Central Arterial Function Measured by Non-invasive Pulse Wave Analysis is Abnormal in Patients with Duchenne Muscular

Thomas D Ryan1, John J Parent2, Zhiqian Gao3

  • 1Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45220, USA. thomas.ryan@cchmc.org.

Pediatric Cardiology
|June 23, 2017
PubMed

Insights

Patients with Duchenne muscular dystrophy (DMD) show abnormal arterial wave reflection, indicating increased arterial stiffness despite lower central blood pressure. Further research is needed to understand these vascular function changes.

Area of Science:

  • Cardiovascular Medicine
  • Neuromuscular Disorders
  • Vascular Physiology

Background:

  • Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder impacting muscle function due to dystrophin mutation.
  • Cardiovascular complications, including dilated cardiomyopathy, are common in DMD patients.
  • Non-invasive vascular function assessment methods have not been extensively evaluated in DMD.

Purpose of the Study:

  • To investigate arterial wave reflection as a non-invasive marker of vascular function in patients with DMD.
  • To test the hypothesis that arterial wave reflection is abnormal in individuals with DMD.

Main Methods:

  • Pulse wave analysis was conducted using a SphygmoCor SCOR-PVx System.
  • Central blood pressure and augmentation index (AIx) were measured to assess arterial wave reflection.
  • DMD patients (n=43) were compared to a control group of healthy individuals (n=43).

Main Results:

  • Patients with DMD exhibited higher augmentation index (AIx-75 and AIx-not corrected) compared to controls.
  • Central systolic blood pressure was lower in the DMD group.
  • Multivariable regression identified DMD diagnosis, height, and heart rate as significant factors affecting AIx-not corrected.

Conclusions:

  • Despite lower central systolic blood pressure, DMD patients demonstrate increased arterial wave reflection, suggesting heightened arterial stiffness.
  • Ventricular systolic function appears unaffected in the short term, but long-term consequences require further investigation.
  • The observed vascular differences may be linked to systemic steroid use or the role of dystrophin in vascular health.

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