Usefulness of tissue doppler imaging for assessing aortic wall stiffness in children with the marfan syndrome

doi:10.1016/j.amjcard.2003.12.067

The American Journal of Cardiology

Volume 93, Issue 8, 15 April 2004, Pages 1072-1075

https://doi.org/10.1016/j.amjcard.2003.12.067 Get rights and content

Abstract

The purpose of this study was to assess wall motion velocities of the abdominal aorta using tissue Doppler imaging (TDI) in 12 patients with Marfan syndrome and 30 age-matched normal controls. The patients with Marfan syndrome displayed an increased aortic stiffness index in the abdominal aorta, and significant relationships were observed between wall motion velocities of the abdominal aorta and the aortic stiffness index. Thus, TDI analyzes aortic expansion and contraction velocities and allows the identification of abdominal aortic stiffness in patients with Marfan syndrome.

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Cited by (31)

Aortic Dimensions, Biophysical Properties, and Plasma Biomarkers in Children and Adults with Marfan or Loeys-Dietz Syndrome
2021, CJC Open
Citation Excerpt :
Of interest was the finding that although the BMI in MFS patients and the younger MFS subgroup was less than in control participants, the BMI in older MFS subgroup was not, suggesting a relative increase in weight. Different methods of measuring arterial stiffening have been used in a number of studies of MFS.19,26-28 Because the elastic aorta acts as a conduit and a cushion for pulsatile arterial flow, stiffness of the aorta is manifested by increased PWV.
Aortic dilation, stiffening, and dissection are common and potentially lethal complications of Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS), which involve abnormal transforming growth factor beta (TGF-β) signalling. The relation of aortic dimensions, stiffness, and biomarker levels is unknown. The objective of this study was to measure aortic dimensions, stiffness, TGF-β and matrix metalloproteinase (MMP) levels, and endothelial function in patients with MFS, and to compare TGF-β levels in patients with MFS receiving different therapeutic regimens.
This was a cohort study of 40 MFS and 4 LDS patients and 87 control participants. Aortic dimension and stiffness indexes, including pulse wave velocity (PWV), were measured using echocardiography and Doppler. Total and free TGF-β and MMP blood levels were measured using Quantikine (R&D Systems, Inc, Minneapolis, MN) and Quanterix (Billerica, MA) kits. Endothelial function was measured using brachial artery flow-mediated dilation.
PWV was increased in patients with MFS. There were increased MMP-2 levels in those with MFS but no increase in free or total TGF-β or MMP-9 levels compared with control participants. There was no difference in TGF-β levels between MFS patients receiving no medications, angiotensin receptor blockers, and β-blockers. PWV correlated most strongly with age. Endothelial function showed premature gradual decline in patients with MFS.
Despite the increased PWV, monitoring aortic stiffness or TGF-β levels would not be helpful in patients with MFS. TGF-β levels were not increased and the increased MMP-2 levels suggest consideration of a different therapeutic target.
La dilatation, la rigidification et la dissection de l’aorte sont des complications fréquentes et parfois mortelles du syndrome de Marfan (SM) et du syndrome de Loeys-Dietz (SLD), qui sont tous deux dûs à une anomalie de la voie de signalisation du facteur de croissance transformant bêta (TGF-β). On ne connaît pas la relation entre les dimensions et la rigidité de l’aorte et la présence de biomarqueurs. Notre étude visait à mesurer les dimensions et la rigidité de l’aorte, les taux de TGF-β et de métalloprotéases matricielles (MMP) et la fonction endothéliale chez des patients atteints du SM, et à les comparer aux taux de TGF-β observés chez des patients également atteints de SM, mais recevant un autre traitement.
Il s’agissait d’une étude de cohorte menée auprès de 40 patients atteints du SM et de quatre patients atteints du SLD, ainsi que de 87 témoins. Les indices des dimensions et de la rigidité aortiques, y compris la vitesse d’onde de pouls (VOP), ont été mesurés par échocardiographie et par échographie Doppler. Les taux sanguins de TGF-β et de MMP totaux et libres ont été mesurés à l’aide de trousses Quantikine (R&D Systems, Inc, Minneapolis, MN) et Quanterix (Billerica, MA). La fonction endothéliale a été mesurée par dilatation liée au flux dans l’artère brachiale.
La VOP était plus élevée chez les patients atteints du SM. On a aussi observé une hausse des taux de MMP-2 chez les patients atteints de SM, mais aucune augmentation des taux de TGF-β ou de MMP-9 libres ou totaux comparativement aux témoins. Il n’y avait pas de différence entre les taux de TGF-β chez les patients atteints de SM ne recevant aucun traitement, ceux qui prenaient un antagoniste des récepteurs de l’angiotensine et ceux qui prenaient un bêtabloquant. La VOP été plus fortement corrélée avec l’âge. La fonction endothéliale a affiché un déclin progressif prématuré chez les patients atteints du SM.
Malgré l’augmentation de la VOP, il ne semble pas utile de surveiller la rigidité aortique ni les taux de TGF-β en cas de SM. Les taux de TGF-β n’étaient pas plus élevés chez les patients atteints du SM, et la hausse des taux de MMP-2 indique qu’il conviendrait de choisir une autre cible thérapeutique.
