The purpose of this study was to investigate 3D flow patterns and vessel wall parameters in patients with dilated ascending aorta, age-matched subjects, and healthy volunteers.
Thoracic time-resolved ...3D phase contrast CMR with 3-directional velocity encoding was applied to 33 patients with dilated ascending aorta (diameter≥40 mm, age=60±16 years), 15 age-matched normal controls (diameter≤37 mm, age=68±7.5 years) and 15 young healthy volunteers (diameter≤30 mm, age=23±2 years). 3D blood flow was visualized and flow patterns were graded regarding presence of supra-physiologic-helix and vortex flow using a semi-quantitative 3-point grading scale. Blood flow velocities, regional wall shear stress (WSS), and oscillatory shear index (OSI) were quantified.
Incidence and strength of supra-physiologic-helix and vortex flow in the ascending aorta (AAo) was significantly higher in patients with dilated AAo (16/33 and 31/33, grade 0.9±1.0 and 1.5±0.6) than in controls (2/15 and 7/15, grade 0.2±0.6 and 0.6±0.7, P<.05) or healthy volunteers (1/15 and 0/15, grade 0.1±0.3 P<.05). Greater strength of the ascending aortic helix and vortex flow were associated with significant differences in AAo diameters (P<.05). Peak systolic WSS in the ascending aorta and aortic arch was significantly lower in patients with dilated AAo (P<.0157-.0488). AAo diameter positively correlated to time to peak systolic velocities (r=0.30-0.53, P<.04), OSI (r=0.33-0.49, P<0.02) and inversely correlated to peak systolic WSS (r=0.32-0.40, P<.03). Peak systolic WSS was significantly lower in AAo aneurysms at the right and outer curvature within the AAo and proximal arch (P<.01-.05).
Increase in AAo diameter is significantly correlated with the presence and strength of supra-physiologic-helix and vortex formation in the AAo, as well with decrease in systolic WSS and increase in OSI.
Objectives
To comprehensively and quantitatively analyse flow and vorticity in the right heart of patients after repair of tetralogy of Fallot (rTOF) compared with healthy volunteers.
Methods
...Time-resolved flow-sensitive 4D MRI was acquired in 24 rTOF patients and 12 volunteers. Qualitative flow evaluation was based on consensus reading of two observers. Quantitative analysis included segmentation of the right atrium (RA) and ventricle (RV) in a four-chamber view to extract volumes and regional haemodynamic information for computation of regional mean and peak vorticity.
Results
Right heart intra-atrial, intraventricular and outflow tract flow patterns differed considerably between rTOF patients and volunteers. Peak RA and mean RV vorticity was significantly higher in patients (
p
= 0.02/0.05). Significant negative correlations were found between patients’ maximum and mean RV and RA vorticity and ventricular volumes (
p
< 0.05). The main pulmonary artery (MPA) regurgitant flow was associated with higher RA and RV vorticity, which was significant for RA maximum and RV mean vorticity (
p
= 0.01/0.03).
Conclusion
The calculation of vorticity based on 4D flow data is an alternative approach to assess intracardiac flow changes in rTOF patients compared with qualitative flow visualization. Alterations in intracardiac vorticity could be relevant with regard to the development of RV dilation and impaired function.
Key points
• 4D flow MRI with vorticity calculation enables a novel approach to assess intracardiac flow.
• Significantly higher intracardiac vorticity occurred in patients after repair of tetralogy of Fallot.
• Regurgitant flow in the main pulmonary artery is associated with higher right heart vorticity.
To assess the diagnostic accuracy of contrast material-enhanced magnetic resonance (MR) imaging of superficial cranial arteries in the initial diagnosis of giant cell arteritis ( GCA giant cell ...arteritis ).
