Clinical Trial: Cardiovascular Consequences of NIV Withdrawal in Patients With Myotonic Dystrophy
Study Status: Recruiting
Recruit Status: Unknown status
Study Type: Interventional
Official Title: Four Weeks Withdrawal of Non-invasive Ventilation (NIV) in Patients With Myotonic Dystrophy: Cardiovascular, Metabolic and Daytime Vigilance Induced Changes
Brief Summary:
Background: Myotonic dystrophy lead to highly heterogeneous, multisystemic symptoms including myotonia, progressive muscle weakness, cardiac conduction defects, cataract, metabolic dysfunction, and excessive daytime somnolence. This last symptom is related to respiratory failure and/or to involvement of the central nervous system. However the metabolic disturbances could contribute to it. From the respiratory point of view this disease is characterised by the progressive appearance of respiratory failure of muscular origin but mainly associated with a defect in the central respiratory drive. The treatment for this hypoventilation is non-invasive ventilation (NIV).
It is not currently absolutely clear as to the best choice of criteria to judge long term effectiveness of NIV. The most usual criteria are normalisation of daytime blood gases, diminution of respiratory work, improvement in daytime symptoms and improvement in sleep structure. Other criteria are currently little studied, for instance the contribution of the interaction between alveolar hypoventilation and oxygen desaturation during the night and biological deficiencies such as systemic inflammation, glucose intolerance or insulin resistance. Likewise there is little information about the interaction between alveolar hypoventilation and endothelial dysfunction and arterial stiffness both being accurate predictive factors for cardiovascular risks.
Aim: to evaluate the impact of NIV on endothelial dysfunction in patients with myotonic dystrophy. The secondary objectives are to assess the impact of NIV on systemic inflammation, arterial stiffness, insulin-resistance, quality of sleep, and daytime vigilance in these patients.
Methods: Patients with chronic alveolar hypoventilation already treated by long term NIV will
Detailed Summary:
NIV is a technique of assisted ventilation that does not use the endotracheal route as the interface between the patient and the ventilator. NIV by positive pressure assistance involves ventilating the patient by means of a mask adjusted on the nose or covering the nose and mouth. This technique is now the recommended therapeutic strategy for the treatment of chronic alveolar hypoventilation. It improves survival and quality of life, and improves daytime blood gases in patients suffering form chronic restrictive respiratory failure.
For each check-up (3 visits), patients will have a polysomnography, a complete respiratory function measurement (Flows and lung volumes, CO2 sensitivity test, SNIF test, blood gazes analysis), a test of endothelial function (peripheral arterial tone), a test of arterial stiffness (pulse wave velocity), an assessment of systemic inflammation (ultra sensitive CRP, TNFa, IL6), assessment of diurnal vigilance tests ( OSLER test and sleepiness scale) and assessment of metabolic and endocrinal function (insulinemia, glucose blood level, Leptin, DHEA).
Sponsor: University Hospital, Grenoble
Current Primary Outcome: To evaluate endothelial dysfunction (as measured by Peripheral arterial tone (PAT)) and its evolution after four weeks withdrawal of non-invasive ventilation (NIV). [ Time Frame: 8 weeks ]
Original Primary Outcome: Same as current
Current Secondary Outcome: To assess arterial stiffness, systemic inflammation (IL6, TNFα, Leptin, CRP), insulin resistance, DHEA, sleep quality, objective and subjective daytime somnolence and their evolution after four weeks withdrawal of NIV. [ Time Frame: 8 weeks ]
Original Secondary Outcome: Same as current
Information By: University Hospital, Grenoble
Dates:
Date Received: September 1, 2008
Date Started: June 2008
Date Completion: December 2012
Last Updated: August 10, 2012
Last Verified: September 2008
