Congenital heart disease

Adults with Fontan circulation: prognostic value of exercise pulmonary vascular resistance index

Eur Heart JPublished Retrospective cohort study

Authors' conclusion

In adults post-Fontan, PVRI response to exercise is a robust predictor of clinical outcomes and offers additive prognostic information beyond resting invasive haemodynamic data and clinical risk factors

Study context

Population
Adults (≥18 years) post-Fontan palliation undergoing exercise (supine cycling) cardiac catheterization
Participants
88 adults
Exposure
Absence vs presence of exercise-induced reduction in PVR index (PVRI)
  • No PVRI reductionn = 23
  • PVRI reductionn = 65reference
Outcome
Composite of:
  • Death
  • Transplantation
Follow-up
2.2 ± 1.4 (IQR 1.2-2.6) years

Main finding

As reported in the abstract

Death or transplantation
Hazard ratio, per WU·m2 (95% CI)P value
Exercise PVRI
2.15(1.24–3.73)
P = 0.007
Resting PVRI
0.95(0.52–1.74)
P = 0.86
Exercise PVRIadjusted for exercise pulmonary artery wedge pressure
2.08(1.19–3.62)
P = 0.01

Per WU·m², as printed

Observational data: associations, not effects.

Further findings

Two-year event-free survival · 2 years

P < 0.001

PVRI at rest and exercise

Resting PVRI 1.9 ± 0.9 WU·m²
Exercise PVRI 1.2 (IQR 0.8-2.1) WU·m²
Clinical, biochemical and echocardiographic parameters not associated with PVRI response to exercise

Original publication

Adults with Fontan circulation: prognostic value of exercise pulmonary vascular resistance index. Eur Heart J. Published 23 December 2025. doi:10.1093/eurheartj/ehaf941

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