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Aims In vitro studies have shown impairment of energy metabolism in cardiac fibroblasts from Fabry patients. A recent in vivo study reported an association between cardiac energy metabolism and increased myocardial mass in Fabry patients. We therefore assessed possible disturbances of cardiac energy metabolism in Fabry patients by in vivo 31P-MR-spectroscopy. Additionally, the effect of enzyme replacement therapy (ERT) on cardiac energetics was tested. Methods and results Twenty-three patients (41 ± 9 years; 10 females) with genetically proven Fabry disease were examined with a 1.5 T Scanner, and compared with an age-matched healthy control group. Eight patients underwent ERT and had follow-up examinations after 3 and 14 months. The high-energy phosphate molecules phosphocreatine (PCr) and adenosine triphosphate (ATP) were quantified in localized 31P-spectra by SLOOP (spectral localization with optimum point spread function). Cine- and late gadolinium enhancement (LGE) studies were also performed. When compared with healthy controls, Fabry patients demonstrated reduced PCr- (6.1 ± 1.9 vs. 8.8 ± 2.6 mmol/kg; P = 0.003) and ATP concentrations (3.9 ± 1.5 vs. 4.6 ± 1.0 mmol/kg; P = 0.048). During ERT, PCr concentrations increased (7.1 ± 1.5 mmol/kg vs. 6.1 ± 1.9; P < 0.05) and left ventricular mass decreased (215 ± 55 vs. 185 ± 45 g; P = 0.012). Disturbances in cardiac energetics were not correlated to the presence or absence of cardiac fibrosis on LGE. Conclusion Cardiac energy metabolism is disturbed in Fabry disease; this may play an important role in the pathogenesis of Fabry cardiomyopathy. Enzyme replacement therapy ameliorates energetic depression.

The value of 31P-magnetic resonance spectroscopy (MRS) as a possible tool to distinguish viable from non-viable tissue after myocardial infarction was analysed in humans. Fifteen patients 3 weeks after anterior myocardial infarction were studied with breath-hold cine MRI and 3D-CSI MRS (1.5 T system). 31P-spectra were obtained from infarcted as well as non-infarcted myocardium (voxel size 25 cm3 each). Gold standard for viability was recovery of regional function, as determined by a control MRI 6 months after revascularization. Ten age-matched healthy volunteers served as control group. No significant difference was found between the phosphocreatine to adenosinetriphosphate (PCr/ ATP) ratio of volunteers (SD 1.72+/-0.31) and non-infarcted septal myocardium of patients. Cine MRI demonstrated recovery of regional function in 10 patients, i. e. 10 patients showed viable and 5 non-viable myocardium. In viable myocardium, the PCr/ATP ratio was 1.47+/-0.38 (non-significant vs volunteers; p>0.05). In the 5 patients with akinetic myocardium, PCr peaks could not be detected. Therefore, calculation of PCr/ATP ratios was not possible. However, a significant reduction of the ATP signal-to-noise ratio (SNR) was observed (2.92+/-0.73 vs 6.68+/-0.80; patients vs volunteers; p

PURPOSE: To demonstrate the feasibility of sodium 23 ((23)Na) magnetic resonance (MR) imaging for assessment of subacute and chronic myocardial infarction and compare with cine, late enhancement, and T2-weighted imaging. MATERIALS AND METHODS: Thirty patients underwent MR imaging 8 days +/- 4 (subacute, n = 15) or more than 6 months (chronic, n = 15) after myocardial infarction by using a (23)Na surface coil with a double angulated electrocardiogram-triggered three-dimensional gradient-echo sequence at 1.5 T. In addition, cine, inversion-recovery gradient-echo, and, in the subacute group, T2-weighted images (n = 9) were obtained. Myocardial infarction mass was depicted as elevated signal intensity or wall motion abnormalities and expressed as a percentage of total left ventricular mass for all modalities. Correlations were tested with correlation coefficients. RESULTS: All patients after subacute infarction and 12 of 15 patients with chronic infarction had an area of elevated (23)Na signal intensity that significantly correlated with wall motion abnormalities (subacute; r = 0.96, P

