Myocardial strain assessment in the human fetus by cardiac MRI using Doppler ultrasound gating and feature tracking.

OBJECTIVES: Assessment of myocardial strain by feature tracking magnetic resonance imaging (FT-MRI) in human fetuses with and without congenital heart disease (CHD) using cardiac Doppler ultrasound (DUS) gating. METHODS: A total of 43 human fetuses (gestational age 28-41 weeks) underwent dynamic cardiac MRI at 3 T. Cine balanced steady-state free-precession imaging was performed using fetal cardiac DUS gating. FT-MRI was analyzed using dedicated post-processing software. Endo- and epicardial contours were manually delineated from fetal cardiac 4-chamber views, followed by automated propagation to calculate global longitudinal strain (GLS) of the left (LV) and right ventricle (RV), LV radial strain, and LV strain rate. RESULTS: Strain assessment was successful in 38/43 fetuses (88%); 23 of them had postnatally confirmed diagnosis of CHD (e.g., coarctation, transposition of great arteries) and 15 were heart healthy. Five fetuses were excluded due to reduced image quality. In fetuses with CHD compared to healthy controls, median LV GLS (- 13.2% vs. - 18.9%; p < 0.007), RV GLS (- 7.9% vs. - 16.2%; p < 0.006), and LV strain rate (1.4 s-1 vs. 1.6 s-1; p < 0.003) were significantly higher (i.e., less negative). LV radial strain was without a statistically significant difference (20.7% vs. 22.6%; p = 0.1). Bivariate discriminant analysis for LV GLS and RV GLS revealed a sensitivity of 67% and specificity of 93% to differentiate between fetuses with CHD and healthy fetuses. CONCLUSION: Myocardial strain was successfully assessed in the human fetus, performing dynamic fetal cardiac MRI with DUS gating. Our study indicates that strain parameters may allow for differentiation between fetuses with and without CHD. CLINICAL RELEVANCE STATEMENT: Myocardial strain analysis by cardiac MRI with Doppler ultrasound gating and feature tracking may provide a new diagnostic approach for evaluation of fetal cardiac function in congenital heart disease. KEY POINTS: • MRI myocardial strain analysis has not been performed in human fetuses so far. • Myocardial strain was assessed in human fetuses using cardiac MRI with Doppler ultrasound gating. • MRI myocardial strain may provide a new diagnostic approach to evaluate fetal cardiac function.

Rapid MRI Assessment of Long-Axis Strain to Indicate Systolic Dysfunction in Patients With Sickle Cell Disease.

BACKGROUND: Patients with sickle cell disease (SCD) have a unique form of cardiomyopathy. However, left ventricular ejection fraction (LVEF) is often preserved. Monoplanar long-axis strain (LAS) can be assessed from MRI four-chamber views and may be better at detecting mild systolic dysfunction in these patients. PURPOSE: To compare LAS (monoplanar and biplanar) with LVEF as a marker of systolic dysfunction in SCD patients. STUDY TYPE: Retrospective. SUBJECTS: A total of 20 patients with genetically proven SCD (35 MRI examinations), 39 healthy controls, and 124 patients with systemic iron overload (for validation purposes). FIELD STRENGTH/SEQUENCE: 1.5 T/3 T. Cine balanced steady-state free-precession. ASSESSMENT: Rapidly assessed biplanar LAS from four- and two-chamber views was correlated with age and compared to LVEF by two operators. For validation, biplanar LAS was compared to global longitudinal strain (GLS) using MRI feature-tracking in 124 patients with systemic iron overload. STATISTICAL TESTS: Bland-Altman analysis. Wilcoxon-Mann-Whitney test and Spearman-rank correlation (correlation coefficient, rS ). Receiver-operating-characteristic (ROC) curve analysis (area under the curve, AUC). Bivariate discriminant analysis. Significance level: P < 0.01. RESULTS: There was strong correlation between biplanar LAS and GLS using feature tracking (rS  = 0.73). Interoperator agreement showed nonsignificant bias for biplanar LAS (-0.02%; ±95%-agreement interval -2.2%/2.2%, P = 0.9). Biplanar LAS increased significantly with age in controls (rS  = 0.70). In SCD patients, biplanar LAS was better correlated with age than monoplanar LAS (r2  = 0.53, standard error of estimate, SEE = 1.4% vs. r2  = 0.37;SEE = 2.0%). ROC analysis of LVEF, biplanar LAS, and age-adjusted Z-scores Z (LAS(age)) showed AUCs of 0.69, 0.75, and 0.86 for differentiation between SCD patients and controls. Bivariate discriminant analysis of biplanar Z (LAS(age)) and LVEF revealed a sensitivity of 63% and a specificity of 95%. DATA CONCLUSION: Rapidly assessed biplanar LAS demonstrated high diagnostic accuracy and was an indicator of mild systolic dysfunction in patients with SCD. Biplanar LAS provided more precise measurements than monoplanar, and normalization to age increased diagnostic accuracy. EVIDENCE LEVEL: 3. TECHNICAL EFFICACY: Stage 2.

