scholarly journals Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome

2018 ◽  
Vol 121 (9) ◽  
pp. 1094-1101 ◽  
Author(s):  
Elif Seda Selamet Tierney ◽  
Jami C. Levine ◽  
Lynn A. Sleeper ◽  
Mary J. Roman ◽  
Timothy J. Bradley ◽  
...  
2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
L Dux-Santoy ◽  
G Teixido-Tura ◽  
A Ruiz-Munoz ◽  
L La Mura ◽  
F Valente ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): Spanish Ministry of Science, Innovation and Universities Instituto de Salud Carlos III Background. Dilation of the aortic root is a key feature of Marfan syndrome and it is related to the occurrence of aortic events and death. On top of maximum diameter, rapid annual growth rate is suggested by guidelines for indication of aortic root replacement. Current gold-standard for aortic root diameter assessment is manual quantification on multiplanar reformatted 3D computed tomography (CT) or magnetic resonance angiogram. However, inter- and intra-observer reproducibility are limited and different measurement methods, i.e. cusp-to-cusp and cusp-to-commissure, may be used in different clinical centres, leading to difficulties in the clinical assessment of progressive dilation. Purpose. We aimed to test whether aortic root growth rate during follow-up can be reliably quantified by semi-automatic co-registration of two CT angiograms. Methods. Seven Marfan syndrome patients, free from previous aortic surgery, with a total of 11 pairs of CT were identified. Manual assessment of six aortic root diameters (right-non coronary -RN- , right-left -RL- and left-non coronary -LN- cusp-to-cusp and R, L and N cusp-to-commissure) was obtained from all CTs by an experienced researcher blind to semi-automatic results. The thoracic aorta and the outflow tract were semi-automatically segmented in the baseline CT and commissure and cusps were manually located. A 10 mm-thick region of interest containing the aortic wall was automatically generated from segmentation boundary. Co-registration was obtained with three, fully-automatic steps. Firstly, baseline and follow-up CT scans were aligned by means of a rigid registration. Then, scans were co-registered with multi-resolution affine followed by b-spline non-rigid registrations based on mutual information metric. The transformation pertaining to the location of baseline commissure and cusps points was used to locate the same points in the follow-up scan (Fig. 1 top). Results. Follow-up duration was 35 ± 22 (range 12-70.3) months. Automatic quantification of diameter growth during the follow-up was obtained in 62 out of 66 (94%) diameter comparisons. High Pearson correlation coefficients (R) and ICC were found between manual and semi-automatic assessment of growth rate, both for cusp-to-cusp and cusp-to-commissure diameters: R = 0.727 and ICC = 0.678 for RN; R = 0.822 and ICC = 0.602 for RL; R = 0.648 and ICC = 0.668 for LN; R = 0.726 and ICC = 0.711 for R; R = 0.911 and ICC = 0.895 for L and R = 0.553 and ICC = 0.482 for N. Scatter and Bland-Altman plots for all growth rates (Fig. 1) confirmed very good correlation (R = 0.810) but a slight tendency (R=-0.270) for underestimation at high growth rate.  No correlation was found between follow-up duration and difference between techniques (R = 0.06). Conclusions. Semi-automatic quantification of aortic root growth rate by co-registration of pairs of CT angiograms is feasible for follow-up as short as one year. Larger studies are needed to confirm these preliminary data. Abstract Figure. CT measurements. Automatic vs manual.


Open Heart ◽  
2019 ◽  
Vol 6 (2) ◽  
pp. e001095
Author(s):  
Froso Sophocleous ◽  
Bostjan Berlot ◽  
Maria Victoria Ordonez ◽  
Mai Baquedano ◽  
Elena Giulia Milano ◽  
...  

ObjectivesThis study aimed to identify determinants of aortic growth rate in bicuspid aortic valve (BAV) patients. We hypothesised that (1) BAV patients with repaired coarctation (CoA) exhibit decreased aortic growth rate, (2) moderate/severe re-coarctation (reCoA) results in increased growth rate, (3) patients with right non-coronary (RN) valve cusps fusion pattern exhibit increased aortic growth rate compared with right-left cusps fusion and type 0 valves.MethodsStarting from n=521 BAV patients with cardiovascular magnetic resonance data, we identified n=145 patients with at least two scans for aortic growth analysis. Indexed areas of the sinuses of Valsalva and ascending aorta (AAo) were calculated from cine images in end-systole and end-diastole. Patients were classified based on dilation phenotype, presence of CoA, aortic valve function and BAV morphotype. Comparisons between groups were performed. Linear regression was carried out to identify associations between risk factors and aortic growth rate.ResultsPatients (39±16 years of age, 68% male) had scans 3.7±1.8 years apart; 32 presented with AAo dilation, 18 with aortic root dilation and 32 were overall dilated. Patients with repaired CoA (n=61) showed decreased aortic root growth rate compared with patients without CoA (p≤0.03) regardless of sex or age. ReCoA, aortic stenosis, regurgitation and history of hypertension were not associated with growth rate. RN fusion pattern showed the highest aortic root growth rate and type 0 the smallest (0.30 vs 0.08 cm2/m*year, end-systole, p=0.03).ConclusionsPresence of CoA and cusp fusion morphotype were associated with changes in rate of root dilation in our BAV population.


