Steinert's Disease
Conditions
Brief summary
Steinert's disease is an orphan disease. The prognosis of patients with this disease is conditioned by cardiac involvement. Search an early stage alterations in contractile function in subjects suffering from dystrophy would detect patients at risk of sudden death. The first stage of work is to validate the tools to detect early stage of infringement systolic function in a population of patients with myotonic dystrophy.
Interventions
Sponsors
Eligibility
Inclusion criteria
* patients suffering from muscular dystrophy type 1 followed CHU Poitiers. * age ≥ 18 years
Exclusion criteria
* duration QRS\> 120 ms ECG permanent ventricular stimulation * trouble segmental kinetic obvious to echocardiography in visual analysis
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Ventricular Ejection Fraction Left 3D | Immediately |
| Global longitudinal systolic Strain | Immediately |
| Strain of the dispersion index | Immediately |