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Am J Cardiol. 2018 Jul 15;122(2):353-355. doi: 10.1016/j.amjcard.2018.04.001. Epub 2018 Apr 16.

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

Author information

1
CHU Raymond Poincaré, APHP, Université de Versailles Saint Quentin en Yvelines, Garches, France; Service de Neurologie, Institut de Myologie, APHP, CHU Pitié Salpetrière, Paris, France; Center of Clinical Investigation Paris-Est, Pitié Salpetrière, APHP, ICAN, Sorbonne Université, Paris, France; Service de Physiologie-Exploration fonctionnelles, CHU Raymond Poincaré, APHP, Université de Versailles Saint Quentin en Yvelines, Garches, France; Laboratoire de biochimie et génétique moléculaire, Hôpital Cochin, AP-HP, Université Paris Descartes-Sorbonne Paris Cité, Paris, France; Centre d'Investigation clinique et Innovation technologique CIC 14.29, APHP, CHU Raymond Poincaré, Garches, France. Electronic address: abdallah.fayssoil@aphp.fr.
2
CHU Raymond Poincaré, APHP, Université de Versailles Saint Quentin en Yvelines, Garches, France; Service de Neurologie, Institut de Myologie, APHP, CHU Pitié Salpetrière, Paris, France; Center of Clinical Investigation Paris-Est, Pitié Salpetrière, APHP, ICAN, Sorbonne Université, Paris, France; Service de Physiologie-Exploration fonctionnelles, CHU Raymond Poincaré, APHP, Université de Versailles Saint Quentin en Yvelines, Garches, France; Laboratoire de biochimie et génétique moléculaire, Hôpital Cochin, AP-HP, Université Paris Descartes-Sorbonne Paris Cité, Paris, France; Centre d'Investigation clinique et Innovation technologique CIC 14.29, APHP, CHU Raymond Poincaré, Garches, France.

Abstract

Cardiac and respiratory function may be impaired in sarcoglycanopathies, a subgroup of muscular dystrophies due to sarcoglycan 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 insufficiency. 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 HMV unit of the Raymond Poincare University Hospital (4 patients with α-sarcoglycanopathy and 6 patients with γ-sarcoglycanopathy). We collected cardiorespiratory clinical baseline data and left ventricular ejection fraction (LVEF) at baseline before initiation of HMV and at the end of follow-up. At baseline, median age was 30.5 years (27 to 39) and median pulmonary vital capacity was 27% of the predicted value (21 to 36). Forty percent of the patients had documented sleep apnea. Cardiomyopathy, defined as LVEF <50%, was found in 3 patients with γ-sarcoglycanopathy. After a median follow-up of 3 years (1.0 to 4.5), there was a significant increase in LVEF after initiation of HMV, that is, 62% (48 to 65) versus 53% (45.5 to 56.5) (p = 0.0039). In conclusion, HMV in sarcoglycanopathies is not harmful and may protect left ventricular function by its thoracic physiological effects.

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