Nephropathy in patients after Fontan palliation
Introduction
Fontan palliation is a series of surgical procedures that separate deoxygenated from oxygenated blood in patients with a single ventricle physiology [1]. One effect of this palliation is chronically elevated central venous pressures (CVP). Although the increased CVP in Fontan patients has been associated with protein-losing enteropathy, hepatic dysfunction, and arrhythmias [2], [3], [4], [5], its effect on the kidneys has not been defined. The purpose of this study was to determine whether post-Fontan patients show evidence of renal injury and if this process is associated with elevated CVP.
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Methods and materials
The study was approved by the Wayne State University Institutional Review Board. Subjects were recruited from patients followed in the Cardiology Clinic at Children's Hospital of Michigan that were at least 2 years after the Fontan procedure. Each patient underwent a clinical and echocardiographic evaluation. Only those with a normal ventricular function by echocardiography, a history of normal renal ultrasound and no comorbifdities known to affect kidneys (such as diabetes, or sickle cell
Results
Twenty-one subjects were evaluated, including 13 males and 8 females. The age was 15.2 +/− 8.8 years. Primary diagnoses included hypoplastic left ventricle in 9, double inlet left ventricle in 3, tricuspid valve atresia in 5, pulmonary atresia with intact ventricular septum in 2, and unbalanced atrio-ventricular canal in 2 patients. Two subjects were after a classic Fontan procedure, 17 after an intracardiac tunnel, and two after an extracardiac Fontan modification. Current medications included
Discussion
The significant finding of the study is the presence of microalbuminuria in a large proportion of post-Fontan patients, even when ventricular function is normal. Microalbuminuria is an earliest indicator of glomerulopathy and is routinely used for monitoring the onset and progression of renal disease [11]. In diabetic patients the presence of microalbuminuria corresponds to two to fourfold increase in cardiovascular mortality and morbidity [12]. As survival of patients after Fontan palliation
Conclusions
The study demonstrates that a significant proportion of patients with Fontan circulation have pathologic microalbuminuria, an indicator of renal injury. The injury is likely related to the elevated CVP in these patients. The use of ACE-I may be beneficial in preserving their renal function.
Acknowledgments
The authors are grateful to Dr. Thomas L'Ecuyer, Mrs. BaBette Chirunga, Mr. Paul Webster, and Ms. Masha Zilberman for their help in collecting the data and preparing the manuscript.
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Cited by (46)
EASL-ERN position paper on liver involvement in patients with Fontan-type circulation
2023, Journal of HepatologyHepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation
2022, Canadian Journal of CardiologyCitation Excerpt :Among Fontan patients, a higher CVP has been documented in those with kidney dysfunction.37 Furthermore, it has been demonstrated that elevated CVP, due to any etiology, causes an increase in glomerular filtration pressure, leading to microalbuminuria, a known early indicator of glomerulopathy.38 Similarly, pulmonary vascular resistance and SVC pressures in a Fontan patient correlate with urine albumin-to-creatinine ratio (uACR).39
3-Month Enalapril Treatment in Pediatric Fontan Patients With Moderate to Good Systolic Ventricular Function
2022, American Journal of CardiologyLong-Term Kidney Function After the Fontan Operation: JACC Review Topic of the Week
2020, Journal of the American College of CardiologyCitation Excerpt :Elevation in CVP, caused by a lack of a subpulmonic ventricular pump, reduced venous capacitance, and occasionally, anatomic obstruction in the Fontan pathway, is associated with reduced GFR, independent of the cardiac index (7,21,22). By raising the glomerular filtration pressure, elevated CVP can lead to albuminuria (23,24). In line with this, both post-Fontan pulmonary vascular resistance and superior vena cava pressures correlate with urine albumin-to-creatinine ratio (uACR) (23).
Renal function and Fontan patients: What is the real impact in the long-term outcomes?
2020, International Journal of CardiologyFontan-associated nephropathy: Predictors and outcomes
2020, International Journal of CardiologyCitation Excerpt :Arrhythmia and liver fibrosis have been broadly investigated [1–3], but Fontan-associated nephropathy has barely been explored in the literature [4]. Approximately 10–30% of patients living with a Fontan circulation have evidence of mild to moderate renal dysfunction [1,4–6]. In adults living with congenital heart disease, those who have concomitant kidney disease have been shown to have a mortality rate 3-times higher than those with normal kidney function [7].