scholarly journals Aortic rotational flow patterns and stiffness by 4D flow CMR in patients with Loeys-Dietz syndrome compared to healthy volunteers and patients with Marfan syndrome

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
A Ruiz Munoz ◽  
A Guala ◽  
JF Rodriguez-Palomares ◽  
L Dux-Santoy ◽  
L Servato ◽  
...  
Keyword(s):  
Healthy Volunteers ◽  
Marfan Syndrome ◽  
Aortic Stiffness ◽  
Aortic Flow ◽  
Aortic Diameter ◽  
Rotational Flow ◽  
4D Flow ◽  
National Budget ◽  
4D Flow Cmr ◽  
Public Grant

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): La Marató de TV3, Instituto de Salud Carlos III through the project and Spanish Ministry of Science, Innovation and Universities. BACKGROUND Loeys-Dietz (LDS) and Marfan (MFS) syndromes are rare genetic connective tissue disorders associated with progressive aortic dilation, however, aortic dissections have been observed at lower aortic root diameters in LDS than in MFS. Recent CMR studies in MFS patients reported increased aortic stiffness (1–3) and altered rotational flow (4), but research on aortic flow dynamics and biomechanics in LDS is lacking. PURPOSE The aim of this study was to assess rotational aortic flow and aortic stiffness in LDS compared to healthy volunteers (HV) and MFS patients, using 4Dflow CMR. METHODS Twenty-one LDS and 44 MFS patients, without previous aortic dissection or surgery, and 43 HV underwent a non-contrast-enhanced 4D flow CMR. Aortic stiffness was quantified at the AAo and DAo using pulse wave velocity (PWV). In-plane rotational flow (IRF), systolic flow reversal ratio (SFRR) (5) and local aortic diameters were obtained at 20 equidistant planes from the ascending (AAo) to the proximal descending aorta (DAo). RESULTS LDS patients had lower IRF at the distal AAo and proximal DAo compared to HV (p = 0.053 and 0.004, respectively), once adjusted for age, stroke volume and local aortic diameter; but no differences were found with respect to MFS (Figure). Although SFRR at the proximal DAo was increased in LDS patients compared to both HV (p = 0.037) and MFS populations (p = 0.015), once adjusted for age and aortic diameter, the difference in magnitude was small (Figure). On the other hand, AAo and DAo PWV revealed stiffer aortas in LDS patients compared to HV but no differences versus MFS patients (Table). CONCLUSIONS Patients with Loeys-Dietz syndrome showed decreased in-plane rotational flow and abnormally-high regional aortic stiffness compared to healthy controls, and similar hemodynamics and aortic stiffness with respect to patients with Marfan syndrome.

scholarly journals Aortic stiffness and hemodynamics in Loeys-Dietz syndrome by 4Dflow CMR: a comparison with healthy volunteers and patients with Marfan syndrome

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Ruiz Munoz ◽  
A Guala ◽  
J.F Rodriguez-Palomares ◽  
L Dux-Santoy ◽  
L Servato ◽  
...  
Keyword(s):  
Healthy Volunteers ◽  
Aortic Stiffness ◽  
Genetic Diseases ◽  
Aortic Flow ◽  
Public Institution ◽  
Aortic Diameter ◽  
Funding Source ◽  
Rotational Flow ◽  
4D Flow ◽  
4D Flow Cmr

Abstract Background Connective tissue disorders, such as Loeys-Dietz (LDS) and Marfan (MFS) syndromes, are rare genetic diseases associated with progressive aortic dilation. Aortic dissections have been observed at lower aortic root diameters in LDS than in MFS, and research on aortic flow dynamics and biomechanics in LDS is lacking. Purpose To evaluate rotational aortic flow and aortic stiffness in LDS compared to healthy volunteers (HV) and MFS patients, using 4Dflow CMR. Methods Twenty-one LDS and 44 MFS patients, without previous aortic dissection or surgery, and 44 HV underwent a non-contrast-enhanced 4D flow CMR. In-plane rotational flow (IRF), systolic flow reversal ratio (SFRR) and local aortic diameters were obtained at 20 equidistant planes from the ascending (AAo) to the proximal descending aorta (DAo). Aortic stiffness was quantified at the AAo and DAo using pulse wave velocity (PWV). Results LDS patients had lower IRF at the distal AAo and proximal DAo compared to HV (p=0.053 and 0.004, respectively), once adjusted for age, stroke volume and local aortic diameter; but no differences were found with respect to MFS (Figure). Although SFRR at the proximal DAo was increased in LDS patients compared to both HV (p=0.037) and MFS populations (p=0.015), once adjusted for age and aortic diameter, the difference in magnitude was small (Figure). On the other hand, AAo and DAo PWV revealed stiffer aortas in LDS patients compared to HV but no differences versus MFS patients (Table). Conclusions LDS patients showed decreased in-plane rotational flow and abnormally-high regional aortic stiffness compared to healthy controls, and similar hemodynamics and aortic stiffness with respect to MFS patients Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): Instituto de Salud Carlos III, La Maratό TV3