Longitudinal follow-up by MR angiography reveals progressive dilatation of the distal aorta after aortic root replacement in Marfan syndrome.

OBJECTIVES: To define and compare growth rates of the distal aorta in Marfan patients with and without aortic root replacement using serial MR angiography (MRA). METHODS: We retrospectively included 136 Marfan patients with a total of 645 MRAs who underwent a median of five MRAs (range: 2-13) at 1.5 T and 3 T in annual intervals. Of these, 41 patients (34.8 ± 12 years) had undergone aortic root replacement. The remaining 95 patients (29.0 ± 17 years) still had a native aorta and served as the control group. Thoracic aortic diameters were independently measured at eleven predefined levels. Estimated growth rates were calculated using a mixed effects model adjusted for sex, age, BMI, and medication. RESULTS: Marfan patients with aortic root replacement revealed the highest mean estimated growth rate in the proximal descending aorta (0.77 mm/year, CI: 0.31-1.21). Mean growth rates at all levels of the distal thoracic aorta were significantly higher in patients with aortic root replacement (0.28-0.77 mm/year) when compared to patients without aortic root replacement (0.03-0.07 mm/year) (all p < 0.001). Antihypertensive medication, gender, and BMI had no significant impact on the distal aortic growth rates. CONCLUSION: Distal thoracic aortic diameters increase at a significantly higher rate in Marfan patients with aortic root replacement compared to Marfan patients without aortic root replacement. Further studies are warranted to investigate if the increased growth rate of the distal thoracic aorta after aortic root replacement is caused by altered hemodynamics due to the rigid aortic root graft or due to the general genetic disposition of post-operative Marfan patients. CLINICAL RELEVANCE STATEMENT: High growth rates of the distal aorta after aortic root replacement underline the need for careful life-long aortic imaging of Marfan patients after aortic root replacement. KEY POINTS: • Aortic growth rates in Marfan patients with aortic root replacement are highest in the mid-aortic arch, the proximal- and mid-descending aorta. • Growth rates of the distal thoracic aorta are significantly higher in Marfan patients with aortic root replacement compared to Marfan patients without aortic root replacement. • Antihypertensive medication, gender, and BMI have no significant impact on distal aortic growth rates in Marfan patients.

Assessment of aortic diameter in Marfan patients: intraindividual comparison of 3D-Dixon and 2D-SSFP magnetic resonance imaging.

OBJECTIVES: To compare the accuracy and precision of 3D-Dixon and 2D-SSFP MR-imaging for assessment of aortic diameter in Marfan patients. METHODS: This prospective single-center study investigated respiratory-gated 3D-Dixon and breath-hold 2D-SSFP non-contrast MR-imaging at 3 T in 47 Marfan patients (36.0 ± 13.2 years, 28♀,19♂). Two radiologists performed individual diameter measurements at five levels of the thoracic aorta and evaluated image quality on a four-grade scale (1 = poor, 4 = excellent) and artifacts (1 = severe, 4 = none). Aortic root diameters acquired by echocardiography served as a reference standard. Intraclass correlation coefficient, Bland-Altman analyses, F-test, t-test, and regression analyses were used to assess agreement between observers and methods. RESULTS: Greatest aortic diameters were observed at the level of the sinuses of Valsalva (SOV) for 3D-Dixon (38.2 ± 6.8 mm) and 2D-SSFP (38.3 ± 7.1 mm) (p = 0.53). Intra- and interobserver correlation of diameter measurements was excellent at all aortic levels for both 3D-Dixon (r = 0.94-0.99 and r = 0.94-0.98) and 2D-SSFP (r = 0.96-1.00 and r = 0.95-0.99). 3D-Dixon-derived and 2D-SSFP-derived diameter measurements at the level of the SOV revealed a strong correlation with echocardiographic measurements (r = 0.92, p < 0.001 and r = 0.93, p < 0.001, respectively). The estimated mean image quality at the level of SOV was higher for 2D-SSFP compared to that for 3D-Dixon (3.3 (95%-CI: 3.1-3.5) vs. 2.9 (95%-CI: 2.7-3.1)) (p < 0.001). Imaging artifacts were less at all aortic levels for 3D-Dixon compared to 2D-SSFP (3.4-3.8 vs. 2.8-3.1) (all p < 0.002). CONCLUSION: Respiratory-gated 3D-Dixon and breath-hold 2D-SSFP MR-imaging provide accurate and precise aortic diameter measurements. We recommend 3D-Dixon imaging for monitoring of aortic diameter in Marfan patients due to fewer imaging artifacts and the possibility of orthogonal multiplanar reformations of the aortic root. KEY POINTS: • Respiratory-gated 3D-Dixon and breath-hold 2D-SSFP imaging provide accurate and precise aortic diameter measurements in patients suffering from Marfan syndrome. • Imaging artifacts are stronger in 2D-SFFP imaging than in 3D-Dixon imaging. • We recommend 3D-Dixon imaging for monitoring of aortic diameter in Marfan patients due to fewer imaging artifacts and the possibility of orthogonal multiplanar reformations.

