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

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

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P3-290: Reduced Middle Cerebral Artery Blood Flow is Associated with Decrements in Executive Functioning, Attention, and Verbal Learning and Memory in Aging Individuals with Heart Failure: a 4D Flow Mri Study

Alzheimer s & Dementia ◽

10.1016/j.jalz.2016.06.1954 ◽

2016 ◽

Vol 12 ◽

pp. P950-P952 ◽

Cited By ~ 3

Author(s):

Kelsey E. Melah ◽

Brianna Jankowski ◽

Chooza Moon ◽

Leonardo A. Rivera ◽

Oliver Wieben ◽

...

Keyword(s):

Heart Failure ◽

Blood Flow ◽

Executive Functioning ◽

Middle Cerebral Artery ◽

Verbal Learning ◽

Artery Blood Flow ◽

4D Flow Mri ◽

4D Flow ◽

Flow Mri ◽

Mri Study

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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

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeab090.078 ◽

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.

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