Quantitative assessment of left ventricular longitudinal function and myocardial deformation in Duchenne muscular

Roman Panovský1,2, Martin Pešl3,4,5, Jan Máchal3,6

  • 1International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic. panovsky@fnusa.cz.

Insights

Cardiac magnetic resonance (CMR) strain analysis and mitral annular plane systolic excursion (MAPSE) can detect early left ventricular (LV) dysfunction in Duchenne muscular dystrophy (DMD) patients, even with normal ejection fraction and no late gadolinium enhancement.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Duchenne muscular dystrophy (DMD) primarily affects skeletal muscles but also causes cardiac dysfunction.
  • Assessing early cardiac involvement in DMD patients via echocardiography is challenging.
  • Cardiac magnetic resonance (CMR) offers advanced imaging capabilities for cardiac assessment.

Purpose of the Study:

  • To evaluate the utility of CMR strain analysis and MAPSE in detecting early left ventricular (LV) dysfunction in DMD patients.
  • To compare CMR-derived parameters between DMD patients and healthy controls.
  • To identify sensitive markers for subclinical cardiac impairment in DMD.

Main Methods:

  • 51 male DMD patients and 18 controls underwent CMR imaging.
  • Feature tracking (FT) software was used for MAPSE measurement and functional analysis.
  • Patients were categorized into three groups based on late gadolinium enhancement (LGE) and LV ejection fraction (LVEF).

Main Results:

  • DMD patients with LGE and reduced LVEF (Group A) and those with LGE but normal LVEF (Group B) showed significantly reduced MAPSE, global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) compared to controls.
  • DMD patients without LGE and normal LVEF (Group C) exhibited significantly reduced MAPSE and GCS compared to controls.
  • These findings indicate impaired LV systolic function even in the absence of LGE and with normal LVEF.

Conclusions:

  • CMR strain analysis and MAPSE are sensitive tools for assessing early cardiac dysfunction in DMD patients.
  • These methods can detect subclinical left ventricular systolic impairment.
  • FT and MAPSE provide valuable insights into early cardiac involvement in DMD, aiding timely intervention.
Abstract