Left ventricular myocardial strain responding to chronic pressure overload in patients with resistant hypertension evaluated by feature-tracking CMR.

OBJECTIVES: The study aimed to investigate the alterations of myocardial deformation responding to long-standing pressure overload and the effects of focal myocardial fibrosis using feature-tracking cardiac magnetic resonance (FT-CMR) in patients with resistant hypertension (RH). METHODS: Consecutive RH patients were prospectively recruited and underwent CMR at a single institution. FT-CMR analyses based on cine images were applied to measure left ventricular (LV) peak systolic global longitudinal (GLS), radial (GRS), and circumferential strain (GCS). Functional and morphological CMR variables, and late gadolinium enhancement (LGE) imaging were also obtained. RESULTS: A total of 50 RH patients (63 ± 12 years, 32 men) and 18 normotensive controls (57 ± 8 years, 12 men) were studied. RH patients had a higher average systolic blood pressure than controls (166 ± 21 mmHg vs. 116 ± 8 mmHg, p < 0.001) with the intake of 5 ± 1 antihypertensive drugs. RH patients showed increased LV mass index (78 ± 15 g/m2 vs. 61 ± 9 g/m2, p < 0.001), decreased GLS (- 16 ± 3% vs. - 19 ± 2%, p = 0.001) and GRS (41 ± 12% vs. 48 ± 8%, p = 0.037), and GCS was reduced by trend (- 17 ± 4% vs. - 19 ± 4%, p = 0.078). Twenty-one (42%) RH patients demonstrated a LV focal myocardial fibrosis (LGE +). LGE + RH patients had higher LV mass index (85 ± 14 g/m2 vs. 73 ± 15 g/m2, p = 0.007) and attenuated GRS (37 ± 12% vs. 44 ± 12%, p = 0.048) compared to LGE - RH patients, whereas GLS (p = 0.146) and GCS (p = 0.961) were similar. CONCLUSION: Attenuation of LV GLS and GRS, and GCS decline by tendency, might be adaptative changes responding to chronic pressure overload. There is a high incidence of focal myocardial fibrosis in RH patients, which is associated with reduced LV GRS. CLINICAL RELEVANCE STATEMENT: Feature-tracking CMR-derived myocardial strain offers insights into the influence of long-standing pressure overload and of a myocardial fibrotic process on cardiac deformation in patients with resistant hypertension. KEY POINTS: • Variations of left ventricular strain are attributable to the degree of myocardial impairment in resistant hypertensive patients. • Focal myocardial fibrosis of the left ventricle is associated with attenuated global radial strain. • Feature-tracking CMR provides additional information on the attenuation of myocardial deformation responding to long-standing high blood pressure.

Validation of 4D flow cardiovascular magnetic resonance in TIPS stent grafts using a 3D-printed flow phantom.

BACKGROUND: Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) is feasible for portal blood flow evaluation after placement of transjugular intrahepatic portosystemic shunts (TIPS) in patients with liver cirrhosis. However, clinical acceptance of 4D flow CMR in TIPS patients is limited due to the lack of validation studies. The purpose of this study was to validate 4D flow CMR-derived measurements in TIPS stent grafts using a three-dimensional (3D)-printed flow phantom. METHODS: A translucent flow phantom of the portal vasculature was 3D-printed. The phantom consisted of the superior mesenteric vein and the splenic vein draining into the portal vein, the TIPS-tract, and the hepatic vein. A TIPS stent graft (Gore® Viatorr®) was positioned within the TIPS-tract. Superior mesenteric vein and splenic vein served as inlets for blood-mimicking fluid. 4D flow CMR acquisitions were performed at 3T at preset flow rates of 0.8 to 2.8 l/min using velocity encoding of both 1.0 and 2.0 m/s. Flow rates and velocities were measured at predefined levels in the portal vasculature and within the stent graft. Accuracy of 4D flow CMR was assessed through linear regression with reference measurements obtained by flow sensors and two-dimensional (2D) phase contrast (PC) CMR. Intra- and interobserver agreement were assessed through Bland-Altman analyses. RESULTS: At a velocity encoding of 2.0 m/s, 4D flow CMR-derived flow rates and velocities showed an excellent correlation with preset flow rates and 2D PC CMR-derived flow velocities at all vascular levels and within the stent graft (all r ≥ 0.958, p ≤ 0.003). At a velocity encoding of 1.0 m/s, aliasing artifacts were present within the stent graft at flow rates ≥ 2.0 l/min. 4D flow CMR-derived measurements revealed high intra- and interobserver agreement. CONCLUSIONS: The in vitro accuracy and precision of 4D flow CMR is unaffected by the presence of TIPS stent grafts, suggesting that 4D flow CMR may be used to monitor TIPS patency in patients with liver cirrhosis.

