Functional Capacity in Anderson-Fabry Disease Patients

NCT ID: NCT06884358

Last Updated: 2025-03-19

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Total Enrollment

100 participants

Study Classification

OBSERVATIONAL

Study Start Date

2024-11-13

Study Completion Date

2032-01-31

Brief Summary

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The goal of this observational study is to observe the relation between excercise parameters - assessed by CPET - and rest/stress hemodynamic parameters - assessed by echocardiogram and CMR - in patients with a genetic diagnosis of Anderson-Fabry Disease.

Participants will undergo:

* baseline evaluation: clinical evaluation, disease staging with FASTEX and MSSI, KCCQ for quality of life assessment, resting 12-leads ECG, 6MWT, CPET-ESE and contrast-enhanced CMR;
* before 36 months from baseline: resting 12-leads ECG, 2D rest and stress echocardiogram, CPET-ESE, contrast-enhanced CMR, disease staging with FASTEX and MSSI and KCCQ for quality of life assessment;
* up to 7 years from baseline: clinical follow-up.

Detailed Description

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Conditions

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Anderson-Fabry Disease

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Normal cardiac parameters and normal T1

AFD patients with normal cardiac parameters and normal T1.

No interventions assigned to this group

Normal cardiac parameters and reduced T1

AFD patients with no LVH and myocardial reduced T1 .

No interventions assigned to this group

LVH without LGE

Patients with LVH without LGE.

No interventions assigned to this group

Advanced cardiomyopathy with LVH and LGE

AFD patients with advanced cardiomyopathy with LVH and LGE.

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* Patients with a genetic diagnosis of AFD, according to current guidelines;
* Informed written consent with the understanding that consent may be withdrawn by the patient at any time without prejudice to future medical care (for patients age \<18 years old, written consent from a caregiver is mandatory).

Exclusion Criteria

* eGFR \<30 ml/min and other contraindications for CMR (relative controindication: patients with implantable device);
* Musculoskeletal limitation for exercise test on the cyclo ergometer;
* Pregnant or breastfeeding women;
* Any serious medical or psychiatric illness that could, in the investigator's opinion, potentially interfere with a full comprehension of the written consent form.
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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IRCCS Policlinico S. Donato

OTHER

Sponsor Role lead

Responsible Party

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Massimo Piepoli

MD, PhD

Responsibility Role PRINCIPAL_INVESTIGATOR

Locations

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Spedali Civili Hospital

Brescia, Brescia, Italy

Site Status ACTIVE_NOT_RECRUITING

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

Milan, Milan, Italy

Site Status ACTIVE_NOT_RECRUITING

IRCCS Policlinico San Donato

San Donato Milanese, Milan, Italy

Site Status RECRUITING

Fondazione IRCCS San Gerardo dei Tintori

Monza, Monza, Italy

Site Status ACTIVE_NOT_RECRUITING

Regina Margherita Hospital

Turin, Turin, Italy

Site Status ACTIVE_NOT_RECRUITING

Countries

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Italy

Central Contacts

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Massimo Piepoli, MD, PhD

Role: CONTACT

+39 0252774942

Gianluigi Guida, MD

Role: CONTACT

+39 0252774942

Facility Contacts

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Massimo Piepoli, MD, PhD

Role: primary

+39 0252774942

References

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Linhart A, Germain DP, Olivotto I, Akhtar MM, Anastasakis A, Hughes D, Namdar M, Pieroni M, Hagege A, Cecchi F, Gimeno JR, Limongelli G, Elliott P. An expert consensus document on the management of cardiovascular manifestations of Fabry disease. Eur J Heart Fail. 2020 Jul;22(7):1076-1096. doi: 10.1002/ejhf.1960. Epub 2020 Aug 14.

Reference Type BACKGROUND
PMID: 32640076 (View on PubMed)

Reant P, Testet E, Reynaud A, Bourque C, Michaud M, Rooryck C, Goizet C, Lacombe D, de-Precigout V, Peyrou J, Cochet H, Lafitte S. Characterization of Fabry Disease cardiac involvement according to longitudinal strain, cardiometabolic exercise test, and T1 mapping. Int J Cardiovasc Imaging. 2020 Jul;36(7):1333-1342. doi: 10.1007/s10554-020-01823-7. Epub 2020 May 8.

Reference Type BACKGROUND
PMID: 32385539 (View on PubMed)

Bierer G, Kamangar N, Balfe D, Wilcox WR, Mosenifar Z. Cardiopulmonary exercise testing in Fabry disease. Respiration. 2005 Sep-Oct;72(5):504-11. doi: 10.1159/000087675.

Reference Type BACKGROUND
PMID: 16210890 (View on PubMed)

Lobo T, Morgan J, Bjorksten A, Nicholls K, Grigg L, Centra E, Becker G. Cardiovascular testing in Fabry disease: exercise capacity reduction, chronotropic incompetence and improved anaerobic threshold after enzyme replacement. Intern Med J. 2008 Jun;38(6):407-14. doi: 10.1111/j.1445-5994.2008.01669.x.

Reference Type BACKGROUND
PMID: 18613897 (View on PubMed)

Ditaranto R, Leone O, Lovato L, Niro F, Cenacchi G, Papa V, Baldovini C, Ferracin M, Salamon I, Kurdi H, Parisi V, Capelli I, Pession A, Liguori R, Potena L, Seri M, Martin Suarez S, Galie N, Moon JC, Biagini E. Correlations Between Cardiac Magnetic Resonance and Myocardial Histologic Findings in Fabry Disease. JACC Cardiovasc Imaging. 2023 Dec;16(12):1629-1632. doi: 10.1016/j.jcmg.2023.06.011. Epub 2023 Aug 2. No abstract available.

Reference Type BACKGROUND
PMID: 37542504 (View on PubMed)

Linhart A, Elliott PM. The heart in Anderson-Fabry disease and other lysosomal storage disorders. Heart. 2007 Apr;93(4):528-35. doi: 10.1136/hrt.2005.063818. No abstract available.

Reference Type BACKGROUND
PMID: 17401074 (View on PubMed)

Pieroni M, Moon JC, Arbustini E, Barriales-Villa R, Camporeale A, Vujkovac AC, Elliott PM, Hagege A, Kuusisto J, Linhart A, Nordbeck P, Olivotto I, Pietila-Effati P, Namdar M. Cardiac Involvement in Fabry Disease: JACC Review Topic of the Week. J Am Coll Cardiol. 2021 Feb 23;77(7):922-936. doi: 10.1016/j.jacc.2020.12.024.

Reference Type BACKGROUND
PMID: 33602475 (View on PubMed)

Other Identifiers

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OPTIMA-FD

Identifier Type: -

Identifier Source: org_study_id

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