scholarly journals Quantitative 4D Flow CMR analysis of intracardiac blood flow energetics in ischemic cardiomyopathy patients

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Riva ◽  
A Camporeale ◽  
F Sturla ◽  
S Pica ◽  
L Tondi ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Ischemic Cardiomyopathy ◽  
Inverse Correlation ◽  
Lv Function ◽  
Funding Source ◽  
4D Flow ◽  
Anterior Myocardial Infarction ◽  
National Budget ◽  
4D Flow Cmr ◽  
Public Grant

Abstract Background Ischemic cardiomyopathy (ICM) is often associated with negative LV remodelling after myocardial infarction, sometimes resulting in impaired LV function and dilation (iDCM). 4D Flow CMR has been recently exploited to assess intracardiac hemodynamic changes in presence of LV remodelling. Purpose To quantify 4D Flow intracardiac kinetic energy (KE) and viscous energy loss (EL) and investigate their relation with LV dysfunction and remodelling. Methods Patients with prior anterior myocardial infarction underwent a CMR study with 4D Flow sequences acquisition; they were divided into ICM (n=10) and iDCM (n=10, EDV>208 ml and EF<40%). 10 controls were used for comparison. LV was semi-automatically segmented using short axis CMR stacks and co-registered with 4D Flow. Global KE and EL were computed over the cardiac cycle. NT-proBNP measurements were correlated with average and peak values, during systole and diastole. Results Both LV volume and EF significantly differ (P<0.0001) between iDCM (EDV=294±56 ml, EF=24±8%), ICM (EDV=181±32 ml, EF=34±6%) and controls (EDV=124±29 ml, EF=72±5%). If compared to controls, both ICM and iDCM showed significantly lower KE (P≤0.0008); though lower than controls, EL was higher in iDCM than ICM. Within the iDCM subgroup, diastolic mean KE and peak EL reported good inverse correlation with NT-proBNP (r=−0.75 and r=−0.69, respectively). EL indexed (ELI) to average KE during systole was higher in the entire ischemic group as compared to controls (ELI(ischemic) = 0.17 vs. ELI(controls) = 0.10, P=0.0054). Conclusions 4D Flow analyses effectively mapped post-ischemic LV energetic changes, highlighting the disproportionate intraventricular EL relative to produced KE; preliminary good correlation between LV energetic changes and NT-proBNP will deserve further investigation in order to contribute to early detection of heart failure. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Italian Ministry of Health

scholarly journals 108 4D-flow CMR demonstrates the regional distribution of aortic flow disturbance in Marfan syndrome

Heart ◽  
2011 ◽  
Vol 97 (Suppl 1) ◽  
pp. A62-A62 ◽  
Author(s):  
A. Pitcher ◽  
T. E. Cassar ◽  
J. Suttie ◽  
J. M. Francis ◽  
P. Leeson ◽  
...  
Keyword(s):  
Marfan Syndrome ◽  
Regional Distribution ◽  
Aortic Flow ◽  
4D Flow ◽  
Flow Disturbance ◽  
4D Flow Cmr

scholarly journals P372 Aortic stiffness in Loeys-Dietz syndrome: a comparison with Marfan syndrome patients and healthy volunteers

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Ruiz Munoz ◽  
A Guala ◽  
J F Rodriguez-Palomares ◽  
A Lopez-Sainz ◽  
C Granato ◽  
...  
Keyword(s):  
Healthy Volunteers ◽  
Marfan Syndrome ◽  
Aortic Stiffness ◽  
Pathogenic Mutation ◽  
P Value ◽  
4D Flow Mri ◽  
Aortic Diseases ◽  
4D Flow ◽  
Flow Mri ◽  
Mri Study

