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

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

JACC: Advances (Dec 2024)

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

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

Affiliations

Gaston van Hassel, MD: Center for Congenital Heart Diseases, Paediatric Cardiology, Beatrix Children’s Hospital, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands; Address for correspondence: Dr Gaston van Hassel, Center for Congenital Heart Diseases, Beatrix Children’s Hospital, University of Groningen, P.O. Box 30.001, internal zip code AB43, 9700 RB Groningen, the Netherlands.
Dion Groothof, BSc: Division of Nephrology, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
Johannes M. Douwes, MD, PhD: Center for Congenital Heart Diseases, Paediatric Cardiology, Beatrix Children’s Hospital, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands
Elke S. Hoendermis, MD, PhD: Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
Eryn T. Liem, MD, PhD: Center for Congenital Heart Diseases, Paediatric Cardiology, Beatrix Children’s Hospital, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands
Tineke P. Willems, MD, PhD: Department of Radiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
Tjark Ebels, MD, PhD: Department of Cardiothoracic Surgery, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
Adriaan A. Voors, MD, PhD: Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
Stephan J.L. Bakker, MD, PhD: Division of Nephrology, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
Rolf M.F. Berger, MD, PhD: Center for Congenital Heart Diseases, Paediatric Cardiology, Beatrix Children’s Hospital, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands
Joost P. van Melle, MD, PhD: Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands

Journal volume & issue: Vol. 3, no. 12
p. 101399

Abstract

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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.

Published in JACC: Advances

ISSN: 2772-963X (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the circulatory (Cardiovascular) system; Medicine: Internal medicine: Medical emergencies. Critical care. Intensive care. First aid
Website: https://www.sciencedirect.com/journal/jacc-advances

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