Congenital heart disease

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

JACC AdvPublished Longitudinal cohort study

Authors' conclusion

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.

Study context

Population
Patients with a Fontan circulation
Participants
82 patients
Outcome
All-cause mortality (survival analyses)

Main finding

As reported in the abstract

11-year period

Mean eGFRcr decline per year, faster than healthy Dutch cohort

1.36 ml/min/1.73 m²/year

Observational data: associations, not effects.

Further findings

Renal dysfunction at baseline (eGFR <90 ml/min/1.73 m²)

Renal dysfunction by eGFRcr
12%
Renal dysfunction by eGFRcys
24%

Factors associated with eGFRcr decline

Higher baseline N-terminal prohormone of brain natriuretic peptide (NT-proBNP) z-scores associated with more rapid eGFRcr decline

Age at baseline

Median age (IQR 14-27 years)
20 years

Original publication

Deterioration in Renal Function in Patients With a Fontan Circulation and Association With Mortality. JACC Adv. Published 19 November 2024. doi:10.1016/j.jacadv.2024.101399

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