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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.
Background:
Extracellular matrix remodeling in the aortic wall results in increased aortic stiffness (AoS) in Marfan syndrome (MFS). Pulsed-wave velocity (PWV) constitutes the best indirect AoS measurement. We aimed to assess PWV in MFS patients using two-dimensional (2D) and Doppler echocardiography.
Methods:
Thirty-one MFS patients, (mean age 31 ± 14 years, 16 men) and 31 controls were examined. Blood flow was recorded in the aorta near the aortic valve and immediately after in the descending aorta with simultaneous electrocardiography. PWV was calculated by dividing the distance between the two sample volume positions (D) by the time difference (TD) between the intervals from the QRS start to the ascending and descending aortic flow onsets. B-stiffness was also measured.
Results:
TD (described in "Methods" section) and, aortic arch length were significantly increased in MFS patients, P < 0.001. Thus, PWV values were significantly higher in patients when compared with controls, 7.20 m/s (5.12, 9.43) versus 4.64 m/s (3.37, 6.24), P < 0.001. B-stiffness was also significantly increased in MFS patients; 5.15 (3.69, 7.65) versus 2.44 (1.82, 3.66), P < 0.001. Multiple regression analysis showed a positive association with MFS diagnosis and age, (P = 0.002 and 0.009, respectively). Reproducibility of PWV measurements was <5%.
Conclusions:
AoS was significantly higher in MFS patients as expected. Our data demonstrated that PWV measurements can be performed, in the absence of serious musculoskeletal abnormalities in MFS adults, as part of a cardiac ultrasound scan. This technique can be helpful in diagnosis and management in MFS.
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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