Purpose To evaluate the sodium longitudinal relaxation (T1) characteristics for myocardium and blood in humans. Materials and Methods Eleven healthy volunteers were examined by using a 23Na heart surface coil at a 1.5 T clinical scanner equipped with a broadband spectroscopy option. 23Na MR measurements were performed by using a three-dimensional spoiled gradient echo sequence (in-plane resolution, 3.5 mm × 7 mm; slice thickness, 24 mm; TE, 3.1 msec; bandwidth, 65 Hz/pixel; TR, 21 to 150 msec). Results Longitudinal T1 relaxation time components were 31.6±7.0 msec and 31.1±7.5 msec for myocardium and blood, respectively. Conclusion 23Na T1 relaxation times of myocardium and blood can be determined in humans. The results are in agreement with values obtained from animal studies. J. Magn. Reson. Imaging 2003;17:726–729. © 2003 Wiley-Liss, Inc.

(23)Na MRI may allow distinction of normal and ischemically injured myocardium. The aim of this study was to optimize (23)Na MRI of the human heart by improvement of spatial resolution and ECG-triggering and to measure the signal/noise of blood and myocardium and the myocardium/blood signal ratios in a volunteer study. A spoiled gradient echo sequence was developed on a 1.5T scanner equipped with a (23)Na heart surface coil. 3D short axis ECG-triggered (23)Na MRI was performed in 10 healthy subjects. The signal/noise of myocardium and blood were 8.2 +/- 0.7 and 18.3 +/- 1.3, respectively, signal ratio myocardium/blood was 0.44 +/- 0.03. This value is in good agreement with the theoretical value of 0.45. ECG gated 3D (23)Na MRI of the human heart is feasible with sufficient spatial resolution and signal/noise ratio. Magn Reson Med 45:164-166, 2001.

Human cardiac 31P nuclear magnetic resonance (NMR) spectra are usually quantified in relative terms, i.e., the ratio of metabolite signals is calculated. If 31P NMR spectroscopy of the heart is to emerge as a clinically relevant diagnostic modality, reliable quantification of absolute concentrations of 31P metabolites is required. We applied spectral localization with optimal point spread function (SLOOP) 31P NMR spectroscopy to measure absolute concentrations of phosphocreatine (PCr) and adenosine triphosphate (ATP) in human myocardium. The accuracy of the quantification was first validated in a phantom study. Seven healthy volunteers (aged 19-29 years) were then examined at 1.5 T using a nominal spatial resolution of 25 mL. SLOOP allowed us to obtain localized spectra from compartments anatomically matched to the left ventricular wall. The a priori knowledge of the anatomical structure was obtained from 1H images. The spatially varying effects of saturation, off-resonance, and sensitivity were considered during the reconstruction process. Metabolites were quantified with reference to an external 31P standard. Concentrations of 9.0 +/- 1.2 and 5.3 +/- 1.2 mmol/kg wet wt (mean +/- SD, n = 9) were determined for PCr and ATP in normal heart, respectively. The influence of nuclear Overhauser enhancement on metabolite quantification is discussed.

To investigate whether coronary artery calcium (CAC) scoring performed on three different workstations generates comparable and thus vendor-independent results.Institutional review board and Federal Office for Radiation Protection approval were received, as was each patient's written informed consent. Fifty-nine patients (37 men, 22 women; mean age, 57 years±3 [standard deviation]) underwent CAC scoring with use of 64-section multidetector computed tomography (CT) with retrospective electrocardiographic gating (one examination per patient). Data sets were created at 10% increments of the R-R interval from 40%-80%. Two experienced observers in consensus calculated Agatston and volume scores for all data sets by using the calcium scoring software of three different workstations. Comparative analysis of CAC scores between the workstations was performed by using regression analysis, Spearman rank correlation (rs), and the Kruskal-Wallis test.Each workstation produced different absolute numeric results for Agatston and volume scores. However, statistical analysis revealed excellent correlation between the workstations, with highest correlation at 60% of the R-R interval (minimal rs=0.998; maximal rs=0.999) for both scoring methods. No significant differences were detected for Agatston and volume score results between the software platforms. At analysis of individual reconstruction intervals, each workstation demonstrated the same score variability, with the consequence that 12 of 59 patients were assigned to divergent cardiac risk groups by using at least one of the workstations.While mere numeric values might be different, commercially available software platforms produce comparable CAC scoring results, which suggests a vendor-independence of the method; however, none of the analyzed software platforms appears to provide a distinct advantage for risk stratification, as the variability of CAC scores depending on the reconstruction interval persists across platforms.