2015 ◽  
Vol 31 (8) ◽  
pp. 1583-1590 ◽  
Author(s):  
Madelien V. Regeer ◽  
Philippe J. van Rosendael ◽  
Vasileios Kamperidis ◽  
Martin J. Schalij ◽  
Jeroen J. Bax ◽  
...  

Agronomy ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 2058
Author(s):  
Iván P. Ordóñez ◽  
Ignacio F. López ◽  
Peter D. Kemp ◽  
Daniel J. Donaghy ◽  
Yongmei Zhang ◽  
...  

The increase in drought events due to climate change have enhanced the relevance of species with greater tolerance or avoidance traits to water restriction periods, such as Bromus valdivianus Phil. (B. valdivianus). In southern Chile, B. valdivianus and Lolium perenne L. (L. perenne) coexist; however, the pasture defoliation criterion is based on the physiological growth and development of L. perenne. It is hypothesised that B. valdivianus needs a lower defoliation frequency than L. perenne to enhance its regrowth and energy reserves. Defoliation frequencies tested were based on B. valdivianus leaf stage 2 (LS-2), leaf stage 3 (LS-3), leaf stage 4 (LS-4) and leaf stage 5 (LS-5). The leaf stage development of Lolium perenne was monitored and contrasted with that of B. valdivianus. The study was conducted in a glasshouse and used a randomised complete block design. For Bromus valdivianus, the lamina length, photosynthetic rate, stomatal conductance, tiller number per plant, leaf area, leaf weights, root growth rate, water-soluble carbohydrates (WSCs) and starch were evaluated. Bromus valdivianus maintained six live leaves with three leaves growing simultaneously. When an individual tiller started developing its seventh leaf, senescence began for the second leaf (the first relevant leaf for photosynthesis). Plant herbage mass, the root growth rate and tiller growth were maximised at LS-4 onwards. The highest leaf elongation rate, evaluated through the slope of the lamina elongation curve of a fully expanded leaf, was verified at LS-4. The water-soluble carbohydrates (WSCs) increased at LS-5; however, no statistical differences were found in LS-4. The LS-3 and LS-2 treatments showed a detrimental effect on WSCs and regrowth. The leaf photosynthetic rate and stomatal conductance diminished while the leaf age increased. In conclusion, B. valdivianus is a ‘six-leaf’ species with leaf senescence beginning at LS-4.25. Defoliation at LS-4 and LS-5 was optimum for plant regrowth, maximising the aboveground plant parameters and total WSC accumulation. The LS-4 for B. valdivianus was equivalent to LS-3.5 for L. perenne. No differences related to tiller population in B. valdivianus were found in the different defoliation frequencies.


2016 ◽  
Vol 1 (1) ◽  
pp. 013
Author(s):  
Aprilia Ike Nurmalasari ◽  
Eka Tarwaca Susila Putra ◽  
Prapto Yudono

The research aims to study the change of morphology root characters of eight hybrid oil palms under iron toxicity (Fe). Field experiment done in arranged in a Randomized Complete Block Design (RCBD) two factors and three blocks as replications. The first factor was Fe concentration. It consists of two levels which are concentration 0µ.g-1 and concentration 600 µg.g-1 Fe. The second factor is the hybrid of oil palms which consists of eight hybrid oil palms as Yangambi, Avros, Langkat, PPKS 239, Simalungun, PPKS 718, PPKS 540 and Dumpy. Fe was applied by pouring FeSO4 solvent for 600 µg.g-1 500 ml.-1plant.-1day-1 on two months of plants after transplanting in the main nursery. Data were collected on root morphology and plant dry weight The data were analysis of variance (ANOVA) at 5% significanly, followed by Duncan's multiple range test (DMRT). The relationships by among variables were determined by correlation analysis. The results showed that Fe concentration 600 µg.g-1 inhibits relatively root growth rate, narrows surface area, reduces the diameter, and shrinks root volume of all hybrid oil palms tested. The slowing relatively root growth rate, narrowing of root surface area and root diameter also root volume shrinkage due to Fe stress. It was also shown that the dry weight of plants was inhibit by existing of Fe toxicity.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Nitya Viswanathan ◽  
Claudia Pedroza ◽  
Shaine A Morris

