scholarly journals P5492Regional aortic stiffness in bicuspid aortic valve patients assessed by 4D-flow CMR: influence of aortic dilation and comparison with Marfan syndrome and degenerative aortic aneurysm

2018 ◽  
Vol 39 (suppl_1) ◽  
Author(s):  
A Guala ◽  
J Rodriguez-Palomares ◽  
G Teixido-Tura ◽  
L Dux-Santoy ◽  
A Ruiz-Munoz ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Aneurysm ◽  
Marfan Syndrome ◽  
Bicuspid Aortic Valve ◽  
Aortic Stiffness ◽  
Aortic Dilation ◽  
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).

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.

477Partial fusion of two aortic valve leaflets is related to alterations in ascending aorta flow: 4D flow CMR study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Guala ◽  
L Galian ◽  
G Teixido Tura ◽  
L Dux-Santoy ◽  
A Ruiz Munoz ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Arch ◽  
Ascending Aorta ◽  
Aortic Dilation ◽  
4D Flow ◽  
4D Flow Cmr ◽  
Flow Displacement ◽  
Aortic Valve Leaflets ◽  
Partial Fusion ◽  
Cine Cmr

Abstract Introduction Bicuspid aortic valve (BAV) is the most common congenital valve defect. It consists in the fusion of two aortic valve leaflets, and it is associated with a high prevalence of proximal aorta dilation. Dilation is highly prevalent (around 30%) in BAV patient relatives with a tricuspid valve (TAV) identified by echocardiography. However, the presence of partial aortic valve leaflet fusion (also called mini-raphe or forme fruste BAV, see figure 1A) is easily missed by echocardiography. A recent study reported that 44% of patients from a small cohort of BAV patient relatives with aortic dilation followed by CT showed mini-raphe. Purpose We aimed to use 4D flow CMR to assess if the presence of mini-raphe is associated with aortic flow alterations, which may be concurs in the etiology of aortic dilation in BAV patient relatives. Methods Twenty BAV patients first-degree relatives with partial fusion (<50%) of aortic valve leaflets and proximal aorta dilation were identified by CT or cine CMR and prospectively included. One-hundred twenty-five BAV and 95 patients with TAV from our prospective dataset of 4D flow CMR were included for comparison. Propensity score matching was used throughout the study to correct the comparisons between mini-raphe and BAV and mini-raphe and TAV patients for differences in age, maximum aortic diameter, sex, height, weight, proximal aortic pulse wave velocity and, only for BAV, fusion pattern. The hemodynamic parameters previously related to aortic dilation were computed. They were jet angle, normalized flow displacement and systolic flow reversal ratio (SFRR, identifying through-plane vortexes) were computed and compared in the ascending aorta and in the aortic arch. Results The presence of mini-raphe was statistically-significantly associated with increase in jet angle (Figure 1B), flow displacement (Figure 1C) and vortexes (Figure 1D) in most of the ascending aorta and aortic arch when mini-raphe patients were compared with TAV patients. The severity of flow asymmetry found in mini-raphe patients was lower than the one characteristic of BAV patients, but vortexes were even higher in a small region at the distal ascending aorta. Figure 1 Conclusion Partial fusion of the aortic valve leaflets is related to increase in proximal aorta flow eccentricity and vorticity. These flow abnormalities are not as marked as those associated with BAV. Data regarding prevalence of mini-raphe as evaluated with CT or cine CMR are needed, especially in familiar of BAV patients. Acknowledgement/Funding European FP7/People 267128; Spanish Ministry of Economy and Competitiveness RTC-2016-5152-1 and Instituto de Salud Carlos III PI14/0106

scholarly journals Influence of Aortic Dilation on the Regional Aortic Stiffness of Bicuspid Aortic Valve Assessed by 4-Dimensional Flow Cardiac Magnetic Resonance

2019 ◽  
Vol 12 (6) ◽  
pp. 1020-1029 ◽  
Author(s):  
Andrea Guala ◽  
Jose Rodriguez-Palomares ◽  
Lydia Dux-Santoy ◽  
Gisela Teixido-Tura ◽  
Giuliana Maldonado ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Stiffness ◽  
Aortic Dilation ◽  
Dimensional Flow

ALTERATION OF ELASTIC PROPERTIES IN CONGENITAL/INHERITED AORTIC DISEASES: MRI STUDY IN MARFAN SYNDROME AND BICUSPID AORTIC VALVE