Abstract Funding Acknowledgements ISCIII PI14/0106 and PI17/00381, La Marató de TV3 (20151330), Eur FP7/People 267128 and CIBERCV BACKGROUND Genetic syndromic aortic diseases are rare, with Marfan syndrome (MFS) being the most common. However, less is known of Loeys-Dietz syndrome (LDS) which has much lower prevalence and presumed worse prognosis. Increased aortic stiffness in MFS has been previously described but no studies have evaluated aortic biomechanics in LDS. Pulse wave velocity (PWV) is the gold standard measure for arterial stiffness and can be quantified by 4D flow MRI. We aim to evaluate regional aortic PWV by 4D flow MRI in LDS compared to MFS and healthy volunteers. METHODS Sixteen LDS patients with a pathogenic mutation, 76 MFS and 49 healthy volunteers were prospectively and consecutively included. No patient had previous aortic dissection or surgery. All underwent a 4D flow MRI study in a 1.5 T clinical scanner. Ascending (AAo) and descending (DAo) aorta PWV were computed using wavelet analysis of the systolic upslope for transit time calculation (Figure). Statistical comparison was made with non-parametric analysis to account for the non-normality of data and multivariate analysis was evaluated separately for AAo and DAo PWV. RESULTS Ascending and descending aortic PWV revealed stiffer aortas in LDS patients than in healthy volunteers, even after adjustment for diameter of sinus of Valsalva (SoV) and sex. Conversely, no differences in aortic stiffness were found between LDS and MFS patients (Table). CONCLUSIONS Abnormally high regional aortic stiffness was observed in LDS patients when compared with controls. The severity of increased regional aortic stiffness was found similar to the one affecting MFS patients. Table Controls (N = 49) LDS (N = 16) MFS (N = 76) LDS vs. HV LDS vs. MFN Parameter Unadjusted p-value Adjusted p-value Unadjusted p-value Adjusted p-value Age [years] 39 ± 12 39 ± 16 36 ± 12 0.903 0.599 Men 32 (65%) 6 (37%) 34 (45%) 0.079 0.782 Weight [kg] 72 ± 11 69 ± 13 74 ± 16 0.288 0.194 Height [cm] 172 ± 8 172 ±12 181 ± 11 0.834 0.008 Systolic BP [mmHg] 126 ± 18 125 ± 14 127 ± 17 0.957 0.523 Diastolic BP [mmHg] 70 ± 11 77 ± 6 75 ± 12 0.011 0.318 SoV diameter [mm] 30.6 ± 3.9 35.4 ± 4.6 38.1 ± 5.9 0.001 0.060 AAo diameter [mm] 27.7 ± 3.8 29,0 ± 5.0 29.7 ± 5.4 0.458 0.579 DAo diameter [mm] 20.0 ± 2.0 21.3 ± 3.6 22.9 ± 3.8 0.546 0.124 AAo PWV [m/s] 5.2 ± 1.9 7.6 ± 2.4 7.3 ± 2.8 0.001 0.050* 0.534 NS DAo PWV [m/s] 7.1 ± 2.2 9.4 ± 2.6 10.7 ± 4.6 0.003 0.025** 0.493 NS Abstract P372 Figure

scholarly journals Reproducibility of left ventricular blood flow kinetic energy measured by four-dimensional flow CMR

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Ciaran Grafton-Clarke ◽  
Saul Crandon ◽  
Jos J. M. Westenberg ◽  
Peter P. Swoboda ◽  
John P. Greenwood ◽  
...  
Keyword(s):  
Blood Flow ◽  
Kinetic Energy ◽  
Healthy Volunteers ◽  
Statistical Power ◽  
Left Ventricular ◽  
Diastolic Filling ◽  
4D Flow ◽  
Energy Data ◽  
Dimensional Flow ◽  
4D Flow Cmr

