Muscular dystrophy
Conditions
Interventions
None listed
Sponsors
Hokkaido University Hospital
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patient group: 1. Patients 6 years of age or older are eligible. 2. Patients diagnosed as Duchenne muscular dystrophy, Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy, limb-girdle muscular dystrophy, or myotonic dystrophy are eligible. 3. Patients who are scheduled for cardiac magnetic resonance imaging are eligible. 4. Patients who can be scheduled for cardiac magnetic resonance imaging 24 months after the first scan are eligible. 5. Patients who provided informed consent, parental permission, or assent are eligible. Muscular dystrophy carrier group: 1. Proven or suspected muscular dystrophy carriers 20 years of age or older are eligible. 2. Proven or suspected carriers of Duchenne or Becker muscular dystrophy who have at least one first-degree male relatives with a previously established Duchenne or Becker muscular dystrophy diagnosis. 3. Proven or suspected carriers who are scheduled for cardiac magnetic resonance imaging are eligible. 4. Proven or suspected carriers who can be scheduled for cardiac magnetic resonance imaging 24 months after the first scan are eligible. 5. Proven or suspected carriers who provided informed consent are eligible. Normal volunteer group: 1. Persons 20 years of age or older are eligible. 2. Persons without cardiac or kidney disease are eligible. 3. Persons who provided informed consent are eligible.
Exclusion criteria
Exclusion criteria: 1. Patients with impaired renal function (estimated glomerular filtration rate <30 mL/min/1.73m2) 2. Patients with claustrophobia 3. Patients expected to live a year or less 4. Patients who contact persons judge as ineligible for this study
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The prespecified primary outcome of the study is to assess improvement of left ventricular ejection fraction 24 months after the first cardiovascular magnetic resonance imaging in patients with muscular dystrophy, suspected muscular dystrophy carriers, and volunteers. | — |
Secondary
| Measure | Time frame |
|---|---|
| The prespecified secondary outcomes of the study are as follows: 1. To assess predictors of the occurrence of left ventricular dysfunction (left ventricular ejection fraction <50% or decrease by 5% or more per year) in patients with muscular dystrophy or suspected muscular dystrophy carriers. 2. To assess the association between absolute change and percent change in left ventricular ejection fraction per year and baseline values of the extent of late gadolinium enhancement, T1 mapping measurements, circumferential strain analysis by the tagging method, coronary flow reserve, echocardiography measurements, and biomarkers. 3. To assess the cross-sectional and longitudinal association between left ventricular ejection fraction, the extent of late gadolinium enhancement, T1 mapping measurements, circumferential strain analysis by the tagging method, coronary flow reserve, echocardiography measurements, and biomarkers. 4. To assess predictors of all-cause death, cardiovascular death, left ventricular assist device implantation, cardiac transplantation, heart failure admission, life-threatening arrhythmia (ventricular tachycardia, ventricular fibrillation). | — |
Countries
Japan
Contacts
Public ContactTadao Aikawa
Faculty of Medicine and Graduate School of Medicine, Hokkaido University Department of Cardiovascular Medicine
Outcome results
None listed