Assessment of aortic stiffness in marfan syndrome using two-dimensional and Doppler echocardiography

Anatoli Kiotsekoglou1, James C Moggridge, Samir K Saha

  • 1Department of Cardiac and Vascular Sciences, St. George's University of London, London, UK.

Abstract

Insights

Pulsed-wave velocity (PWV) effectively measures increased aortic stiffness in Marfan syndrome (MFS) patients. This non-invasive echocardiography technique aids in diagnosing and managing MFS by assessing aortic wall changes.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Marfan syndrome (MFS) is characterized by extracellular matrix remodeling, leading to increased aortic stiffness (AoS).
  • Pulsed-wave velocity (PWV) is a key non-invasive indicator for assessing AoS.
  • Echocardiography offers a valuable tool for evaluating cardiovascular parameters in MFS.

Purpose of the Study:

  • To evaluate the efficacy of PWV measurements in assessing aortic stiffness in Marfan syndrome patients.
  • To compare PWV values between MFS patients and healthy controls using echocardiography.
  • To determine the utility of PWV as a diagnostic and management tool for MFS.

Main Methods:

  • Thirty-one MFS patients and 31 controls underwent two-dimensional and Doppler echocardiography.
  • PWV was calculated by measuring the time difference (TD) in blood flow transit between the ascending and descending aorta.
  • Simultaneous electrocardiography and B-stiffness measurements were also performed.

Main Results:

  • MFS patients exhibited significantly increased time differences (TD) and aortic arch length compared to controls (P < 0.001).
  • PWV values were significantly higher in MFS patients (7.20 m/s) versus controls (4.64 m/s) (P < 0.001).
  • B-stiffness was also significantly elevated in MFS patients (P < 0.001), with reproducible PWV measurements (<5% variability).

Conclusions:

  • Aortic stiffness is significantly elevated in Marfan syndrome patients.
  • PWV measurement via echocardiography is a feasible and reproducible method for assessing AoS in MFS adults.
  • This technique can be a valuable aid in the diagnosis and management of Marfan syndrome.

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