Background: Marfan Syndrome (MS) is associated with progressive aortic dilation and aortic dissection. Aortic dissection is typically preceded by aortic dilation. The goal of this study is to identify factors associated with faster rates of aortic root dilation in children with MS. Methods: Patients undergoing serial transthoracic echocardiograms (TTE) with MS were retrospectively identified from an institutional database. Those with >2 TTEs over 1 year apart and <21 years of age at first TTE were included. TTEs performed after aortic surgery were excluded. Using multivariable longitudinal linear regression analysis, sex, medication, presence of ectopia lentis, need for scoliosis surgery and infantile type of MS were evaluated for associations with rate of change in aortic root dimension and aortic root z-score over time. Results: Of 240 patients with MS, 146 were included. Median age at first TTE was 8.1 yrs (range 0-20.9 years), median length of follow up 6.5 years (range 1.0-20.1 years), and median number of studies was 8 (range 2-25). Sixty-one percent were male. Of the 146 patients, 123 (84%) were documented to be on medical therapy: 14 angiotensin receptor blocker (ARB), 66 B-Blocker (BB), 10 prior history of both, 5 BB+ARB, 27 were in the Pediatric Heart Network medication trial, 1 ACE inhibitor. Sixteen patients underwent root replacement surgery at a median age of 14.6 years (range 1.8-24). No patients had aortic dissection. Three patients had infantile MS. All of these patients underwent root replacement at 1.8, 2 and 4 years of age. Two underwent subsequent aortic root replacement at 6 and 8 years old. Mean rate of aortic root growth in the cohort was 0.12cm/year, and mean change in z-score was 0.02/year (p=0.23 compared to expected rate of no change). The only variable associated with faster root growth was infantile MS (1.3cm/year, p<0.001; z-score change of 6.9/year, p<0.001). There was no significant difference in the rate of aortic root growth between patients who received therapy with BB vs. ARB vs. BB+ARB. Conclusions: Children with MS did not have a significant change in aortic root z-score over time, and the only factor associated with more rapid aortic root growth was infantile type MS.


1980 ◽  
Vol 20 (4) ◽  
pp. 389-396 ◽  
Author(s):  
Christopher Cox ◽  
Henry T. Davis ◽  
Morton W. Miller ◽  
Dominique Robertson

1998 ◽  
Vol 31 ◽  
pp. 30
Author(s):  
M. Groenink ◽  
L. Rozendaal ◽  
M.S. Naoff ◽  
R.C. Hennekam ◽  
A.A. Hart ◽  
...  

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Guala ◽  
G Teixido Tura ◽  
J F Rodriguez-Palomares ◽  
A Ruiz Munoz ◽  
C Granato ◽  
...  

Abstract Background The most common cardiovascular complications in Marfan syndrome (MFS) are aortic root dilation and type A aortic dissections. Elective aortic root surgery is indicated when maximum aortic diameter is larger than a defined threshold or in the case of fast-progressing dilation. However, maximum aortic diameter is limited for the prediction of aortic events. Indeed, a large international registry of acute aortic syndromes reported that as much as 40% of aortic dissections happen with maximum aortic diameter lower than 50 mm. Consequently, there is a need for new, non-invasive biomarkers to improve the prediction of aortic complications. Purpose The aim of the present study was to assess if proximal aorta circumferential and longitudinal strain and ascending aorta distensibility were associated with progressive aortic dilation and incidence of aortic events in Marfan syndrome patients. Methods Eighty seven Marfan syndrome patients free from previous cardiac/aortic surgery or dissection, were prospectively included in a multicenter follow-up. Patients were diagnosed by original Ghent criteria. Proximal aorta longitudinal and circumferential strain and distensibility were computed from baseline cine CMR images by means of feature-tracking. The predictive capacity of each stiffness biomarkers was separately tested with multivariable linear regression analysis (aortic growth) and with Cox logistic regression analysis (aortic events), both corrected for clinical and demographic variables, including baseline maximum aortic diameter. Results During a follow-up of 81.6±17 months, mean diameter growth-rate was 0.65±0.67 mm/year and z-score growth rate was 0.07±0.13 / year. Elective aortic root replacement was performed in 11 patients while two patients presented type A aortic dissection.Baseline proximal aorta longitudinal strain was independently related to diameter growth-rate (p=0.001), z-score growth-rate (p=0.018) and aortic events (p=0.018). Conversely, neither circumferential strain nor distensibility were independent predictors of diameter growth-rate (p=0.385 and p=0.381, respectively), z-score growth-rate (p=0.515 and p=0.484, respectively) and aortic events (p=0.064 and p=0.205, respectively). Conclusions Proximal aorta longitudinal strain predicts aortic root dilation and major aortic events in Marfan syndrome patients beyond aortic root diameter and clinical and demographic characteristics. Acknowledgement/Funding ISCIII PI14/0106, La Maratό de TV3 (20151330) and CIBERCV. Guala A. FP7/People n° 267128


2019 ◽  
Vol 50 (5) ◽  
pp. 257-260
Author(s):  
N. V. Zhukovskaya ◽  
E. I. Bystrova ◽  
N. F. Lunkova ◽  
V. B. Ivanov

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