Elastic Aortic Properties in Patients with X Syndrome
2014, Heart Lung and Circulation
Citation Excerpt :
Another novelty of this study is that we used TDI to examine the aortic wall. The only other studies that used this technique for the aortic wall were by Vitarelli and Harada in Marfan syndrome [15–17]. Strain represents the difference between the reference state of the wall and the state of deformation, and is expressed as a percentage of end-systolic aortic wall length.
Elastic properties of the aorta represent an important determinant of left ventricular function and coronary blood flow but there are few data about aortic stiffness in patients with X syndrome.
To investigate the elastic aortic proprieties (aortic stiffness and distensibility) and arterial wall motion velocities as measured by tissue Doppler imaging (TDI) in patients with cardiac X syndrome.
Fifteen patients with X syndrome (typical chest pain and angiographically normal coronary arteries associated with a positive exercise test) were enrolled in the study. The control group consisted of 15 healthy patients. The aortic elastic indexes, namely distensibility (cm² dyne⁻¹) and stiffness index (β index) were calculated from M-mode echocardiographically-derived thoracic aortic diameters using accepted formulae, and TDI parameters were measured on the wall of the ascending aorta 3 cm above the aortic valve. Anterior wall aortic expansion velocity (S), early (E) and late (A) diastolic retraction velocity and peak systolic strain were determined.
Aortic elastic proprieties were more impaired in the syndrome X group than in the control group. Aortic distensibility was significantly lower in the syndrome X group (3.2 ± 1.3 vs. 7.95 ± 4 cm² dyne⁻¹, p < 0.001), while stiffness index was higher (7.3 ± 2.1 vs. 4.1 ± 1.6, p < 0.001) than in the control group. Peak systolic (S) and diastolic waves (E and A waves) of the aortic wall TDI were similar in patients and controls (S wave: 5.7 ± 1.6 cm/s vs. 5.8 ± 1.6 cm/s, E wave: −4.8 ± 2.0 vs. −4.1 ± 2.0 cm/s; A wave: −4.32 ± 2.1 vs. −4.76 ± 1.8 cm/s) while tissue strain of the aortic wall was lower in patients with X syndrome than controls (−12.80 ± 7% vs. −22.3 ± 5.9%, p < 0.00001).
Deterioration in aortic elasticity properties in patients with cardiac syndrome X suggests that this disease may be a more generalised disturbance of the vasculature.
Marfan Syndrome and Related Disorders
2013, Emery and Rimoin's Principles and Practice of Medical Genetics
Aortic stiffness: Current understanding and future directions
2011, Journal of the American College of Cardiology
Citation Excerpt :
Patients with Marfan syndrome have genetic mutation in the FBN-1 resulting in aortic root dilation and subsequent aortic dissection. Increased aortic wall stiffness seems to start early in childhood and progresses with aging (52). Thus, tissue Doppler imaging can be used to assess alteration of aortic mechanics with good correlation and prediction for aortic dilation and dissection (53).
The aorta stiffens with aging, a process that is accelerated by arterial hypertension. Decreased arterial compliance is one of the earliest detectable manifestations of adverse structural and functional changes within the vessel wall. The use of different imaging techniques optimized for assessment of vascular elasticity and quantification of luminal and vessel wall parameters allows for a comprehensive and detailed view of the vascular system. In addition, several studies have also documented the prognostic importance of arterial stiffness (AS) in various populations as an independent predictor of cardiovascular morbidity and all-cause mortality. Measurement of AS by applanation tonometry with pulse-wave velocity has been the gold-standard method and is well-validated in large populations as a strong predictor of adverse cardiovascular outcomes. Because aortic stiffness depends on the prevailing blood pressure, effective antihypertensive treatment is expected to reduce it in proportion to the blood pressure reduction. Nevertheless, drugs lowering blood pressure might differ in their effects on structure and function of the arterial walls. This review paper not only will discuss the current understanding and clinical significance of AS but also will review the effects of various pharmacological and nonpharmacological interventions that can be used to preserve the favorable profile of a more compliant and less stiff aorta.
Systemic circulation
2010, Paediatric Cardiology
Systemic Circulation
2009, Paediatric Cardiology

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Brief reportUsefulness of tissue doppler imaging for assessing aortic wall stiffness in children with the marfan syndrome

Abstract

Metabolism

J Am Coll Cardiol

Am Heart J

Ultrasound Med Biol

Ultrasound Med Biol

Abnormalities of lipid metabolism and arterial rigidity in young subjects with borderline hypertension

Clin Invest Med

Age dependency of stiffness of the abdominal aorta and the mechanical properties of the aorta in Kawasaki disease in children

Pediatr Cardiol

Brief report
Usefulness of tissue doppler imaging for assessing aortic wall stiffness in children with the marfan syndrome