Following institutional review board approval and informed consent, 185 patients suspected of having GCA giant cell arteritis were included in a prospective three-university medical center trial. GCA giant cell arteritis was diagnosed or excluded clinically in all patients (reference standard final clinical diagnosis). In 53.0% of patients (98 of 185), temporal artery biopsy ( TAB temporal artery biopsy ) was performed (diagnostic standard TAB temporal artery biopsy ). Two observers independently evaluated contrast-enhanced T1-weighted MR images of superficial cranial arteries by using a four-point scale. Diagnostic accuracy, involvement pattern, and systemic corticosteroid ( sCS systemic corticosteroid ) therapy effects were assessed in comparison with the reference standard (total study cohort) and separately in comparison with the diagnostic standard TAB temporal artery biopsy ( TAB temporal artery biopsy subcohort). Statistical analysis included diagnostic accuracy parameters, interobserver agreement, and receiver operating characteristic analysis.
Sensitivity of MR imaging was 78.4% and specificity was 90.4% for the total study cohort, and sensitivity was 88.7% and specificity was 75.0% for the TAB temporal artery biopsy subcohort (first observer). Diagnostic accuracy was comparable for both observers, with good interobserver agreement ( TAB temporal artery biopsy subcohort, κ = 0.718; total study cohort, κ = 0.676). MR imaging scores were significantly higher in patients with GCA giant cell arteritis -positive results than in patients with GCA giant cell arteritis -negative results ( TAB temporal artery biopsy subcohort and total study cohort, P < .001). Diagnostic accuracy of MR imaging was high in patients without and with sCS systemic corticosteroid therapy for 5 days or fewer (area under the curve, ≥0.9) and was decreased in patients receiving sCS systemic corticosteroid therapy for 6-14 days. In 56.5% of patients with TAB temporal artery biopsy -positive results (35 of 62), MR imaging displayed symmetrical and simultaneous inflammation of arterial segments.
MR imaging of superficial cranial arteries is accurate in the initial diagnosis of GCA giant cell arteritis . Sensitivity probably decreases after more than 5 days of sCS systemic corticosteroid therapy; thus, imaging should not be delayed. Clinical trial registration no. DRKS00000594 .
Abstract
Background
There has been a shift in recent years to using ultrasound (US) and magnetic resonance imaging (MRI) as first-line investigations for suspected cranial large vessel vasculitis ...(LVV) and is a new recommendation by the EULAR 2018 guidelines for imaging in LVV. This cross-sectional study compares the performance of US and MRI and contrast-enhanced magnetic resonance angiography (MRA) for detecting vasculitis in patients with giant cell arteritis (GCA).
Methods
Patients with new-onset or already diagnosed GCA were recruited. The common temporal arteries and supra-aortic large vessels were evaluated by US and MRI/MRA. Blinded experts read the images and applied a dichotomous score (vasculitis: yes/no) in each vessel.
Results
Thirty-seven patients with giant cell arteritis (GCA) were recruited. Two patients were excluded. Of the remaining patients, nine had new-onset disease and 26 had established disease. Mean age was 71 years, and median C-reactive protein (CRP) was 7.5 mg/L. The median time between US and MRI was 1 day. Overall, US revealed vasculitic changes more frequently than MRI (
p
< 0.001). US detected vascular changes in 37% of vessels compared to 21% with MRI. Among patients with chronic disease, US detected vascular changes in 23% of vessels compared to 7% with MRI in (
p
< 0.001). The same was true for patients with new-onset disease. US detected vasculitic changes in 22% of vessels and MRI detected disease in 6% (
p
= 0.0004). Compared to contrast-enhanced MRA, US was more sensitive in detecting vasculitic changes in the large arteries, including the axillary, carotid, and subclavian arteries.
Conclusion
US more frequently detects vasculitic changes in the large arteries compared to contrast-enhanced MRA. When evaluating the cranial vessels, US performs similarly to MRI. This data supports the recommendation that US be considered as a first-line evaluation in patients suspected to have GCA.
Purpose
Lung magnetic resonance imaging (MRI) using conventional sequences is limited due to strong signal loss by susceptibility effects of aerated lung. Our aim is to assess lung signal intensity ...in children on ultrashort echo-time (UTE) and zero echo-time (ZTE) sequences. We hypothesize that lung signal intensity can be correlated to lung physical density.
