2D Strain Evaluation: Children With Duchenne Muscular Dystrophy Versus Healthy Children

Evaluation of Myocardial Function by Two Dimensional Speckle Tracking Echocardiography in Children With Duchenne Muscular Dystrophy Versus Healthy Children: a Comparative Transversal Multicenter Study

Compare systolic function of left ventricle (LV) and right ventricle (VD) by 2D strain evaluation in Duchenne muscular dystrophy children versus a control group.

Study Overview

• Status: Completed
• Conditions: Duchenne Muscular Dystrophy
• Intervention / Treatment: Other: speckle tracking (2D strain) echocardiography

Detailed Description

Among the new echocardiographic techniques, two dimensional Speckle Tracking Imaging or "2D Strain" allows a dynamic study of regionalized and global ventricular contraction. With software for the spatial and temporal processing of a standard 2D image, this technique measures the displacement of natural markers present on the myocardial wall.

Used in adult cardiology since 2000, it could be eligible to pediatric population because of many potential applications. In Duchenne muscular dystrophy, myocardial disease leads to death in the third decade. Early diagnosis and preventive therapy remain a challenge. No clinic, biologic or radiologic markers can predict myocardial disease before advanced and irreversible heart failure.

Recent animals and human studies suggest that 2D strain anomalies could predict left ventricular systolic dysfunction in Duchenne muscular dystrophy. If it is confirmed, 2D strain could be used as early marker of myocardial disease in routine patient follow-up and in future therapeutic studies.

Purpose : - To compare systolic function of left ventricle (LV) and right ventricle (RV) by 2D strain evaluation in Duchenne muscular dystrophy children versus a control group.

• To correlate 2D Strain variables to conventional LV and RV echo variables.
• To evaluate 2D strain feasibility in normal children and Duchenne muscular myopathy population

• Study Type: Interventional
• Enrollment (Actual): 99
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Montpellier, France, 34295
    • Pediatric and Congenital Cardiology and Pulmonology Department, Arnaud De Villeneuve University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: No older than 13 years (Child)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Duchenne's muscular dystrophy (group 1) followed in our institution's referral center of neuromuscular diseases, during regular annual echocardiography follow-up.
• Healthy children (group 2), addressed in pediatric cardiology

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Diagnostic
• Allocation: Non-Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: dmd children; echocardiography	Other: speckle tracking (2D strain) echocardiography; 2D echocardiography videos of both ventricles to perform 2D strain post-examination evaluation
Other: healthy children; echocardiography	Other: speckle tracking (2D strain) echocardiography; 2D echocardiography videos of both ventricles to perform 2D strain post-examination evaluation

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
global longitudinal LV 2D strain	day 1

Collaborators and Investigators

• Sponsor: University Hospital, Montpellier
• Investigators: Principal Investigator: Pascal AMEDRO, MD, PhD, UH Montpellier; Principal Investigator: Pierre MP Meyer, MD, PhD, UH Montpellier; Principal Investigator: Arnaud BERTRAND, MD, PhD, UH Montpellier; Principal Investigator: Jean-Michel RAUZIER, MD, PhD, UH Nîmes; Principal Investigator: Charlène BREDY, MD, PhD, UH Montpellier; Principal Investigator: François RIVIER, MD, PhD, UH Montpellier; Principal Investigator: Grégoire DE LA VILLEON, MD, PhD, UH Montpellier; Principal Investigator: Ulrike WALTHER-LOUVIER, MD, PhD, UH Montpellier; Principal Investigator: Sophie GUILLAUMONT, MD, PhD, UH Montpellier; Principal Investigator: Camille SOULATGES, MD, PhD, UH Montpellier; Principal Investigator: Marie VINCENTI, MD, PhD, UH Montpellier

Publications and helpful links

General Publications

• Amedro P, Vincenti M, De La Villeon G, Lavastre K, Barrea C, Guillaumont S, Bredy C, Gamon L, Meli AC, Cazorla O, Fauconnier J, Meyer P, Rivier F, Adda J, Mura T, Lacampagne A. Speckle-Tracking Echocardiography in Children With Duchenne Muscular Dystrophy: A Prospective Multicenter Controlled Cross-Sectional Study. J Am Soc Echocardiogr. 2019 Mar;32(3):412-422. doi: 10.1016/j.echo.2018.10.017. Epub 2019 Jan 21.
• Lanot N, Vincenti M, Abassi H, Bredy C, Agullo A, Gamon L, Mura T, Lavastre K, De La Villeon G, Barrea C, Meyer P, Rivier F, Meli AC, Fauconnier J, Cazorla O, Lacampagne A, Amedro P. Assessment of left ventricular dyssynchrony by speckle tracking echocardiography in children with duchenne muscular dystrophy. Int J Cardiovasc Imaging. 2022 Jan;38(1):79-89. doi: 10.1007/s10554-021-02369-y. Epub 2021 Dec 14.

Study record dates

Study Major Dates

• Study Start: January 1, 2014
• Primary Completion (Actual): June 1, 2017
• Study Completion (Actual): June 1, 2017

Study Registration Dates

• First Submitted: April 13, 2015
• First Submitted That Met QC Criteria: April 15, 2015
• First Posted (Estimate): April 16, 2015

Study Record Updates

• Last Update Posted (Actual): June 7, 2019
• Last Update Submitted That Met QC Criteria: June 5, 2019
• Last Verified: June 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Nervous System Diseases; Genetic Diseases, Inborn; Genetic Diseases, X-Linked; Musculoskeletal Diseases; Muscular Diseases; Neuromuscular Diseases; Muscular Disorders, Atrophic; Muscular Dystrophies; Muscular Dystrophy, Duchenne
• Other Study ID Numbers: 9458 (Other Identifier: CTEP)