Fetal dynamic magnetic resonance imaging using Doppler ultrasound gating for the assessment of the aortic isthmus: A feasibility study.

INTRODUCTION: Cardiovascular magnetic resonance imaging (MRI) is established in cardiac evaluation in postnatal life, but its application to the fetus has been hampered by technical limitations. We aimed to investigate the feasibility of dynamic MRI of the fetal aortic isthmus using a magnetic resonance-compatible Doppler ultrasound device for cardiac gating. MATERIAL AND METHODS: This prospective study included 19 fetuses at a median gestational age of 32.3 weeks (range 26-38 weeks). Imaging of the fetal aortic isthmus was assessed by (a) dynamic steady-state free precession MRI using a magnetic resonance-compatible Doppler ultrasound device for cardiac gating and (b) echocardiography. Diameters of the aortic isthmus were compared by two blinded observers. Magnetic resonance image quality was assessed independently by two observers using a four-point scale (1 = low quality, 4 = high quality). Furthermore, we performed four-dimensional flow MRI of the fetal aorta in three of these fetuses. RESULTS: The Doppler ultrasound device for cardiac gating allowed successful dynamic MRI examinations of the aortic isthmus in 18/19 (95%) fetuses. Evaluation of the fetal aortic isthmus was possible by both MRI (15/18, 83%) and echocardiography (16/18, 89%) (P < .05). Diameters of the aortic isthmus were concordant for MRI (3.8 ± 0.9 mm) and echocardiography (4.0 ± 1.1 mm), with a variability of 10.8% (bias -2.3%, 95% limits of agreement -23.9% to 19.3%). Overall magnetic resonance image quality was good (score 4 in 67% and score 3 in 23%) with good inter-observer agreement (κ = 0.75; 95% CI 0.5-1). Fetal four-dimensional flow MRI allowed visualization of aortic flow dynamics. CONCLUSIONS: Doppler ultrasound-gating allows dynamic MRI of the fetal aorta with the potential to serve as a complementary imaging tool in cases where echocardiography is inconclusive.

4D flow cardiovascular magnetic resonance for monitoring of aortic valve repair in bicuspid aortic valve disease.

BACKGROUND: Aortic valve repair has become a treatment option for adults with symptomatic bicuspid (BAV) or unicuspid (UAV) aortic valve insufficiency. Our aim was to demonstrate the feasibility of 4D flow cardiovascular magnetic resonance (CMR) to assess the impact of aortic valve repair on changes in blood flow dynamics in patients with symptomatic BAV or UAV. METHODS: Twenty patients with adult congenital heart disease (median 35 years, range 18-64; 16 male) and symptomatic aortic valve regurgitation (15 BAV, 5 UAV) were prospectively studied. All patients underwent 4D flow CMR before and after aortic valve repair. Aortic valve regurgitant fraction and systolic peak velocity were estimated. The degree of helical and vortical flow was evaluated according to a 3-point scale. Relative flow displacement and wall shear stress (WSS) were quantified at predefined levels in the thoracic aorta. RESULTS: All patients underwent successful aortic valve repair with a significant reduction of aortic valve regurgitation (16.7 ± 9.8% to 6.4 ± 4.4%, p < 0.001) and systolic peak velocity (2.3 ± 0.9 to 1.9 ± 0.4 m/s, p = 0.014). Both helical flow (1.6 ± 0.6 vs. 0.9 ± 0.5, p < 0.001) and vortical flow (1.2 ± 0.8 vs. 0.5 ± 0.6, p = 0.002) as well as both flow displacement (0.3 ± 0.1 vs. 0.25 ± 0.1, p = 0.031) and WSS (0.8 ± 0.2 N/m2 vs. 0.5 ± 0.2 N/m2, p < 0.001) in the ascending aorta were significantly reduced after aortic valve repair. CONCLUSIONS: 4D flow CMR allows assessment of the impact of aortic valve repair on changes in blood flow dynamics in patients with bicuspid aortic valve disease.

Dynamic fetal cardiovascular magnetic resonance imaging using Doppler ultrasound gating.

