Effects of Home Mechanical Ventilation on Left Ventricular Function in Sarcoglycanopathies (Limb Girdle Muscular Dystrophies)

Cardiac and respiratory function may be impaired in sarcoglycanopathies, a subgroup of muscular dystrophies due to sarcoglycans proteins ( α-, β-, γ-, and δ) genes mutations. Management of patients with restrictive respiratory failure mainly relies on home mechanical ventilation (HMV). Little is known about the cardiac effects of prolonged mechanical ventilation in patients with muscular dystrophy and restrictive respiratory insuff iciency. We aimed to assess the effects of HMV on cardiac function in sarcoglycanopathies.We retrospectively included 10 genetically proven patients with sarcoglycanopathy followed at the home mechanical ventilation unit of the Raymond Poincare university hospital (4 patients with α-sarcoglycanopat hy and 6 patients with γ-sarcoglycanopathy).

Source: The American Journal of Cardiology - April 16, 2018 Category: Cardiology Authors: Abdallah Fayssoil, Lee S. Nguyen, Adam Ogna, Paris Meng, Olivier Nardi, Pascal Laforet, Bernard Clair, Helene Prigent, Frederic Lofaso, France Leturcq, Rabah Ben Yaou, Djillali Annane, David Orlikowski Source Type: research