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November 2, 2023Frontiers in Cardiovascular MedicineOpen Access

FD-specific treated patients showed stabilization of left atrial strain over time (p > 0.05) and stable left ventricular mass index (p > 0.05).

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Why the study?

Cardiomyopathy in Fabry disease is a major determinant of morbidity and mortality, prompting investigation into the effects of enzyme replacement therapy and chaperone therapy on left atrial function using 2DSTE.

Does enzyme replacement therapy or migalastat stabilize left atrial strain in patients with Fabry disease?

Population

20 migalastat-treated, 48 ERT-treated, and 30 untreated FD patients

Comparison

Migalastat vs ERT vs untreated controls

Design

Prospective observational single-center study

Follow-up

Mean follow-up ranged from 26 to 81 months

Authors

CPChristian PogodaSBStefan‐Martin BrandTDThomas Duning

Discussion

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Overview

Stabilization of LA strain with ERT or migalastat suggests disease control in FD; leaves open causal benefits in this observational cohort.

Structured PICO

Does enzyme replacement therapy or migalastat stabilize left atrial strain in patients with Fabry disease?

P
Population
98 patients with Fabry disease, including 20 treated with migalastat (50% female), 48 treated with ERT (50% female), and 30 untreated controls (100% female).
I
Intervention
Fabry disease-specific treatment using enzyme replacement therapy (agalsidase-alfa and agalsidase-beta) or chaperone therapy (migalastat)
C
Comparator
Untreated Fabry disease patients
O
Outcome
Left atrial strain (LAS) measured by two-dimensional speckle tracking echocardiography (2DSTE)surrogate

In patients with Fabry disease, treatment with enzyme replacement therapy or migalastat is associated with stabilization of left atrial strain and left ventricular mass index over time.

Cite This Study

Pogoda et al. (2023) studied this question.

synapsesocial.com/papers/6a7440ef73ed22dccea7f99bhttps://doi.org/10.3389/fcvm.2023.1223635
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Impact of enzyme replacement therapy and migalastat on disease progression in females with fabry disease2025
  2. 2Conventional and 2-Dimensional Speckle Tracking Echocardiography in Fabry Disease2024
  3. 3Utilization of Transthoracic Echocardiography and Biochemical Markers in Detecting Cardiomyopathy in Fabry Disease2025 · 2 citations
  4. 4Safety and efficacy of migalastat in Fabry's disease treatment with cardiac impact : a systematic review and meta-analysis2025
  5. 5Long-Term Effects of Enzyme Replacement Therapy on Fabry Cardiomyopathy2009 · 488 citations