Materials and methods
Lung MRI was performed in 17 children with morphologically normal lungs (median age: 4.7 years, range 15 days to 17 years). Both lungs were manually segmented in UTE and ZTE images and the average signal intensities were extracted. Lung-to-background signal ratios (LBR) were compared for both sequences and between both patient groups using non-parametric tests and correlation analysis. Anatomical region-of-interest (ROI) analysis was performed for the normal cohort for assessment of the anteroposterior lung gradient.
Results
There was no significant difference between LBR of normal lungs using UTE and ZTE (
p
< 0.05). Both sequences revealed a LBR age-dependency with a high negative correlation for UTE (R
s
= – 0.77; range 2.98–1.41) and ZTE (
R
s
= – 0.82; range 2.66–1.38)). Signal-to-noise (SNR) and contrast-to-noise ratios (CNR) were age-dependent for both sequences. SNR was higher for children up to 2 years old with 3D UTE Cones while for the rest it was higher with 4D ZTE. CNR was similar for both sequences. Posterior lung areas exhibited higher signal intensity compared to anterior ones (UTE 9.4% and ZTE 12% higher), both with high correlation coefficients (
R
2
UTE
= 0.94,
R
2
ZTE
= 0.97).
Conclusion
The ZTE sequence can measure signal intensity similarly to UTE in pediatric patients. Both sequences reveal an age- and gravity-dependency of LBR.
The aim of this 4D flow cardiovascular magnetic resonance (CMR) follow-up study was to investigate longitudinal changes in aortic hemodynamics in adolescent patients with Marfan syndrome (MFS).
4D ...flow CMR for the assessment of in-vivo 3D blood flow with full coverage of the thoracic aorta was performed twice (baseline scan t1/follow-up scan t2) in 19 adolescent MFS patients (age at t1: 12.7 ± 3.6 years, t2: 16.2 ± 4.3 years) with a mean follow-up duration of 3.5 ± 1.2 years. Ten healthy volunteers (24 ± 3.8 years) served as a control group. Data analysis included aortic blood flow visualization by color-coded 3D pathlines, and grading of flow patterns (helices/vortices) on a 3-point scale (none, moderate, severe; blinded reading, 2 observers). Regional aortic peak systolic velocities and systolic 3D wall shear stress (WSS) along the entire aortic wall were quantified. Z-Scores of the aortic root and proximal descending aorta (DAo) were assessed.
Regional systolic WSS was stable over the follow-up duration, except for a significant decrease in the proximal inner DAo segment (p = 0.02) between t1 and t2. MFS patients revealed significant lower mean systolic WSS in the proximal inner DAo compared with volunteers (0.78 ± 0.15 N/m
) at baseline t1 (0.60 ± 0.18 N/m
; p = 0.01) and follow-up t2 (0.55 ± 0.16 N/m
; p = 0.001). There were significant relationships (p < 0.01) between the segmental WSS in the proximal inner DAo, DAo Z-scores (r = -0.64) and helix/vortex pattern grading (r = -0.55) at both t1 and t2. The interobserver agreement for secondary flow patterns assessment was excellent (Cohen's k = 0.71).
MFS patients have lower segmental WSS in the inner proximal DAo segment which correlates with increased localized aberrant vortex/helix flow patterns and an enlarged diameter at one of the most critical sites for aortic dissection. General aortic hemodynamics are stable but these subtle localized DAo changes are already present at young age and tend to be more pronounced in the course of time.
Background
MRI of lung parenchyma is challenging because of the rapid decay of signal by susceptibility effects of aerated lung on routine fast spin-echo sequences.
Objective
To assess lung signal ...intensity in children on ultrashort echo-time sequences in comparison to a fast spin-echo technique.
Materials and methods
We conducted a retrospective study of lung MRI obtained in 30 patients (median age 5 years, range 2 months to 18 years) including 15 with normal lungs and 15 with cystic fibrosis. On a fast spin-echo sequence with radial readout and an ultrashort echo-time sequence, both lungs were segmented and signal intensities were extracted. We compared lung-to-background signal ratios and histogram analysis between the two patient cohorts using non-parametric tests and correlation analysis.
