Left ventricular hypertrophy secondary to Fabry disease: mechanical dispersion and arrhythmic burden
Key result
LVH in Fabry disease linked to ~34 ms higher mechanical dispersion and more PVCs.
- P<0.001
- n=91
Why the study?
Fabry disease can lead to left ventricular hypertrophy, and mechanical dispersion is linked to ventricular dysrhythmias and sudden death in several cardiomyopathies, prompting evaluation of mechanical dispersion and arrhythmic burden in these patients.
Does the presence of left ventricular hypertrophy in Fabry disease patients associate with increased mechanical dispersion and arrhythmic burden?
Population
91 Fabry disease patients followed at a Reference Center of Lysosomal Storage Disorders
Comparison
FD patients with LVH vs FD patients without LVH
Design
Prospective study
Authors
Discussion
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Member takes
Implication
Higher MD and PVC burden in FD with LVH may prompt closer arrhythmia surveillance; leaves open whether MD improves risk stratification beyond LVH.
Study Design
Type
Observational (n=91)
Multicenter
No
Structured PICO
Does the presence of left ventricular hypertrophy in Fabry disease patients associate with increased mechanical dispersion and arrhythmic burden?
P
Population
91 patients with Fabry disease (62.6% female) followed at a reference center, evaluated for mechanical dispersion and arrhythmic burden.
C
Comparator
Fabry disease patients without left ventricular hypertrophy (LVH)
O
Outcome
Mechanical dispersion and arrhythmic burden (premature ventricular contractions and non-sustained ventricular tachycardia)surrogate
Main Result
Absolute Event Rate: 72.4% vs 38.3%
p-value: p=<0.001
In patients with Fabry disease, left ventricular hypertrophy is associated with worse global longitudinal strain, higher mechanical dispersion, and a greater burden of premature ventricular contractions.
Cite This Study
Fernandes et al. (2021) conducted an observational in Fabry disease (n=91). Left ventricular hypertrophy vs. No left ventricular hypertrophy was evaluated on Mechanical dispersion (p=<0.001). In patients with Fabry disease, left ventricular hypertrophy was associated with higher mechanical dispersion (72.4 vs 38.3 ms; p<0.001) and a higher prevalence of premature ventricular contractions.