Abstract Objectives Four-dimensional flow CMR allows for a comprehensive assessment of the blood flow kinetic energy of the ventricles of the heart. In comparison to standard two-dimensional image acquisition, 4D flow CMR is felt to offer superior reproducibility, which is important when repeated examinations may be required. The objective was to evaluate the inter-observer and intra-observer reproducibility of blood flow kinetic energy assessment using 4D flow of the left ventricle in 20 healthy volunteers across two centres in the United Kingdom and the Netherlands. Data description This dataset contains 4D flow CMR blood flow kinetic energy data for 20 healthy volunteers with no known cardiovascular disease. Presented is kinetic energy data for the entire cardiac cycle (global), the systolic and diastolic components, in addition to blood flow kinetic energy for both early and late diastolic filling. This data is available for reuse and would be valuable in supporting other research, such as allowing for larger sample sizes with more statistical power for further analysis of these variables.

scholarly journals Decreased rotational flow and circumferential wall shear stress as early markers of descending aorta dilation in Marfan syndrome: a 4D flow CMR study

2019 ◽  
Vol 21 (1) ◽  
Author(s):  
A. Guala ◽  
G. Teixido-Tura ◽  
L. Dux-Santoy ◽  
C. Granato ◽  
A. Ruiz-Muñoz ◽  
...  
Keyword(s):  
Shear Stress ◽  
Wall Shear Stress ◽  
Pulse Wave Velocity ◽  
Marfan Syndrome ◽  
Pulse Wave ◽  
Rotational Flow ◽  
4D Flow ◽  
Descending Aorta ◽  
Early Markers ◽  
Wall Shear

Abstract Background Diseases of the descending aorta have emerged as a clinical issue in Marfan syndrome following improvements in proximal aorta surgical treatment and the consequent increase in life expectancy. Although a role for hemodynamic alterations in the etiology of descending aorta disease in Marfan patients has been suggested, whether flow characteristics may be useful as early markers remains to be determined. Methods Seventy-five Marfan patients and 48 healthy subjects were prospectively enrolled. In- and through-plane vortexes were computed by 4D flow cardiovascular magnetic resonance (CMR) in the thoracic aorta through the quantification of in-plane rotational flow and systolic flow reversal ratio, respectively. Regional pulse wave velocity and axial and circumferential wall shear stress maps were also computed. Results In-plane rotational flow and circumferential wall shear stress were reduced in Marfan patients in the distal ascending aorta and in proximal descending aorta, even in the 20 patients free of aortic dilation. Multivariate analysis showed reduced in-plane rotational flow to be independently related to descending aorta pulse wave velocity. Conversely, systolic flow reversal ratio and axial wall shear stress were altered in unselected Marfan patients but not in the subgroup without dilation. In multivariate regression analysis proximal descending aorta axial (p = 0.014) and circumferential (p = 0.034) wall shear stress were independently related to local diameter. Conclusions Reduced rotational flow is present in the aorta of Marfan patients even in the absence of dilation, is related to aortic stiffness and drives abnormal circumferential wall shear stress. Axial and circumferential wall shear stress are independently related to proximal descending aorta dilation beyond clinical factors. In-plane rotational flow and circumferential wall shear stress may be considered as an early marker of descending aorta dilation in Marfan patients.

scholarly journals P6022Aortic geometry in marfan syndrome in relation to age: a 4D-flow CMR-derived study

2017 ◽  
Vol 38 (suppl_1) ◽  
Author(s):  
A. Guala ◽  
L. Dux-Santoy ◽  
G. Teixido-Tura ◽  
J. Rodriguez-Palomares ◽  
G. Maldonado ◽  
...  
Keyword(s):  
Marfan Syndrome ◽  
4D Flow ◽  
4D Flow Cmr

The length of the fusion between leaflets in bicuspid aortic valve is independently related to ascending aorta dilation and flow dynamics alterations assessed by 4D-flow CMR

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Guala ◽  
A Evangelista ◽  
L La Mura ◽  
G Teixido-Tura ◽  
L Dux-Santoy ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Statistical Significance ◽  
Ascending Aorta ◽  
Funding Source ◽  
Aortic Dilation ◽  
4D Flow ◽  
National Budget ◽  
4D Flow Cmr ◽  
Aortic Replacement

