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Purpose: We assessed the impact of bicuspid aortic valve (BAV), aortic stenosis (AS), and regurgitation (AR) on the metrics of left ventricular (LV) remodeling, as measured by electrocardiogram (ECG), transthoracic echocardiography (TTE), and cardiac magnetic resonance (CMR). Methods: This retrospective CMR study included 11 patients with both AS and AR (BAV-ASR), 30 with AS (BAV-AS), 28 with AR (BAV-AR), 47 with neither AS nor AR (BAV-no_AS/AR), and 40 with trileaflet aortic valve (TAV-no_AS/AR). CMR analysis included the LV end-diastolic volume index (LVEDVi), mass index (LVMi), and extracellular volume fraction (ECV). The Sokolow-Lyon and Cornell products by ECG and TTE-derived E/e' were measured. Results: There were no differences in the ECG, TTE, and CMR parameters between BAV-no_AS/AR and TAV-no_AS/AR. However, the presence of aortic valve dysfunction resulted in an elevated Sokolow-Lyon product for BAV-ASR (p = 0.017) and BAV-AR (p = 0.001), as well as increased Cornell product (p = 0.04) and E/e' (p < 0.001) for BAV-AS compared with BAV-no_AS/AR. LVEDVi and LVMi were elevated in patients with BAV-ASR and BAV-AR compared with those with BAV-no_AS/AR (LVEDVi: 101 ± 29 ml/m2 and 112 ± 32 ml/m2 vs. 74 ± 15 ml/m2, p = 0.005 and p < 0.001, LVMi: 75 ± 7 g/m2 and 64 ± 14 g/m2 vs. 47 ± 9 g/m2, respectively; p < 0.001). There was no difference in ECV between the BAV and TAV-no_AS/AR subgroups. Conclusion: Normally functioning BAV did not result in LV remodeling. However, concomitant AV dysfunction was associated with statistically significant morphological remodeling. [ABSTRACT FROM AUTHOR]

Objectives: To study the pulmonary artery (PA) hemodynamics in patients with systemic sclerosis (SSc) using 4D flow MRI (4D-flow).Methods: Twenty-three patients with SSc (M/F: 2/21, 57 ± 15 years, 3 manifest PA hypertension (PAH) by right heart catheterization) and 10 control subjects (M/F: 1/9, 55 ± 17 years) underwent 4D-flow for the in vivo measurement of 3D blood flow velocities in the PA. Data analysis included area-averaged flow quantification at the main PA, 3D wall shear stress (WSS), oscillatory shear index (OSI) calculation along the PA surface, and Reynolds number. The composite outcome of all-cause death and major adverse cardiac events was also investigated.Results: The maximum PA flow at the systole did not differ, but the minimum flow at the diastole was significantly greater in patients with SSc compared with that in control subjects (7.7 ± 16.0 ml/s vs. ‑ 13.0 ± 17.3 ml/s, p < 0.01). The maximum WSS at the peak systole was significantly lower and OSI was significantly greater in patients with SSc compared with those in control subjects (maximum WSS: 1.04 ± 0.20 Pa vs. 1.33 ± 0.34 Pa, p < 0.01, OSI: 0.139 ± 0.031 vs. 0.101 ± 0.037, p < 0.01). The cumulative event-free rate for the composite event was significantly lower in patients with minimum flow in main PA ≤ 9.22 ml/s (p = 0.012) and in patients with Reynolds number ≤ 2560 (p < 0.001).Conclusions: 4D-flow has the potential to detect changes of PA hemodynamics noninvasively and predict the outcome in patients with SSc at the stage before manifest PAH.Key Points: • The WSS at the peak systolic phase was significantly lower (p < 0.05), whereas OSI was greater (p < 0.01) in patients with SSc without manifest PAH than in controls. • The hemodynamic change detected by 4D-flow may help patient management even at the stage before manifest PAH in SSc. • The minimum PA flow and Reynolds number by 4D-flow will serve as a predictive marker for SSc. [ABSTRACT FROM AUTHOR]

