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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.
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.
Abstract:
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder caused by mutation of dystrophin. Cardiovascular involvement includes dilated cardiomyopathy. Non-invasive assessment of vascular function has not been evaluated in DMD. We hypothesize arterial wave reflection is abnormal in patients with DMD. Pulse wave analysis was performed on DMD patients with a SphygmoCor SCOR-PVx System to determine central blood pressure and augmentation index (AIx) as an assessment of arterial wave reflection. Results were compared to a control group. A total of 43 patients with DMD were enrolled, and compared to 43 normal controls. Central systolic blood pressure was lower, while both AIx-75 (7.8 ± 9.6% vs. 2.1 ± 10.4%, p 0.01, DMD vs. normal) and AIx-not corrected (16.8 ± 10.1% vs. -3.6 ± 10.9, p < 0.001, DMD vs. normal) were higher in the DMD compared to control. Using multivariable linear regression model, the variables found to have a significant effect on AIx-not corrected included diagnosis of DMD, height, and heart rate (r 2 = 0.257). The current data suggest that, despite lower central systolic blood pressure, patients with DMD have higher wave reflection when compared to normal controls, which may represent increased arterial stiffness. Overall there appears to be no effect on ventricular systolic function, however the long-term consequence in this group is unknown. Further study is required to determine the mechanism of these differences, which may be related to the effects of systemic steroids or the role of dystrophin in vascular function.
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