Incidence and risk factors for development of left ventricular hypertrophy in Fabry disease
Authors' conclusion
Approximately one-quarter of patients with FD developed LVH during follow-up. Age, male sex and ECG abnormalities were associated with a higher risk of developing LVH in patients with FD.
Study context
- Population
- Adults (aged ≥16 years) with Fabry disease and no left ventricular hypertrophy (LVH) at first evaluationof a cohort of 393
- Participants
- 214 adults
- Outcome
- First detection of LVH (LV mass index ≥115 g/m² in men, ≥95 g/m² in women)
Methodological detailsobservational, specialist cardiomyopathy centre, consecutively referred
- Observational
- Specialist cardiomyopathy centre
- Consecutively referred
Main finding
As reported in the abstract
Incidence of LVH · median follow-up 9.4 years (IQR 4.7-12.7)
Developed LVH during follow-up
55 patients
24.6%
Estimated incidence at 5 years
11.3%
95% CI 6.5% to 16.1%
Estimated incidence at 10 years
29.1%
95% CI 21.5% to 36.7%
Estimated incidence at 15 years
45.0%
95% CI 33.8% to 62.4%
Observational data: associations, not effects.
Further findings
| Outcome | HR (95% CI) | P value | |
|---|---|---|---|
| Independent predictors of LVH development (multivariable)Age · per 1-year increase | 1.04 (1.02–1.06) | P < 0.001 | |
| 0.512Hazard ratio (log scale) |
Independent predictors of LVH development (multivariable)
| HR (95% CI) | P value | ||
|---|---|---|---|
| Male sex | 2.90 (1.66–5.09) | P < 0.001 | |
| Abnormal ECG | 3.10 (1.72–5.57) | P < 0.001 | |
| 0.20.5125Hazard ratio (log scale) |
g/m²/year
| Outcome | Males | Females | P value |
|---|---|---|---|
| Annual rate of change in LV mass index, by sex | +2.77 (IQR 1.45–4.62) | +1.38 (IQR 0.09–2.85) | P < 0.001 |
Original publication
Incidence and risk factors for development of left ventricular hypertrophy in Fabry disease. Heart. Published 30 April 2024. doi:10.
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