Heart, Respiratory, Disease, Functional Clinical Trial
Official title:
Natural History of Cardiac and Respiratory Function in Patients With Muscular Dystrophies on Home Mechanical Ventilation
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 .
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.
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