystatin formula); 10% of Fontan subjects had an eGFR<90 mL/min/1.73 m2. Median intact parathyroid hormone level was higher at 59.4 pg/mL (IQR 43.0, 83.1) in the Fontan group compared to 23.4 pg/mL (IQR 16.7, 30.0) in controls (P ≤.001). Proteinuria was present in 10% of the Fontan group compared to 4.7% in controls (P =.27). Conclusion: Ten percent of long-term survivors post-Fontan palliation had eGFR <90 ml/min/1.73 m2, and higher median parathyroid hormone levels compared to controls. Taken together, these measures may indicate early kidney disease. Future studies will focus on longitudinal assessment of kidney function and evaluation of risk factors for CKD post-Fontan palliation.

Original languageEnglish (US)
Pages (from-to)630-636
Number of pages7
JournalCongenital Heart Disease
Volume11
Issue number6
DOIs
StatePublished - Nov 1 2016
Externally publishedYes

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Chronic Renal Insufficiency
Survivors
Parathyroid Hormone
Glomerular Filtration Rate
Cystatins
Kidney
Proteinuria
Creatinine
Healthy Volunteers
Fontan Procedure
Kidney Diseases
Heart Diseases
Cohort Studies
Survival Rate
Retrospective Studies

Keywords

  • Chronic Kidney Disease
  • Fontan Palliation
  • Single Ventricle Heart Disease

ASJC Scopus subject areas

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

Cite this

Sharma, S., Ruebner, R., Furth, S. L., Dodds, K. M., Rychik, J., & Goldberg, D. J. (2016). Assessment of Kidney Function in Survivors Following Fontan Palliation. Congenital Heart Disease, 11(6), 630-636. https://doi.org/10.1111/chd.12358

Assessment of Kidney Function in Survivors Following Fontan Palliation. / Sharma, Sheena; Ruebner, Rebecca; Furth, Susan L.; Dodds, Kathryn M.; Rychik, Jack; Goldberg, David J.

In: Congenital Heart Disease, Vol. 11, No. 6, 01.11.2016, p. 630-636.

Research output: Contribution to journalArticle

Sharma, S, Ruebner, R, Furth, SL, Dodds, KM, Rychik, J & Goldberg, DJ 2016, 'Assessment of Kidney Function in Survivors Following Fontan Palliation', Congenital Heart Disease, vol. 11, no. 6, pp. 630-636. https://doi.org/10.1111/chd.12358
Sharma, Sheena ; Ruebner, Rebecca ; Furth, Susan L. ; Dodds, Kathryn M. ; Rychik, Jack ; Goldberg, David J. / Assessment of Kidney Function in Survivors Following Fontan Palliation. In: Congenital Heart Disease. 2016 ; Vol. 11, No. 6. pp. 630-636.
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abstract = "Objective: The Fontan operation is a palliative procedure for congenital single ventricle heart disease. Long-term kidney function in this cohort is not well-known. Our aim was to evaluate renal function in long-term survivors post-Fontan palliation, and we hypothesize that this cohort will have a higher prevalence of chronic kidney disease (CKD) compared to controls. Design: We performed a retrospective cohort study of 68 subjects evaluated through the Single Ventricle Survivorship Program at the Children's Hospital of Philadelphia between July 2010 and December 2014 compared to 70 healthy children similar in age and sex. Primary outcome was CKD, defined as estimated glomerular filtration rate (eGFR) <90 mL/min/1.73 m2 using creatinine and cystatin C–based estimating equations. Secondary outcomes included proteinuria and elevated intact parathyroid hormone. Results: The Fontan cohort included 68 subjects with median age 13 years (IQR 9.0, 17.3) who were median 11.1 years (IQR 6.5, 15.7) post-Fontan palliation. This cohort was compared to 70 healthy individuals (median age 15.5 years (IQR 12.5, 18.3). Although median eGFRs were comparable: 102.6 vs. 101.9 mL/min/1.73 m2 (P =.89) in Fontan vs. healthy subjects <18 years of age (Full CKiD equation), and 128.5 vs. 129.7 mL/min/1.73 m2 (P =.56) in Fontan vs. healthy subjects ≥18 years of age (CKD-EPI creatinine and cystatin formula); 10{\%} of Fontan subjects had an eGFR<90 mL/min/1.73 m2. Median intact parathyroid hormone level was higher at 59.4 pg/mL (IQR 43.0, 83.1) in the Fontan group compared to 23.4 pg/mL (IQR 16.7, 30.0) in controls (P ≤.001). Proteinuria was present in 10{\%} of the Fontan group compared to 4.7{\%} in controls (P =.27). Conclusion: Ten percent of long-term survivors post-Fontan palliation had eGFR <90 ml/min/1.73 m2, and higher median parathyroid hormone levels compared to controls. Taken together, these measures may indicate early kidney disease. Future studies will focus on longitudinal assessment of kidney function and evaluation of risk factors for CKD post-Fontan palliation.",
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N2 - Objective: The Fontan operation is a palliative procedure for congenital single ventricle heart disease. Long-term kidney function in this cohort is not well-known. Our aim was to evaluate renal function in long-term survivors post-Fontan palliation, and we hypothesize that this cohort will have a higher prevalence of chronic kidney disease (CKD) compared to controls. Design: We performed a retrospective cohort study of 68 subjects evaluated through the Single Ventricle Survivorship Program at the Children's Hospital of Philadelphia between July 2010 and December 2014 compared to 70 healthy children similar in age and sex. Primary outcome was CKD, defined as estimated glomerular filtration rate (eGFR) <90 mL/min/1.73 m2 using creatinine and cystatin C–based estimating equations. Secondary outcomes included proteinuria and elevated intact parathyroid hormone. Results: The Fontan cohort included 68 subjects with median age 13 years (IQR 9.0, 17.3) who were median 11.1 years (IQR 6.5, 15.7) post-Fontan palliation. This cohort was compared to 70 healthy individuals (median age 15.5 years (IQR 12.5, 18.3). Although median eGFRs were comparable: 102.6 vs. 101.9 mL/min/1.73 m2 (P =.89) in Fontan vs. healthy subjects <18 years of age (Full CKiD equation), and 128.5 vs. 129.7 mL/min/1.73 m2 (P =.56) in Fontan vs. healthy subjects ≥18 years of age (CKD-EPI creatinine and cystatin formula); 10% of Fontan subjects had an eGFR<90 mL/min/1.73 m2. Median intact parathyroid hormone level was higher at 59.4 pg/mL (IQR 43.0, 83.1) in the Fontan group compared to 23.4 pg/mL (IQR 16.7, 30.0) in controls (P ≤.001). Proteinuria was present in 10% of the Fontan group compared to 4.7% in controls (P =.27). Conclusion: Ten percent of long-term survivors post-Fontan palliation had eGFR <90 ml/min/1.73 m2, and higher median parathyroid hormone levels compared to controls. Taken together, these measures may indicate early kidney disease. Future studies will focus on longitudinal assessment of kidney function and evaluation of risk factors for CKD post-Fontan palliation.

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