Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Muscular dystrophies are inherited disorders that affect skeletal muscle. Cardiac and respiratory function may be affected in this group of diseases. The investigators sought to analyze the long term cardiac and respiratory function in patients with muscular dystrophies recquiring home mechnaical ventilation .
Muscular dystrophies are neuromuscular disorders with disability and can affect respiratory muscle function and left ventricular function. Medical management of patients with cardiomyopathy relies mainly on angiotensin-converting-enzyme inhibitors and beta blockers. Respiratory management relies on home mechanical ventilation and couph assist.
The investigators aim
Patients will be included retrospectively. Tissue Doppler imaging and Tricuspid annular plane systolic excursion were used for the assessment of the right ventricle. Left ventricular ejection fraction was used for the analysis of the left ventricle. Respiratory function was assessed using the vital capacity, the maximal inspiratory pressure, the maximal expiratory pressure, the peak couph flow rate.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| no intervention | Other | The study will not include any intervention. |
| Measure | Description | Time Frame |
|---|---|---|
| predictive factors for long term survival | 10 years |
| Measure | Description | Time Frame |
|---|---|---|
| predictuve factors for cardiac events | 10 years | |
| predictive factors for respiratory events | 10 years | |
| cumulative cardiac events incidence |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Adult patients with muscular dystrophies
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Abdallah FAYSSOIL, MDPhD | HOPITAL RAYMOND POINCARE | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hopital Raymond Poincare | Garches | 92380 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 37581390 | Derived | Fayssoil A, Mansencal N, Nguyen LS, Nardi O, Yaou RB, Leturcq F, Amthor H, Wahbi K, Becane HM, Lofaso F, Prigent H, Bassez G, Behin A, Stojkovic T, Fontaine B, Duboc D, Dubourg O, Clair B, Laforet P, Annane D, Orlikowski D. Prognosis of Right Ventricular Systolic Dysfunction in Patients With Duchenne Muscular Dystrophy. J Am Heart Assoc. 2023 Aug 15;12(16):e027231. doi: 10.1161/JAHA.122.027231. Epub 2023 Aug 10. | |
| 27526372 |
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D004194 | Disease |
| D009202 | Cardiomyopathies |
| ID | Term |
|---|---|
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
Not provided
Not provided
Not provided
Not provided
Not provided
| 10 years |
| cumulative respiratory events incidence | 10 years |
| annual rate of left ventricular ejection fraction decline | 10 years |
| annual rate of respiratory vital capacity decline | 10 years |
| annual rate of maximal inspiratory pressure decline | 10 years |
| annual rate of maximal expiratory pressure decline | 10 years |
| Derived |
| Fayssoil A, Lazarus A, Wahbi K, Ogna A, Nardi O, Lofaso F, Clair B, Orlikowski D, Annane D. Cardiac implantable electronic devices in tracheotomized muscular dystrophy patients: Safety and risks. Int J Cardiol. 2016 Nov 1;222:975-977. doi: 10.1016/j.ijcard.2016.08.040. Epub 2016 Aug 6. |
| 27120200 | Derived | Fayssoil A, Ogna A, Chaffaut C, Chevret S, Guimaraes-Costa R, Leturcq F, Wahbi K, Prigent H, Lofaso F, Nardi O, Clair B, Behin A, Stojkovic T, Laforet P, Orlikowski D, Annane D. Natural History of Cardiac and Respiratory Involvement, Prognosis and Predictive Factors for Long-Term Survival in Adult Patients with Limb Girdle Muscular Dystrophies Type 2C and 2D. PLoS One. 2016 Apr 27;11(4):e0153095. doi: 10.1371/journal.pone.0153095. eCollection 2016. |