Spinal Muscular Atrophy and Cardiac Autonomic Function

Study Overview
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Brief Summary
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Respiratory dysfunction is the major cause of morbidity and mortality in patients with spinal muscular atrophy (SMA). Air stacking is a clearance airway technique frequently used but its effects on cardiac autonomic function in patients with spinal muscle atrophy is not clear. Objective: To evaluate the acute effect of air stacking and posture on cardiac autonomic function in patients with spinal muscular atrophy types II and III. Methods: Patients with spinal muscle atrophy type II and III will be recruited. Electrocardiogram signals will be recorded for analyses of heart rate variability during air stacking in supine and sitting position.

Detailed Description
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Background: Respiratory dysfunction is the major cause of morbidity and mortality in patients with spinal muscular atrophy. Air stacking is a clearance airway technique frequently used but its effects on cardiac autonomic function in patients with spinal muscle atrophy is not clear. Objective: To evaluate the acute effect of air stacking and posture on cardiac autonomic function in patients with spinal muscular atrophy types II and III. Methods: Patients with spinal muscle atrophy type II and III will be recruited. Electrocardiogram signals will be recorded for analyses of heart rate variability during air stacking in supine and sitting position. Data will collected before, during and after air stacking and will be compared using Anova Repeated Measures or Kruskal-Wallis Anova on Ranks, followed by Tukey test. The relationship between heart rate variability indexes and age will be evaluated by Pearson correlation. Significant level will be set at 5%.

Official Title
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Spinal Muscular Atrophy and Cardiac Autonomic Function

Conditions
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Spinal Muscular Atrophy

Intervention / Treatment
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* Procedure: Air stacking manuever


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: diagnosis of SMA types II and III, absence of acute respiratory disease in the last two weeks, agreement to participate in the study, according written informed consent and at least 5 years old. Exclusion Criteria: respiratory disease in the last two weeks

Ages Eligible for Study
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Minimum Age: 5 Years
Maximum Age: 25 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Air stacking<br>Electrocardiogram signals were recorded for analyses of heart rate variability during air stacking in supine and sitting position. | Procedure: Air stacking manuever<br>* Electrocardiogram signals were recorded for analyses of heart rate variability during air stacking in supine and sitting position<br>* Other names: HRV during air stacking;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Cardiac autonomic function | Evaluation of heart rate variability in patients with spinal muscular atrophy | Thirty minutes during air stacking manuever | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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heart rate variability, posture, air stacking, spinal muscular atrophy