BACKGROUND: Fetal cardiovascular magnetic resonance (CMR) imaging may provide a valuable adjunct to fetal echocardiography in the evaluation of congenital cardiovascular pathologies. However, dynamic fetal CMR is difficult due to the lack of direct in-utero cardiac gating. The aim of this study was to investigate the effectiveness of a newly developed Doppler ultrasound (DUS) device in humans for fetal CMR gating. METHODS: Fifteen fetuses (gestational age 30-39 weeks) were examined using 1.5 T CMR scanners at three different imaging sites. A newly developed CMR-compatible DUS device was used to generate gating signals from fetal cardiac motion. Gated dynamic balanced steady-state free precession images were acquired in 4-chamber and short-axis cardiac views. Gating signals during data acquisition were analyzed with respect to trigger variability and sensitivity. Image quality was assessed by measuring endocardial blurring (EB) and by image evaluation using a 4-point scale. Left ventricular (LV) volumetry was performed using the single-plane ellipsoid model. RESULTS: Gating signals from the fetal heart were detected with a variability of 26 ± 22 ms and a sensitivity of trigger detection of 96 ± 4%. EB was 2.9 ± 0.6 pixels (4-chamber) and 2.5 ± 0.1 pixels (short axis). Image quality scores were 3.6 ± 0.6 (overall), 3.4 ± 0.7 (mitral valve), 3.4 ± 0.7 (foramen ovale), 3.6 ± 0.7 (atrial septum), 3.7 ± 0.5 (papillary muscles), 3.8 ± 0.4 (differentiation myocardium/lumen), 3.7 ± 0.5 (differentiation myocardium/lung), and 3.9 ± 0.4 (systolic myocardial thickening). Inter-observer agreement for the scores was moderate to very good (kappa 0.57-0.84) for all structures. LV volumetry revealed mean values of 2.8 ± 1.2 ml (end-diastolic volume), 0.9 ± 0.4 ml (end systolic volume), 1.9 ± 0.8 ml (stroke volume), and 69.1 ± 8.4% (ejection fraction). CONCLUSION: High-quality dynamic fetal CMR was successfully performed using a newly developed DUS device for direct fetal cardiac gating. This technique has the potential to improve the utility of fetal CMR in the evaluation of congenital pathologies.

MRI-based quantitative susceptibility mapping (QSM) and R2* mapping of liver iron overload: Comparison with SQUID-based biomagnetic liver susceptometry.

PURPOSE: We aimed to determine the agreement between quantitative susceptibility mapping (QSM)-based biomagnetic liver susceptometry (BLS) and confounder-corrected R2* mapping with superconducting quantum interference device (SQUID)-based biomagnetic liver susceptometry in patients with liver iron overload. METHODS: Data were acquired from two healthy controls and 22 patients undergoing MRI and SQUID-BLS as part of routine monitoring for iron overload. Magnetic resonance imaging was performed on a 3T system using a three-dimensional multi-echo gradient-echo acquisition. Both magnetic susceptibility and R2* of the liver were estimated from this acquisition. Linear regression was used to compare estimates of QSM-BLS and R2* to SQUID-BLS. RESULTS: Both QSM-BLS and confounder-corrected R2* were sensitive to the presence of iron in the liver. Linear regression between QSM-BLS and SQUID-BLS demonstrated the following relationship: QSM-BLS = (-0.22 ± 0.11) + (0.49 ± 0.05) · SQUID-BLS with r2 = 0.88. The coefficient of determination between liver R2* and SQUID-BLS was also r2 = 0.88. CONCLUSION: We determined a strong correlation between both QSM-BLS and confounder-corrected R2* to SQUID-BLS. This study demonstrates the feasibility of QSM-BLS and confounder-corrected R2* for assessing liver iron overload, particularly when SQUID systems are not accessible. Magn Reson Med 78:264-270, 2017. © 2016 International Society for Magnetic Resonance in Medicine.

Left atrial active contractile function parameters assessed by cardiac MR are sensitive to myocardial iron.

PURPOSE: To determine the impact of myocardial iron overload on left atrial (LA) volume and function using MR in patients with systemic iron overload. MATERIALS AND METHODS: Thirty-eight patients with systemic iron overload disease and 10 controls underwent 1.5 Tesla MR performing steady state free precession short-axis cine-series of the LA. Three-dimensional-volumetry was assessed to calculate LA volumes and function. Parameters were indexed (i) to body surface area. The myocardial transverse relaxation rate R2* was determined in the ventricular septum using a multi-echo GRE sequence (breathhold; electrocardiography triggered; 12 echoes; echo time = 1.3-25.7 ms). RESULTS: Significantly decreased active atrial emptying fraction (AAEF) (23% [95%-range, 7-34] versus 36% [95%-range, 14-49], P = 0.009), active atrial emptying volume (AAEVi) (5.5 mL/m2 [95%-range, 2-11] versus 11.9 mL/m2 [95%-range, 3-23], P = 0.008), and active peak emptying rate (APERi) (46 mL/s/m2 [95%-range, 29-69] versus 75 mL/s/m2 [95%-range, 45-178], P < 0.001) were found for patients with myocardial iron overload (R2* > 40 s-1 ) compared with patients with normal myocardial iron levels (R2* < 40 s-1 ). Receiver operating characteristics (ROC) analysis revealed higher potential to indicate myocardial iron overload for the AAEF (area under the ROC curve [AUC] = 0.84; P < 0.0001), APERi (AUC = 0.87; P < 0.0001), and AAEVi (AUC = 0.80; P < 0.0001) compared with LA ejection fraction (LAEF) (AUC = 0.68; P = 0.02) with equal sensitivities and specificities of 82% (AAEF), 79% (APERi), 73% (AAEVi), and 57% (LAEF). CONCLUSION: MR parameters of active LA contractile function were associated with myocardial iron overload. This cross-sectional study suggests impaired active LA contractile function to be sensitive to myocardial iron toxicity. LEVEL OF EVIDENCE: 3 J. Magn. Reson. Imaging 2017;45:535-541.

