Deterioration in Renal Function in Patients With a Fontan Circulation and Association With Mortality

Background: Renal dysfunction is a well-established risk factor in cardiovascular disease, but little is known about the prevalence and factors associated with deterioration in renal function in patients with a Fontan circulation. Objectives: The purpose of the study was to investigate the course an...

Full description

Saved in:
Bibliographic Details
Main Authors: Gaston van Hassel, MD, Dion Groothof, BSc, Johannes M. Douwes, MD, PhD, Elke S. Hoendermis, MD, PhD, Eryn T. Liem, MD, PhD, Tineke P. Willems, MD, PhD, Tjark Ebels, MD, PhD, Adriaan A. Voors, MD, PhD, Stephan J.L. Bakker, MD, PhD, Rolf M.F. Berger, MD, PhD, Joost P. van Melle, MD, PhD
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:JACC: Advances
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2772963X24006793
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Background: Renal dysfunction is a well-established risk factor in cardiovascular disease, but little is known about the prevalence and factors associated with deterioration in renal function in patients with a Fontan circulation. Objectives: The purpose of the study was to investigate the course and factors associated with deterioration in renal function in patients with a Fontan circulation and its association with mortality. Methods: This is a longitudinal study of patients with a Fontan circulation (n = 82), in which creatinine-based estimated glomerular filtration rate (eGFRcr) was measured over an 11-year time period. Cystatin C and N-terminal prohormone of brain natriuretic peptide (NT-proBNP) levels were measured at baseline. Renal dysfunction was defined as an eGFR <90 ml/min/1.73 m2. Factors associated with annual change in eGFRcr were investigated with linear mixed-effect models and compared with data from a healthy Dutch cohort. The primary endpoint for the survival analyses was all-cause mortality. Associations between repeated eGFRcr levels and the primary endpoint were assessed using a joint model. Results: The median age at baseline was 20 years (IQR: 14-27 years). Twelve percent of the cohort had renal dysfunction based on eGFRcr and 24% based on cystatin C-based eGFRcys. During follow-up, eGFRcr deteriorated on average by 1.36 ml/min/1.73 m2/year, which is faster than the healthy cohort. Higher baseline NT-proBNP z-scores were associated with a more rapid decline in eGFRcr. A larger decline in eGFRcr was associated with all-cause mortality. Conclusions: Declines in eGFRcr in patients with Fontan circulation are more rapid than in healthy individuals. Higher baseline NT-proBNP z-scores are associated with a more rapid deterioration of eGFRcr, and eGFRcr deterioration is associated with mortality.
ISSN:2772-963X