Small-bowel MRI based on contrast-enhanced T1-weighted sequences has been challenged by diffusion-weighted imaging (DWI) for detection of inflammatory bowel lesions and complications in patients with Crohn disease.To evaluate free-breathing DWI, as compared to contrast-enhanced MRI, in children, adolescents and young adults with Crohn disease.This retrospective study included 33 children and young adults with Crohn disease ages 17 ± 3 years (mean ± standard deviation) and 27 matched controls who underwent small-bowel MRI with contrast-enhanced T1-weighted sequences and DWI at 1.5 T. The detectability of Crohn manifestations was determined. Concurrent colonoscopy as reference was available in two-thirds of the children with Crohn disease.DWI and contrast-enhanced MRI correctly identified 32 and 31 patients, respectively. All 22 small-bowel lesions and all Crohn complications were detected. False-positive findings (two on DWI, one on contrast-enhanced MRI), compared to colonoscopy, were a result of large-bowel lumen collapse. Inflammatory wall thickening was comparable on DWI and contrast-enhanced MRI. DWI was superior to contrast-enhanced MRI for detection of lesions in 27% of the assessed bowel segments and equal to contrast-enhanced MRI in 71% of segments.DWI facilitates fast, accurate and comprehensive workup in Crohn disease without the need for intravenous administration of contrast medium. Contrast-enhanced MRI is superior in terms of spatial resolution and multiplanar acquisition.

To evaluate the technical success, clinical outcome and safety of percutaneously placed totally implantable venous power ports (TIVPPs) approved for high-pressure injections, and to analyse their value for arterial phase CT scans.Retrospectively, we identified 204 patients who underwent TIVPP implantation in the forearm (n=152) or chest (n=52) between November 2009 and May 2011. Implantation via an upper arm (forearm port, FP) or subclavian vein (chest port, CP) was performed under sonographic and fluoroscopic guidance. Complications were evaluated following the standards of the Society of Interventional Radiology. Power injections via TIVPPs were analysed, focusing on adequate functioning and catheter's tip location after injection. Feasibility of automatic bolus triggering, peak injection pressure and arterial phase aortic enhancement were evaluated and compared with 50 patients who had had power injections via classic peripheral cannulas.Technical success was 100%. Procedure-related complications were not observed. Catheter-related thrombosis was diagnosed in 15 of 152 FPs (9.9%, 0.02/100 catheter days) and in 1 of 52 CPs (1.9%, 0.002/100 catheter days) (p0.05). Infectious complications were diagnosed in 9 of 152 FPs (5.9%, 0.014/100 catheter days) and in 2 of 52 CPs (3.8%, 0.003/100 catheter days) (p0.05). Arterial bolus triggering succeeded in all attempts; the mean injection pressure was 213.8 psi. Aortic enhancement did not significantly differ between injections via cannulas and TIVPPs (p0.05).TIVPPs can be implanted with high technical success rates, and are associated with low rates of complications if implanted with sonographic and fluoroscopic guidance. Power injections via TIVPPs are safe and result in satisfying arterial contrast. Conventional ports should be replaced by TIVPPs.

Spontaneous rupture of hepatocellular carcinoma (HCC) after transcatheter arterial chemoembolization (TACE) is a rare and life-threatening complication. Pathophysiologic mechanisms are not yet fully known; it is suggested that rupture is preceded by reactive tissue edema and intratumerous bleeding, leading to a rapid expansion of tumour mass with risk of extrahepatic bleeding in the case of subcapsular localisation. This case report discusses a sudden, unexpected lethal complication in a 74 year-old male patient treated with TACE using DC Bead loaded with doxorubicin (DEBDOX) in a progressive multifocal HCC.

