Natural History of Cardiac and Respiration Function in Patients With Muscular Dystrophies on Home Mechanical Ventilation

Study Overview
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Brief Summary
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Muscular dystrophies are inherited disorders that affect skeletal muscle. Cardiac and respiratory function may be affected in this group of diseases. The investigators sought to analyze the long term cardiac and respiratory function in patients with muscular dystrophies recquiring home mechnaical ventilation .

Detailed Description
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Muscular dystrophies are neuromuscular disorders with disability and can affect respiratory muscle function and left ventricular function. Medical management of patients with cardiomyopathy relies mainly on angiotensin-converting-enzyme inhibitors and beta blockers. Respiratory management relies on home mechanical ventilation and couph assist. The investigators aim to analyze cardiac and respiratory function over time in patients with muscular dystrophies followed at the home mechanical ventilation unit. to determine the long term impact of the home mechanical ventilation on cardiac and respiratory events and to define the predictive factors associated. Patients will be included retrospectively. Tissue Doppler imaging and Tricuspid annular plane systolic excursion were used for the assessment of the right ventricle. Left ventricular ejection fraction was used for the analysis of the left ventricle. Respiratory function was assessed using the vital capacity, the maximal inspiratory pressure, the maximal expiratory pressure, the peak couph flow rate.

Official Title
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Natural History of Cardiac and Respiratory Function in Patients With Muscular Dystrophies on Home Mechanical Ventilation

Conditions
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Heart, Respiratory, Disease, Functional

Intervention / Treatment
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* Other: no intervention


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age higher or equal to 18 years Duchenne muscular dystrophy Becker muscular dystrophy Sarcoglycanopathy limb girdle muscular dystrophy Pompe disease Steinert disease FSH disease Others myopathies Exclusion Criteria: Minor patient Sepsis condition

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 70 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Other: no intervention|The study will not include any intervention.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| predictive factors for long term survival |  | 10 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| predictuve factors for cardiac events |  | 10 years | 
| predictive factors for respiratory events |  | 10 years | 
| cumulative cardiac events incidence |  | 10 years | 
| cumulative respiratory events incidence |  | 10 years | 
| annual rate of left ventricular ejection fraction decline |  | 10 years | 
| annual rate of respiratory vital capacity decline |  | 10 years | 
| annual rate of maximal inspiratory pressure decline |  | 10 years | 
| annual rate of maximal expiratory pressure decline |  | 10 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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cardiomyopathy, echocardiography, prognosis, respiratory, home mechanical ventilation, vital capacity, maximal inspiratory pressure, maximal expiratory pressure