Bicuspid aortic valve (BAV) is associated with abnormal valve-mediated hemodynamics including high velocity jets and elevated wall shear stress (WSS). This study investigated interval changes in flow and WSS in a multi-year follow-up study. This cross-sectional study included n = 44 patients with BAV (age = 44.9 ± 12 years), n = 17 patients with tricuspid aortic valve and thoracic aortic dilatation (TAV with dilation, age = 54.6 ± 16.5 years), and n = 9 healthy controls (age = 49.3 ± 14.7 years) underwent baseline and serial aortic 4D flow MRI (follow-up duration: BAV: 2.6 ± 0.7 years, TAV with dilation: 2.7 ± 0.5 years, controls: 1.1 ± 0.5 years). Data analysis included quantification of aortic dimensions, peak systolic velocities, as well as regional 3D WSS in the ascending aorta. At baseline, BAV patients demonstrated uniformly elevated peak velocity and WSS compared to TAV with dilation and control groups (peak velocity 2.2 m/s vs. 1.6 m/s vs. 1.5 m/s, p < 0.004; WSS: 0.74 Pa vs. 0.45 Pa vs. 0.55 Pa, p < 0.001). For BAV, peak velocity increased from baseline to follow up (2.2 ± 0.8 to 2.3 ± 0.9 m/s, p < 0.001) while WSS decreased (0.74 ± 0.22 to 0.65 ± 0.21 Pa, p < 0.001). Aortic growth was minimal for both BAV (0.05 cm/year) and TAV with dilation (0.03-0.04 cm/year) patients. For BAV patients, increase of ascending aorta peak velocities indicated worsening of valve function at follow-up. Compared to TAV with dilation patients, BAV patients demonstrated a reduction in WSS which may indicate a compensatory mechanism to reduce elevated WSS forces by aortic remodeling. [ABSTRACT FROM AUTHOR]

Purpose: Late gadolinium enhancement cardiac MR (LGE-CMR) and extracellular volume fraction (ECV-CMR) are widely used to evaluate macroscopic and microscopic myocardial fibrosis. Macrocyclic contrast media are increasingly used off-label for myocardial scar assessment, given the superior safety profile of these agents. We aimed to assess the performance of two macrocyclic contrast agents, gadoterate meglumine and gadobutrol, for the evaluation of myocardial scar.Material and Methods: Forty subjects (61 ± 11 years, 67.5% men) who underwent LGE-CMR using gadobutrol were prospectively recruited for a research CMR scan using same-dose gadoterate meglumine (0.2 mmol/kg) at 1.5 T. Myocardial scar quantification was performed using a short-axis phase-sensitive inversion recovery (PSIR) Turbo-FLASH and steady-state free precession (SSFP) images. Pre- and post-contrast T1-mapping was employed to assess myocardial ECV. An intraclass correlation coefficient (ICC) was used to check for reliability between the two contrast agents.Results: Using manual thresholding on PSIR Turbo-FLASH images, mean LGE scar percentage (LGE%) was 9.9 ± 9.7% and 9.4 ± 9.7% for gadobutrol and gadoterate meglumine, respectively (p > 0.05) (ICC: 0.99, 95% CI: 0.97-0.99). Using the PSIR SSFP technique and manual thresholding, LGE% averaged 7.5 ± 9.0% and 7.1 ± 8.6% for gadobutrol and gadoterate meglumine, respectively (p > 0.05) (ICC: 0.99, 95% CI: 0.98-0.99). Average ECV with gadobutrol and gadoterate meglumine were similar at 28.40 ± 4.88 and 28.46 ± 4.73 (p > 0.05) with a strong correlation (ICC: 0.98, 95% CI: 0.94-0.99).Conclusion: We found LGE- and ECV-CMR values derived from gadoterate meglumine comparable to values derived from gadobutrol. Gadoterate meglumine has a comparable performance to gadobutrol in identifying LGE-derived myocardial scar both qualitatively and quantitatively.Key Points: • Late gadolinium-enhancement cardiac MR (LGE-MR) and extracellular volume (ECV) fraction are widely used to evaluate macroscopic and microscopic myocardial fibrosis. • Macrocyclic contrast media are increasingly used off-label for myocardial scar assessment, given the presumed superior safety profile of these agents. • LGE- and ECV-CMR values derived from gadoterate meglumine are comparable to values derived from gadobutrol. [ABSTRACT FROM AUTHOR]

