Effects of Home Mechanical Ventilation on Left Ventricular Function in Sarcoglycanopathies (Limb Girdle Muscular Dystrophies)
Abdallah Fayssoil
(1, 2, 3, 4, 5, 6, 7)
,
Lee Nguyen
(1, 2, 3, 4, 5, 6, 7)
,
Adam Ogna
(1, 2, 3, 4, 5, 6, 7)
,
Paris Meng
(1, 2, 3, 4, 5, 6, 7)
,
Olivier Nardi
(1, 2, 3, 4, 5, 6, 7)
,
Pascal Laforet
(1, 2, 3, 4, 5, 6, 7)
,
Bernard Clair
(1, 2, 3, 4, 5, 6, 7)
,
Helene Prigent
(1, 2, 3, 4, 5, 6, 7)
,
Frederic Lofaso
(1, 2, 3, 4, 5, 6, 7)
,
France Leturcq
(1, 2, 3, 4, 5, 6, 7)
,
Rabah Ben Yaou
(8, 1, 2, 3, 4, 5, 6, 7)
,
Djillali Annane
(1, 2, 3, 4, 5, 6, 7)
,
David Orlikowski
(1, 2, 3, 4, 5, 6, 7)
1
CIC 1429 -
Centre d’Investigation Clinique 1429 [Garches]
2 Hôpital Raymond Poincaré (Garches) [GHU AP-HP Université Paris-Saclay]
3 UVSQ - Université de Versailles Saint-Quentin-en-Yvelines
4 Institut de Myologie
5 CHU Pitié-Salpêtrière [AP-HP]
6 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
7 Hôpital Cochin [AP-HP]
8 Centre de recherche en Myologie – U974 SU-INSERM
2 Hôpital Raymond Poincaré (Garches) [GHU AP-HP Université Paris-Saclay]
3 UVSQ - Université de Versailles Saint-Quentin-en-Yvelines
4 Institut de Myologie
5 CHU Pitié-Salpêtrière [AP-HP]
6 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
7 Hôpital Cochin [AP-HP]
8 Centre de recherche en Myologie – U974 SU-INSERM
Abdallah Fayssoil
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Lee Nguyen
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Adam Ogna
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Paris Meng
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Olivier Nardi
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Pascal Laforet
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Bernard Clair
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Helene Prigent
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Frederic Lofaso
- Fonction : Auteur
France Leturcq
- Fonction : Auteur
Rabah Ben Yaou
- Fonction : Auteur
Djillali Annane
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- PersonId : 761134
- ORCID : 0000-0001-6805-8944
- IdRef : 070300917
David Orlikowski
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Résumé
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.