Abstract Background Aortic dilation in bicuspid aortic valve (BAV) patients has been related to altered flow patterns, which contribute to aortic wall degeneration. However, preventive aortic replacement is currently based on a diameter threshold. Several studies on excised BAV reported wide variability of fusion extent. Purpose To unveil whether leaflet fusion extent can be quantified by CMR and is related to aortic dilation and flow abnormalities in non-dysfunctional BAV. Methods One hundred and twenty adults with non-dysfunctional BAV and no previous aortic or aortic valve surgery and 28 healthy volunteers underwent double-oblique cine and 4D flow CMR. BAV patients with two sinuses of Valsalva or left and non-coronary cusps fusion were excluded. Peak systolic circumferential wall shear stress (WSSc) and pulse wave velocity (PWV) in the ascending aorta (AAo) were assessed by 4D flow CMR. Fusion length between leaflets was measured using a stack of double-oblique cine CMR images of the aortic valve. Results The length of the fusion was effectively measured in 112/120 (93%) patients with good reproducibility (ICC = 0.826) and showed great variability (range 2.3–15.4 mm, 7.8±3.2 mm and tertiles cut-off points 6 and 9.3 mm). In multivariate analysis adjusted for clinical and demographic characteristics and PWV, fusion length was independently associated with the diameter at the sinus of Valsalva (p=0.002) and the AAo (p=0.02) (Table). WSSc progressively increased with larger fusion length (Figure), with statistical significance (p<0.05) in the right and outer regions of the proximal and mid AAo. Conclusions Bicuspid aortic leaflet fusion length varies considerably, and it is independently associated with AAo and aortic root dilation, possibly through flow alterations. Figure 1. Maps of circumferential WSS Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study has been partially funded by Instituto Carlos III, Spanish Ministry of Science and Innovation (PI17/00381). Guala A. has received funding from the Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I).

Patients with blunt traumatic thoracic aortic injury treated with TEVAR present increased flow dynamics alterations and pulse wave velocity: a 4D flow CMR study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Guala ◽  
D Gil Sala ◽  
A Ruiz-Munoz ◽  
M.E Garcia Reyes ◽  
L Dux-Santoy ◽  
...  
Keyword(s):  
Pulse Wave Velocity ◽  
Wave Velocity ◽  
Aortic Stiffness ◽  
Pulse Wave ◽  
Aortic Injury ◽  
Flow Dynamics ◽  
Funding Source ◽  
4D Flow ◽  
4D Flow Cmr ◽  
Thoracic Aortic Injury

Abstract Background Thoracic endovascular aortic repair (TEVAR) is widely used for the treatment of blunt traumatic thoracic aortic injuries. Aortic flow dynamics and mechanical implications of this intervention are poorly investigated and may be of particular interest in the long-term follow-up of these mostly young patients. Purpose To assess whether the presence of TEVAR in a cohort of otherwise healthy subjects was related to dilation of the proximal aorta or increase in aortic stiffness and flow alterations. Methods Nineteen patients who underwent TEVAR implantation after a traumatic injury of the thoracic descending aorta (DAo) (10.0±6.1 years from intervention) and 44 healthy volunteers (HV) underwent 4D flow CMR to compute ascending aorta (AAo) pulse wave velocity (PWV), a marker of aortic stiffness, systolic flow reversal ratio (SFRR), quantifying backward flow during systole and in-plane rotational flow (IRF), measuring in-plane strength of helical flow. IRF and SFRR were assessed at 20 planes between the sinotubular junction and the mid thoracic DAo. Aortic diameters were measured using double-oblique cine CMR. Results Patients with TEVAR and HV did not differ in age, sex, body surface area, blood pressure and DAo diameter distal to TEVAR (Table). However, TEVAR patients presented larger diameters at the sinus of Valsalva and AAo, increased AAo PWV and strong flow alterations: IRF was reduced from the distal AAo to the proximal DAo, while SFRR was increased in the whole thoracic aorta (Figure). Conclusions In patients with blunt traumatic thoracic aortic injury treated with TEVAR the aorta proximal to TEVAR is dilated, stiffer and present potentially pathogenic flow conditions. Longitudinal studies are needed to assess whether these alterations have prognostic value and may improve clinical prevention and management of these patients. Figure 1. IRF and SFRR in healthy vs TEVAR Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study has been funded by Instituto de Salud Carlos III, Spanish Ministry of Science and Innovation (PI19/01480). Guala A. received funding from the Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I).

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