Results
On ultrashort echo-time the lung-to-background ratio was age-dependent, ranging from 3.15 to 1.33 with high negative correlation (R
s
= −0.86). Signal in posterior dependent portions of the lung was 18% and 11% higher than that of the anterior lung for age groups 0–2 and 2–18 years, respectively. The fast spin-echo sequence showed no variation of signal ratios by age or location, with a median of 0.99 (0.98–1.02). Histograms of ultrashort echo-time slices between controls and children with aggravated cystic fibrosis with mucus plugging and wall thickening exhibited significant discrepancies that differentiated between normal and pathological lungs.
Conclusion
Signal intensity of lung on ultrashort echo-time is higher than that on fast spin-echo sequences, is age-dependent and shows a gravity-dependent anterior to posterior gradient. This signal variation appears similar to lung density described on CT.
Regulation of cargo transport via adaptor molecules is essential for neuronal development. However, the role of PDZ scaffolding proteins as adaptors in neuronal cargo trafficking is still poorly ...understood. Here, we show by genetic deletion in mice that the multi-PDZ domain scaffolding protein glutamate receptor interacting protein 1 (GRIP1) is required for dendrite development. We identify an interaction between GRIP1 and 14-3-3 proteins that is essential for the function of GRIP1 as an adaptor protein in dendritic cargo transport. Mechanistically, 14-3-3 binds to the kinesin-1 binding region in GRIP1 in a phospho-dependent manner and detaches GRIP1 from the kinesin-1 motor protein complex thereby regulating cargo transport. A single point mutation in the Thr956 of GRIP1 in transgenic mice impairs dendritic development. Together, our results show a regulatory role for GRIP1 during microtubule-based transport and suggest a crucial function for 14-3-3 proteins in controlling kinesin-1 motor attachment during neuronal development.
•Thr956 in GRIP1 is required for dendritic patterning•14-3-3 proteins bind to phosporylatedThr956 in GRIP1•14-3-3 binding to GRIP1 controls kinesin-1 attachment•Thr956 and 14-3-3 regulate GRIP1-mediated cargo transport
Geiger et al. find that the cargo adaptor GRIP1 is required in mice for proper dendrite patterning. Their data indicate that 14-3-3 proteins act on phosphorylated GRIP1 to control docking of kinesin-1. The accessibility of GRIP1 to kinesin-based transport in turn represents an important means of regulating transport in dendrites.
Abstract
Purpose
To find the best level to measure aortic flow for quantification of aortic regurgitation (AR) in 4D flow CMR.
Methods
In 27 congenital heart disease patients with AR (67% male, 31 ± ...16 years) two blinded observers measured antegrade, retrograde, net aortic flow volumes and regurgitant fractions at 6 levels in 4D flow: (1) below the aortic valve (AV), (2) at the AV, (3) at the aortic sinus, (4) at the sinotubular junction, (5) at the level of the pulmonary arteries (PA) and (6) below the brachiocephalic trunk. 2D phase contrast (2DPC) sequences were acquired at the level of PA. All patients received prior transthoracic echocardiography (TTE) with AR severity grading according to a recommended multiparametric approach.
Results
After assigning 2DPC measurements into AR grading, agreement between TTE AR grading and 2DPC was good (
κ
= 0.88). In 4D flow, antegrade flow was similar between the six levels (
p
= 0.87). Net flow was higher at level 1–2 than at levels 3–6 (
p
< 0.05). Retrograde flow and regurgitant fraction at level 1–2 were lower compared to levels 3–6 (
p
< 0.05). Reproducibility (inter-reader agreement: ICC 0.993, 95% CI 0.986–0.99; intra-reader agreement: ICC 0.982, 95%CI 0.943–0.994) as well as measurement agreement between 4D flow and 2DPC (ICC 0.994; 95%CI 0.989 – 0.998) was best at the level of PA.
Conclusion
For estimating severity of AR in 4D flow, best reproducibility along with best agreement with 2DPC measurements can be expected at the level of PA. Measurements at AV or below AV might underestimate AR.