Effects of Personalized Training at Home Combining Endurance and Resistance in Patients Suffering From Marfan Syndrome

Marfan Syndrome Clinical Trial

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Official title:

Evaluation of the Effects of Personalized Training at Home Combining Endurance and Resistance in Patients Suffering From Marfan Syndrome

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04553094
• Other study ID #: 2020-01
• Status: Completed
• Phase: N/A
• Start date: January 4, 2021
• Est. completion date: September 30, 2022

Study information

• Verified date: September 2022
• Source: French Cardiology Society
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

Marfan syndrome (MS) is an autosomal dominant genetic disorder caused by a mutation in the fibrillin-1 gene (FBN1) encoding the protein fibrillin-1. Fibrillin is the main component of microfibrils, elements found in all of the body's tissues, and this pathology is characterized by the multitude of its clinical manifestations. These patients may develop aneurysms in the aortic root and one of the main factors of morbidity in patients with MS is aortic dissection. Prevention mainly involves preventive aortic surgery. However, the repercussions are global and can affect the functioning of other tissues such as skeletal muscle tissue, bone tissue, lung tissue and the eyes. The association of skeletal (scoliosis, hyperlaxity), muscular and ocular disorders is clearly associated with an impairment in the quality of life. These disorders are associated with pain and disability which affect professional activity, leisure and family life. Physical activity could represent a relevant alternative for these patients. A recent animal study suggests that moderate training is beneficial.

Description:

The main objective is to show that the quality of life of patients with Marfan syndrome can be improved by personalized training at home. The goal is to be able to offer new non-drug management based on physical activity to these patients. Improved quality of life; Improvement of muscle strength by a muscle strengthening protocol; Improvement of cardiovascular function parameters. Regular coaching throughout the study. 1. / Assessment of muscular capacities 2. / Assessment of cardiovascular and respiratory capacities 3. / Response to psychometric questionnaires 4. / Participation in a training circuit (training protocol)

Recruitment information / eligibility

• Status: Completed
• Enrollment: 72
• Est. completion date: September 30, 2022
• Est. primary completion date: September 30, 2022
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years to 75 Years

Eligibility

Inclusion Criteria:

• patient with Marfan syndrome and followed up at Bichat Claude Bernard hospital, Paris. Marfan syndrome should be diagnosed according to the Ghent nosology revised in 2010
• Patient taking protective treatment for the aorta (beta blocker, calcium channel blocker, ACE inhibitor, angiotensin II receptor antagonist).
• Valid patient, able to return to consultation and carry out functional assessments and personalized physical training at home.
• Patients operated prophylactically on the ascending aorta may be included at a distance > 6 months from their surgery.

Exclusion Criteria:

• Myocardial pathology other than Marfan syndrome.
• Thoracic aorta dissection.
• Aortic diameter > 45 millimeters.
• Uncontrolled high blood pressure at rest (systolic blood pressure > 140 Millimeter of mercury and diastolic blood pressure > 90 Millimeter of mercury)
• Increase in systolic blood pressure > 160 Millimeter of mercury during exercise.
• Pregnancy at the time of the study

Related Conditions & MeSH terms

• Marfan Syndrome
• Syndrome

Intervention

Other:
• Endurance training
Aerobic circuit training
• Muscle building training
Muscle strengthening circuit training

Locations

Country	Name	City	State
France	Groupe Hospitalier Bichat - Claude Bernard	Paris

Sponsors (1)

Lead Sponsor	Collaborator
French Cardiology Society

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Quality of life assessment: questionnaire	Assessed with Medical Outcome Study Short Form 36 questionnaire, a scale scored on a 0 to 100 range so that the lowest is the worth condition and highest is the best condition.	3 months
Secondary	Pain assessment: Fibromyalgia Rapid Screening Tool	Assessed with Fibromyalgia Rapid Screening Tool	3 months
Secondary	Self-perception assessment	Assessed with Physical Self Inventory questionnaire, a scale scoring 25 items rated on a 6-point scale ranging from 1 (Not at all) to 6 (Entirely) and assessing 6 dimensions (global self-worth (10-50), physical self-worth (10-50), physical condition (10-50), sport competence (10-40), physical attractiveness (10-30), and physical strength (10-30)) of the physical self-conception.	3 months
Secondary	Aortic diameter	Evolution of the aortic diameter (in millimeter) in echocardiography, safety cardiovascular parameters	3 months
Secondary	Blood pressure	Evolution of the systolic and diastolic blood pressures (in Millimeter of mercury) during exercise, monitoring of physiological parameters	3 months
Secondary	Heart rate	Evolution of heart rate (Beats per minute) during exercise, monitoring of physiological parameters	3 months
Secondary	oxygen consumption	Evolution of oxygen consumption (in liter) during exercise, monitoring of physiological parameters	3 months
Secondary	Maximal voluntary contraction	Evolution of maximal voluntary contraction (in Newtons) during exercise, monitoring of physiological parameters	3 months
Secondary	Left Ventricular Ejection Fraction (LVEF)	Evolution of Left Ventricular Ejection Fraction (in percentage) at rest, monitoring of cardiovascular parameters.	3 months
Secondary	Global longitudinal strain (GLS)	Evolution of GLS (in percentage) at rest, monitoring of cardiovascular parameters	3 months