Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy - ACE Inhibitor Therapy Trial

Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy - Angiotensin-Converting-Enzyme (ACE) Inhibitor Therapy

Status

Completed

Phases

Phase 3

Study type

Interventional

Source

ClinicalTrials.gov

Registry ID

NCT02432885

Enrollment

Registered

2015-05-04

Start date

2009-06-30

Completion date

2013-06-30

Last updated

2015-05-04

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Muscular Dystrophies, Myocardial Fibrosis

Keywords

cardiac magnetic resonance, myocardial fibrosis, muscular dystrophies

Brief summary

This trial intends to evaluate myocardial Fibrosis progression in Duchenne and Becker Muscular Dystrophy, as well the influence of ACE inhibitors in fibrosis progression. Additionally, this study aims to determine genetic predictors of cardiac involvement in these dystrophies.

Detailed description

Duchenne and Becker muscular dystrophies (DMD/BMD) are diseases characterized by progressive skeletal muscle degeneration and replacement by fibrofatty tissue. Data on cardiac involvement (defined as myocardial fibrosis), effect of ACE-inhibitors and specific genetic mutations on myocardial involvement detected by cardiac magnetic resonance (CMR) is lacking. The study will include 76 patients with DMD/BMD. All patients will be referred to two CMRs for assessment of ventricular function and myocardial fibrosis. Patients with myocardial fibrosis and normal left ventricle ejection fraction (LVEF) will be randomized into two groups, each group receiving ACE-inhibitor treatment or no treatment for cardiomyopathy. A genetic profile will be performed in every patient to identify possible mutations related to cardiac involvement.

Interventions

DRUGEnalapril

up to 20mg bid

Study design

Allocation

RANDOMIZED

Intervention model

PARALLEL

Primary purpose

TREATMENT

Masking

NONE

Eligibility

Sex/Gender

ALL

Age

6 Years to No maximum

Healthy volunteers

Inclusion criteria

* Patients with biopsy-proven Muscular Dystrophy of Duchenne or Becker

Exclusion criteria

* Contraindications to cardiovascular magnetic resonance imaging

Design outcomes

Primary

Measure	Time frame	Description
Quantitative Myocardial Fibrosis by CMR in patients with and without ACE inhibitor therapy	2 years	Progression of myocardial fibrosis

Secondary

Measure	Time frame	Description
Specific genetic mutations as predictors of cardiac involvement	2 years	Relation of dystrophin gene site mutations in exons \<45 relation and the extent of myocardial fibrosis measured by cardiac magnetic resonance

Outcome results

None listed

Source: ClinicalTrials.gov · Data processed: Feb 28, 2026