2006 ◽  
Vol 06 (01) ◽  
pp. 47-53
Author(s):  
LUIGI LOVATO ◽  
PAOLO SBARZAGLIA ◽  
SILVIA MAGI ◽  
VINCENZO RUSSO ◽  
ROMANO ZANNOLI ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Marfan Syndrome ◽  
Elastic Properties ◽  
Bicuspid Aortic Valve ◽  
Aortic Distensibility ◽  
Control Group ◽  
Aortic Dilation ◽  
Aortic Diseases ◽  
Magnetic Resonance Imaging Mri ◽  
Mri Study

Marfan Syndrome and Bicuspid Aortic Valve (BAV) are characterized by the congenital/inherited alteration of the fibrillin proteins of aortic wall extracellular matrix, with higher incidence of aortic dissection/rupture even without aortic dilation. Therefore, early identification and treatment of aortic involvement could improve prognosis. The purpose of this study was to evaluate aortic elastic properties of BAV and Marfan Syndrome patients to identify an index of early aortic involvement. Methods: A Magnetic Resonance Imaging (MRI) study was performed on 38 BAV patients, 20 Marfan patients and 14 healthy volunteers as a control group. A high-resolution gradient-echo sequence was applied in the axial plane at the level of pulmonary artery bifurcation to evaluate aortic distensibility. Results: Aortic distensibility was significantly different among the three groups (ANOVA, p = 0.0001) and was sensibly reduced in BAV (0.0056 ± 0.0048 versus 0.025 ± 0.006 control group) and Marfan (0.0085 ± 0.006 versus 0.025 ± 0.006 control group). Intraobserver and interobserver reproducibility for diastolic measurements were 1.2% and 0.4% respectively and for systolic measurements, they were 1.1% and 0.3% respectively. Conclusion: In BAV or Marfan Syndrome patients, MRI evaluation of abnormal distensibility may provide a reliable index of early aortic involvement, even before aortic dilation occurs.

EFFECT OF AORTIC STIFFNESS PARAMETERS BY CARDIAC MAGNETIC RESONANCE ON AORTIC DILATION IN PATIENTS WITH BICUSPID AORTIC VALVE

2020 ◽  
Vol 75 (11) ◽  
pp. 1637
Author(s):  
Vasutakarn Chongthammakun ◽  
Amy Pan ◽  
Stefan Kostelyna ◽  
Benjamin Goot ◽  
Michael Earing ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Stiffness ◽  
Aortic Dilation

scholarly journals Applications of a Specialty Bicuspid Aortic Valve Program: Clinical Continuity and Translational Collaboration

2020 ◽  
Vol 9 (5) ◽  
pp. 1354
Author(s):  
Erin E. Crawford ◽  
Patrick M. McCarthy ◽  
S. Chris Malaisrie ◽  
Jyothy J. Puthumana ◽  
Joshua D. Robinson ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Biomarker Discovery ◽  
Elective Surgery ◽  
Prophylactic Surgery ◽  
Medical Patients ◽  
4D Flow ◽  
Clinical Registry ◽  
Tissue Samples ◽  
4D Flow Cmr

Bicuspid aortic valve (BAV) is a common congenital heart diagnosis and is associated with aortopathy. Current guidelines for aortic resection have been validated but are based on aortic diameter, which is insufficient to predict acute aortic events. Clinical and translational collaboration is necessary to identify biomarkers that can individualize the timing of prophylactic surgery for BAV aortopathy. We describe our multidisciplinary BAV program, including research protocols aimed at biomarker discovery and results from our longitudinal clinical registry. From 2012–2018, 887 patients enrolled in our clinical BAV registry with the option to undergo four dimensional flow cardiovascular magnetic resonance imaging (4D flow CMR) and donate serum plasma or tissue samples. Of 887 patients, 388 (44%) had an elective BAV-related procedure after initial presentation, while 499 (56%) continued with medical management. Of medical patients, 44 (9%) had elective surgery after 2.3 ± 1.4 years. Surgery patients’ biobank donations include 198 (46%) aorta, 374 (86%) aortic valve, and 314 (73%) plasma samples. The 4D flow CMR was completed for 215 (50%) surgery patients and 243 (49%) medical patients. Patients with BAV aortopathy can be safely followed by a multidisciplinary team to detect indications for surgery. Paired tissue and hemodynamic analysis holds opportunity for biomarker development in BAV aortopathy.

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