Diffusion-weighted imaging of the kidneys in haemolytic uraemic syndrome.

OBJECTIVES: To evaluate the kidneys of patients with haemolytic uraemic syndrome (HUS) using diffusion-weighted imaging (DWI) and Doppler ultrasound (US) compared with healthy controls. MATERIALS AND METHODS: Fifteen patients (mean age 33.3 years; three male; 12 female) with diarrhoea-positive HUS and 15 healthy volunteers were prospectively evaluated with DWI and Doppler US. A total apparent diffusion coefficient (ADCTOT), and ADCs predominantly reflecting microperfusion (ADCLOW) and diffusion (ADCHIGH) were calculated. Doppler US evaluated renal vascularity and flow. RESULTS: When compared with controls, kidneys affected by HUS showed reduced cortical ADC values (ADCTOT 1.79±0.22 vs. 2.04±0.1x10-3 mm2/s, P 0.001), resulting in either low corticomedullary differences (11/15 patients) or an inverted corticomedullary pattern (4/15 patients). Reduction of cortical ADC values was associated with a decrease of cortical vascularity on Doppler US (ADCTOT, P<0.001; ADCLOW, P 0.047). Kidneys with complete absence of the cortical vasculature on Doppler US (four patients) also demonstrated limited diffusion (ADCHIGH, P 0.002). Low glomerular filtration rate, requirement for haemodialysis during hospitalization, and longer duration of haemodialysis were associated with decreased cortical diffusivity (ADCTOT: P 0.04, 0.007, and <0.001, respectively). CONCLUSION: DWI shows qualitative and quantitative abnormalities in kidneys affected by HUS, thereby extending the non-invasive assessment of renal parenchymal damage. KEY POINTS: • In HUS, DWI is feasible for functional characterization of kidney involvement. • Kidneys affected by HUS showed reduced cortical diffusivity. • Decreased cortical diffusivity was associated with lower kidney function. • Requirement and duration of haemodialysis was linked to degree of cortical alterations.

Pancreatic iron and fat assessment by MRI-R2* in patients with iron overload diseases.

BACKGROUND: To determine the pancreatic iron (R2*) and fat content (FC) in comparison to hepatic and cardiac R2* in patients with iron overload disorders like ß-thalassemia major (TM), Diamond-Blackfan anemia (DBA) or hereditary hemochromatosis. METHODS: R2* rates were assessed in the liver, heart and pancreas of 42 patients with TM, 29 subjects with other iron overload diseases, and 10 controls using an ECG-gated breathhold sequence (12 echo time [TE] = 1.3-25.7 ms, readout repetition time [TR] = 244 ms). Pancreatic R2* and FC were assessed from TE dependent region of interest based signal intensities performing water-fat chemical shift relaxometry and were compared with laboratory parameters (glucose, HbA1c, amylase and lipase). RESULTS: A pancreatic iron gradient from tail (R2* = 122 s(-1) ) to head (R2* = 114 s(-1) , P < 10(-4) ) was found. The close association between cardiac and pancreatic R2* was also confirmed in patients with TM and other iron overload diseases (rs = 0.64, P < 10(-4) ). Receiver operator characteristic analysis (area: 0.89, P < 10(-4) ) identified patients with elevated cardiac iron at a pancreatic R2* cut-off level of 131s(-1) (sensitivity = specificity at 81%). Highest pancreatic R2* (211s(-1) ) and FC (36%) were found in the tail region of diabetic patients with TM. CONCLUSION: Pancreatic tail showed highest R2* rates and fat contents, especially in patients with thalassemia. Besides iron accumulation fatty degeneration might be an additional risk factor for the development of diabetes in ß-thalassemia major, but this hypothesis needs further studies in prediabetic patients.

BOLD MRI in the brain of fetal sheep at 3T during experimental hypoxia.

