Last reviewed · How we verify

NCT03838237: MAIORA

Effect of Migalastat on Cardiac Involvement in Fabry Disease

Completed Last updated 18 March 2021
What this trial tests

trial testing Cardiological evaluation in Fabry Disease in 18 participants. Completed in 22 January 2021.

Timeline
10 January 2018
Primary endpoint
22 January 2021
22 January 2021

Quick facts

Lead sponsorOspedale San Donato
StatusCompleted
Study typeOBSERVATIONAL
Enrollment18
Start date10 January 2018
Primary completion22 January 2021
Estimated completion22 January 2021
Sites1 location across Italy

Drugs / interventions tested

Conditions studied

Sponsor

Ospedale San Donato — full company profile →

Who can join

16 and older, any sex, with Fabry Disease or Heart Diseases. Patients with the condition only — healthy volunteers not accepted.

Sponsor's own description

Anderson-Fabry Disease (AFD) is one of the rare lysosomal storage disorders for which a cause - specific therapy is available. Recently, a new specific drug has been marketed, namely Migalastat, a small-molecule pharmacological chaperone. The effect of Migalastat on cardiac involvement has been assessed so far by 2D echocardiography, demonstrating a significant reduction in left ventricular (LV) mass after 18 months of therapy. Calculation of LV mass by 2D echocardiography is limited by geometrical assumptions and quality of echocardiographic window, with a strong impact on accuracy. Cardiac Magnetic Resonance (CMR) overcomes these limitations, thus representing the gold standard technique for ventricular mass, volumes and function estimation. Moreover, CMR offers the unique possibility to perform a non-invasive tissue characterization, including the detection of both myocardial fibrosis by Late Gadolinium Enhancement and sphingolipid storage by T1 mapping. Beyond an accurate morphological description and a detailed tissue characterization, a complete cardiological assessment should also integrate functional data and bio-humoral profile. This study is designed to provide a comprehensive evaluation of the therapeutic effect of Migalastat (123 mg every other day) on cardiac involvement after 18 months of therapy, integrating a morphological, functional and bio-humoral assessment.

Publications & conference data

2 peer-reviewed publications reference this trial (live from Europe PMC):

  1. 2021 TSOC Expert Consensus on the Clinical Features, Diagnosis, and Clinical Management of Cardiac Manifestations of Fabry Disease.
    Hung CL, Wu YW, Lin CC, Lai CH, et al · · 2021 · cited 29× · PMID 34257484 · DOI 10.6515/acs.202107_37(4).20210601a
  2. Effect of Migalastat on cArdiac InvOlvement in FabRry DiseAse: MAIORA study.
    Camporeale A, Bandera F, Pieroni M, Pieruzzi F, et al · · 2023 · cited 22× · PMID 36669872 · DOI 10.1136/jmg-2022-108768

Verify or expand the search:

Other recruiting trials for Fabry Disease

Currently open trials in the same condition.

Other Ospedale San Donato trials

Trials by the same sponsor.

Verify against primary sources

Data sources for this page

Drug Landscape aggregates and links these public records for informational use only. Always verify against the primary source before clinical or regulatory decisions. Canonical URL: https://druglandscape.com/trial/NCT03838237.

Primary sources · FDA · ClinicalTrials.gov · EMA · SEC EDGAR · ChEMBL · Wikidata · full sourcing