BACKGROUND: Magnetization transfer (MT) MRI can provide in vivo markers reflecting the severity of irreversible, MS-related brain damage occurring within and outside T2-visible lesions.OBJECTIVE: To assess the effect of interferon (IFN) beta-1b treatment on the accumulation of brain damage in patients with secondary progressive (SP) MS, measured using MT MRI.METHODS: Eighty-two patients with SPMS from five centers participating in a European, multicenter, double-blind, placebo-controlled trial of IFNbeta-1b in SPMS underwent brain T2-weighted and MT MRI at baseline. Evaluable follow-up data were available for 75 patients at 12 months, 54 at 24 months, and 47 at 36 months. MT MRI scans were postprocessed and analyzed to obtain histograms of MT ratio (MTR) values from the whole brain. A region of interest-based analysis of MTR values from the normal-appearing white matter (NAWM) was also performed.RESULTS: In both the treatment arms, there was a decrease of average brain MTR values from baseline to month 24 (mean change -4.9%) and month 36 (mean change -4.3%). These changes were significant for the placebo group at both timepoints and for the IFNbeta-1b group at month 24 only, with no significant treatment effect. A decrease of NAWM MTR was also observed, with no significant difference between the two treatment arms.CONCLUSION: In this cohort of patients with secondary progressive MS, interferon beta-1b did not show an overall effect on the worsening of magnetization transfer MRI measures, when compared with placebo. The data show that change in magnetization transfer ratio is a promising tool for monitoring disease evolution in secondary progressive MS and that the information obtained from magnetization transfer MRI complements that obtained from MRI measures of lesion load and inflammation.

Our objective was to analyze contrast enhancement patterns (CEP) and their time course after myocardial infarction (MI) following injection of Gd-BOPTA in correlation with recovery of regional function. Seven patients with subacute MI (18 +/- nine days) were examined before, as well as three and six (n = six) months after, revascularization of the infarct-related artery. Regional wall motion abnormalities were assessed by cine-MRI, and repetitive images of one representative slice were acquired up to 45 minutes after 0.05 mmol/kg Gd-BOPTA using a T1-w TSE-sequence. Two patients showed mid-wall/subendocardial, one patient subendocardial enhancement of MI associated with mechanical improvement after revascularization. Three patients without improvement revealed a mid-wall hypoenhanced zone within the first five minutes after injection, which was unchanged at follow-up. One patient with partial functional improvement showed transmural enhancement and a mid-wall hypoenhanced zone in adjacent areas. With this feasibility study, we concluded that mid-wall and/or subendocardial enhancement after Gd-BOPTA was associated with viable myocardium, whereas detection of microvascular obstruction correlating with scar formation is suggested by mid-wall hypoenhancement within the first five minutes after injection.

The purpose of this study was to determine the changes in function of both the left and the right ventricles (LV, RV) before and after aortic valve replacement (AVR), compared with age-matched healthy volunteers using magnetic resonance (MR) imaging. Fourteen patients with aortic stenosis underwent MR imaging (1.5 T) before and 3 (n = 14) and 12 (n = 9) months after surgical valve replacement. An electrocardiographically triggered two-dimensional cine fast low-angle shot sequence was used for the evaluation of absolute values and indices related to 1 m(2) body surface area for function, mass, and LV wall thickening. Fourteen age-matched healthy volunteers served as controls. Before surgery, all patients showed significant abnormalities of LV mass and function, whereas RV mass and function were not different from those of volunteers and remained mostly unchanged. After surgery, normalization of LV ejection fraction, absolute mass, and end-systolic wall thickness was observed, whereas the LV mass index failed to normalize, and LV volumes remained elevated. Aortic stenosis combined with a significant, but not severe reduction in LV function only affects the LV, whereas the RV remains unaffected at this stage of disease. AVR leads to improved LV function and reduced hypertrophy, but without normalization of LV volumes or the LV mass index within 1 year.