PURPOSE: To calculate regional fetal brain oxygen saturation (sO2) during hypoxia in sheep. MATERIALS AND METHODS: Eight pregnant ewes were examined at a 3T MR-scanner using blood oxygenation level-dependent magnetic resonance imaging (BOLD-MRI) to measure signal intensity changes of the fetal brain during a control period and a period of induced hypoxia. Regions of interest were placed in the fetal cerebrum to assess ΔR2* from GRE signal intensity plateaus (S(control), S(hypoxia)) and the relation between ΔR2* and ΔpO2 was analyzed. A probe was placed surgically in the fetal brain to directly measure local pO2 as a reference standard. Baseline and hypoxic pO2 values were recorded and compared (ΔpO2). RESULTS: Mean fetal brain pO2 decreased from 14.3 mmHg (95% confidence interval [CI]: 10-19) to 3.4 mmHg (95% CI: 2-5) during hypoxia (mean ΔpO2 = 10.9 mmHg and ΔR2* = -5s(-1)). A significant correlation between ΔR2* and ΔpO2 was noted (r = 0.93, P < 0.001), and conversion of pO2 into sO2 resulted in a linear regression coefficient of (-0.14 ± 0.01)s(-1)/% (r(2) = 0.91). CONCLUSION: Measured fetal brain BOLD-MRI was compared and converted to pO2, followed by calculation of cerebral sO2.

Noncontrast myocardial T1 mapping using cardiovascular magnetic resonance for iron overload.

PURPOSE: To explore the use and reproducibility of magnetic resonance-derived myocardial T1 mapping in patients with iron overload. MATERIALS AND METHODS: The research received ethics committee approval and all patients provided written informed consent. This was a prospective study of 88 patients and 67 healthy volunteers. Thirty-five patients underwent repeat scanning for reproducibility. T1 mapping used the shortened modified Look-Locker inversion recovery sequence (ShMOLLI) with a second, confirmatory MOLLI sequence in the reproducibility group. T2 * was performed using a commercially available sequence. The analysis of the T2 * interstudy reproducibility data was performed by two different research groups using two different methods. RESULTS: Myocardial T1 was lower in patients than healthy volunteers (836 ± 138 msec vs. 968 ± 32 msec, P < 0.0001). Myocardial T1 correlated with T2 * (R = 0.79, P < 0.0001). No patient with low T2 * had normal T1 , but 32% (n = 28) of cases characterized by a normal T2 * had low myocardial T1 . Interstudy reproducibility of either T1 sequence was significantly better than T2 *, with the results suggesting that the use of T1 in clinical trials could decrease potential sample sizes by 7-fold. CONCLUSION: Myocardial T1 mapping is an alternative method for cardiac iron quantification. T1 mapping shows the potential for improved detection of mild iron loading. The superior reproducibility of T1 has potential implications for clinical trial design and therapeutic monitoring.

Fetal blood flow velocimetry by phase-contrast MRI using a new triggering method and comparison with Doppler ultrasound in a sheep model: a pilot study.

OBJECT: We present the first study demonstrating the feasibility of antenatal blood flow velocimetry performing ECG triggered phase-contrast (PC)-MRI in the fetal aorta by using a newly developed Doppler ultrasound trigger. MATERIALS AND METHODS: Five pregnant sheep carrying singleton fetuses (gestational age 121 days) were anesthetized to undergo fetal 2D PC-MRI in the fetal descending aorta (1.5 T) using a newly developed MR-compatible Doppler ultrasound trigger for fetal cardiac triggering. Inter-operator variability was assessed for PC-MR measurements and reproducibility was tested by repeated scans in one fetus. Inter-modality comparison was performed by Doppler ultrasound velocimetry. RESULTS: Fetal cardiac triggering was possible in all examinations. PC-MR velocimetry revealed a mean inter-operator variability of 3 ± 5%. Average peak systolic flow velocities of 62.5 ± 4.4 cm/s were in good agreement with Doppler ultrasound measurements of 62.0 ± 9.2 cm/s (p (Lord's U test) ≫ 0.05). CONCLUSION: Fetal PC-MR velocimetry was successfully performed using the newly developed MR-compatible Doppler ultrasound trigger for intrauterine fetal cardiac triggering, demonstrating high inter-operator and inter-modality agreement. This new method has the high potential for alternative assessment of hemodynamic decompensation of the fetal circulation.

Diffusion-weighted imaging of the healthy pancreas: ADC values are age and gender dependent.

