Heart failure & cardiomyopathies
Prognostic role of myocardial storage, hypertrophy, and inflammation/fibrosis in Fabry cardiomyopathy: a new predictive score including T1 values
Authors' conclusion
We propose a 3-year prognostic score to predict CV outcome in FD, including age, LVMI, number of LGE+ segments, and septal T1. Normal T1 has a negative predictive value for adverse CV events irrespective of the acquisition setting.
Study context
- Population
- Fabry Disease (FD) patients who underwent cardiac magnetic resonance (CMR) at baselineFD patients enrolled
- Participants
- 167 participantsEvents: 27 patients (16%)
- Predictors
- A multivariable model including age, left ventricle mass index (LVMI), number of late gadolinium enhancement (LGE)+ segments, and T1 value
- Predicted outcome
- composite of:
- All-cause death
- Heart failure hospitalization
- New-onset atrial fibrillation/flutter
- Non-sustained/sustained ventricular tachycardia
- Major bradyarrhythmias
- Myocardial infarction with non-obstructive coronary arteries
Model performance
As reported in the abstract
3-year
- Multivariable model (age, LVMI, number of LGE+ segments, T1 value) showed a good performance in predicting the composite endpoint
- Median follow-up 40 months
Further findings
Association of CMR markers with the composite endpoint
| HR (CI) | P value | ||
|---|---|---|---|
| LVMI | 1.02 (1.02–1.03) | P < 0.001 | |
| Presence of late gadolinium enhancement (LGE) | 20.12 (6.02–67.24) | P < 0.001 | |
| Number of LGE + segments | 1.51 (1.33–1.71) | P < 0001 | |
| Septal T1Measured in both ShMOLLI and MOLLI sequences | 0.98 (0.97–0.99) | <0.001 | |
| 0.050.21520Hazard ratio (log scale) |
Adverse cardiovascular (CV) events in patients with normal T1
- None of the patients with normal T1 experienced adverse CV events
Original publication
Prognostic role of myocardial storage, hypertrophy, and inflammation/fibrosis in Fabry cardiomyopathy: a new predictive score including T1 values. Eur Heart J Qual Care Clin Outcomes. Published 15 December 2025. doi:10.
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