Heart failure & cardiomyopathiesCohort study
Prognostic Implications of the Extent of Cardiac Damage in Patients With Fabry Disease
- Multicentre cohort study
- 5 Italian referral centres
Authors' conclusion
In FD patients, a novel staging classification of cardiac damage, evaluated by echocardiography, is strongly associated with cardiovascular outcomes and may be helpful to refine risk stratification.
Study context
- Population
- Patients with Fabry disease at 5 Italian referral centres
- Participants
- 314 patients
- Exposure
- Echocardiographic cardiac damage stages 1–3 vs stage 0
- Stage 0n = 174reference
- Stage 1n = 41
- Stage 2n = 57
- Stage 3n = 42
- Outcome
- Composite of:
- All-cause death
- HF hospitalization
- New-onset AF
- Major brady- or tachyarrhythmias and ischemic stroke
Main finding
Composite CV events by cardiac stage · 8 years
Hazard ratio
2.086
95% CI 1.487–2.927
P < 0.001
Per 1-stage increase
HR 2.086 (95% CI 1.487–2.927); P < 0.001
Per 1-stage increase, as printed
As reported in the abstract
- Progressive increase in composite event rate at 8 years with worsening stage (log-rank)
- P < 0.001
Observational data: associations, not effects.
Original publication
Prognostic Implications of the Extent of Cardiac Damage in Patients With Fabry Disease. J Am Coll Cardiol. Published 10 October 2023. doi:10.1016/j.jacc.2023.07.026
Summarised from the published abstract; every number is checked against it