PURPOSE: To investigate the healthy pancreas with diffusion-weighted imaging (DWI) for characterization of age and gender-related differences in apparent diffusion coefficient (ADC) values. MATERIALS AND METHODS: Sixty six volunteers were prospectively enrolled (33 male, 33 female; range 1.4 to 83.7 years of age) and echo-planar DWI of the pancreas was performed. ADC values were measured in the pancreas head, body, and tail using a pixel-by-pixel approach. Effects of age and gender on ADC values were analyzed using a two-factorial multivariate analysis of variance (MANOVA). RESULTS: ADC values correlated inversely with the age of the volunteers. The mean global pancreatic ADC values (× 10(-3) mm(2)/s) in the age groups 0-20 years, 21-40 years, and > 40 years were 1.18 ± 0.19, 1.07 ± 0.13, and 0.99 ± 0.18, respectively. Female individuals had higher mean global ADC values than male (1.13 ± 0.14 versus 1.02 ± 0.18 × 10(-3) mm(2)/s). MANOVA showed significant effects of age (P value 0.022, eta(2) = 0.13) and gender (P value 0.001, eta(2) = 0.28) on ADC values. CONCLUSION: Pancreatic ADC values decline with ageing and show significant gender differences with higher mean values in females. The awareness of baseline values adjusted to age and gender will be important for correct interpretation of individual cases and design of future studies.

Pre-interventional assessment of right renal to right adrenal vein distance: Impact on procedure time and radiation dose in adrenal vein sampling.

PURPOSE: Adrenal vein sampling (AVS) is the reference standard for evaluation of lateralized hormone production in primary aldosteronism. We aimed to investigate the impact of pre-interventional right renal vein (RRV) to right adrenal vein (RAV) distance measurement on fluoroscopy time, contrast agent exposure and radiation dose during AVS. MATERIALS AND METHODS: Forty-five patients with primary aldosteronism undergoing AVS were enrolled in our retrospective study and divided into three groups. In the group "ruler" (n = 14), RRV-RAV-distances were determined pre-interventionally by cross-sectional imaging (CT/MRI) and AVS was performed by one interventional radiologist with limited experience in AVS. CT/MRI-derived and fluoroscopy-derived RRV-RAV-distances were correlated for aimed cannulation of the RAV. Patients in group "no ruler" (n = 24, three interventional radiologists with limited experience in AVS) and in group "expert", (n = 7, one expert interventional radiologist) underwent AVS without pre-interventional estimation of RRV-RAV-distances. Procedure parameters (fluoroscopy time, contrast agent volume, radiation dose) of group "ruler" were compared to both other groups by Kruskal-Wallis rank-sum test. RESULTS: Correlation of CT/MRI-derived and fluoroscopy-derived RRV-RAV-distances was good (r = 0.74;p = 0.003). The median RRV-RAV-distance was 4.5cm at CT/MRI (95%-CI:4.2-5.0cm) and 4.0cm at fluoroscopy (95%-CI:3.8-4.5cm). Fluoroscopy time (p<0.0001), contrast agent exposure (p = 0.0003) and radiation dose (air kerma and dose area product both p = 0.038) were significantly lower in group "ruler" compared to group "no ruler" (all p<0.05), and similar to group "expert" (all p>0.05). CONCLUSIONS: CT/MRI-derived pre-interventional renal-adrenal vein distance measurements correlate well with angiographic distance measurements. Pre-interventional estimation of the RRV-RAV-distance allows for aimed cannulation of the RAV with potential reduction of fluoroscopy time, contrast agent exposure and radiation-dose during AVS.

Anterior Pituitary Volume in Patients with Transfusion Dependent Anemias: Volumetric Approaches and Relation to Pituitary MRI­R2.

PURPOSE: Anterior pituitary iron overload and volume shrinkage is common in patients with transfusion-dependent anemia and associated with growth retardation and hypogonadotropic hypogonadism. We investigated the accuracy of different MRI-based pituitary volumetric approaches and the relationship between pituitary volume and MRI-R2, particularly with respect to growth and hypogonadism. METHODS: In 43 patients with transfusion-dependent anemia (12-38 years) and 32 healthy controls (12-72 years), anterior pituitary volume was measured by a sagittal T1 GRE 3D sequence at 1.5T and analyzed by 3D semi-automated threshold volumetry (3D-volumetry). This reference method was compared with planimetric 2D-volumetry, approximate volume calculations, and pituitary height. Using a multiple SE sequence, pituitary iron as MRI-R2 was assessed by fitting proton signal intensities to echo times. Growth and hypogonadism were obtained from height percentile tables and patients' medical charts. From body surface area and age adjusted anterior pituitary volumes of controls, Z­scores were calculated for all subjects. Separation of controls and patients with respect to Z and pituitary R2 was performed by bivariate linear discriminant analysis. RESULTS: Tuned 2D volumes showed highest agreement with reference 3D-volumes (bias -4.8%; 95% CI:-8.8%|-0.7%). A linear discriminant equation of Z = -17.8 + 1.45 · R2 revealed optimum threshold sensitivity and specificity of 65% and 100% for discrimination of patients from controls, respectively. Of correctly classified patients 71% and 75% showed hypogonadism and growth retardation, respectively. CONCLUSION: Accurate assessment of anterior pituitary size requires 3D or precise 2D volumetry, with shorter analysis time for the latter. Anterior pituitary volume Z­scores and R2 allow for the identification of patients at risk of pituitary dysfunction.