The aim of the present study was to establish T1 variation (T1v) thresholds for duplicated measurements of regional T1 values in left ventricle (LV) using magnetic resonance imaging (MRI). Eighteen healthy volunteers were recruited to undergo two consecutive cardiac MRI scans using modified Look-Locker Inversion recovery (MOLLI) with two spatial resolutions on different days to repeat T1 measurements on LV. The absolute differences (d) and standard deviations (SDs) of regional T1 values were acquired with the two scans and two readers. T1v threshold (mean difference + 2SD), intra-class correlation coefficient (ICC) and coefficient of variation (CoV) were calculated. T1 mapping using the MOLLI sequence (with multiple spatial resolutions) was successfully performed in all 18 volunteers twice. On a per-slice basis, ICCs for intra-observer, inter-observer, inter-resolution and inter-study T1v were 0.988, 0.899, 0.763 and 0.6. CoVs were 0.72, 2.39, 3.90 and 4.28%. T1v thresholds were 22, 66, 118 and 120 ms. On a per-segment basis, ICCs for intra-observer, inter-observer, inter-resolution and inter-study T1v were 0.974, 0.859, 0.711 and 0.594. CoVs were 1.09, 3.36, 4.69 and 5.01%. T1v thresholds were 33, 94, 140 and 144 ms. Those thresholds may be useful for discriminating disease-initiated T1v from random errors of T1 measurements. [ABSTRACT FROM AUTHOR]

Cardiac involvement in systemic sclerosis (SSc) is considerably frequent in autopsy, but the early identification is clinically difficult. Recent advantages in cardiac magnetic resonance (CMR) enabled to detect myocardial fibrotic scar as late gadolinium enhancement (LGE). We aimed to examine the prevalence and distribution of LGE in patients with SSc, and associate them with clinical features, electrocardiographic abnormalities and cardiac function. Forty patients with SSc (58 ± 14 years-old, 35 females, limited/diffuse 25/15, disease duration 106 ± 113 months) underwent serological tests, 12-lead electrocardiogram (ECG) and CMR. Seven patients (17.5 %) showed LGE in 26 segments of left ventricle (LV). LGE distributed mainly in the basal to mid inter-ventricular septum and the right ventricular (RV) insertion points, but involved all the myocardial regions. More patients with LGE showed NYHA functional class II and more (71 vs. 21 %, p < 0.05), bundle branch blocks (57 vs. 6 %, p < 0.05), LV ejection fraction (LVEF) < 50 % (72 vs. 6 %, p < 0.01), LV asynergy (43 vs. 0 %, p < 0.01) and RVEF < 40 % (100 vs. 39 %, p < 0.01). There was no difference in disease duration, disease types, or prevalence of positive autoimmune antibodies or high serum NT-proBNP level (>125 pg/ml). When cardiac involvement of SSc was defined as low LVEF, ECG abnormalities or high NT-proBNP, the sensitivity, specificity positive and negative predictive values of LGE were 36, 92, 71 and 72 %, respectively. We could clarify the prevalence and distribution of LGE in Japanese patients with SSc. The presence of LGE was associated with cardiac symptom, conduction disturbance and impaired LV/RV contraction. [ABSTRACT FROM AUTHOR]