Non-invasive determination of myocardial lipid content in Fabry disease by 1H-MR spectroscopy.

PURPOSE: In Fabry disease (FD), a progressive deposition of sphingolipids is reported in different organs. The present study applied 1H magnetic resonance spectroscopy (MRS) to investigate the myocardial lipid content in FD. MATERIALS AND METHODS: In patients (PTS, n = 15) with genetically proven FD, 1H MRS of the heart was acquired in the same examination as routine cardiac cine and late enhancement MR imaging. Healthy volunteers (n = 11) without history of cardiac disease served as control (CTL). Myocardial triglycerides in vivo were quantified in 1H MRS. Left ventricular (LV) ejection fraction (EF) and late enhancement were assessed for the determination of LV systolic function, and onset or absence of myocardial fibrosis. RESULTS: All 1H MRS revealed resonances for intramyocardial triglycerides. Clinical parameters, e.g. EF (PTS 64 ± 2 % vs. CTL 61 ± 1 %) were similar in PTS and CTL or showed a non-significant trend (LV mass). Apart from a single patient with elevated myocardial triglycerides, no significant impact of Fabry disease on the triglyceride/water resonance ratio (PTS 0.47 ± 0.11 vs. CTL 0.52 ± 0.11 %) was observed in our patient cohort. CONCLUSION: A comprehensive cardiac evaluation of morphology, function as well as metabolism in Fabry PTS with suspected cardiac involvement is feasible in a single examination. No significant effect of myocardial triglyceride deposition could be observed in patients. The remarkably high myocardial triglyceride content in one patient with advanced FD warrants further studies in PTS with an extended history of the disease.

Right ventricular involvement in patients with Fabry's disease and the effect of enzyme replacement therapy.

PURPOSE: According to echocardiography reports, Fabry cardiomyopathy not only affects the left ventricle (LV) but also the right ventricle (RV). Until now no MRI studies about the effect of enzyme replacement therapy (ERT) on the RV are available. We evaluated the effect of ERT on the RV. MATERIALS AND METHODS: In this prospective trial 14 patients with genetically proven Fabry's disease were examined using a 1.5 T MR scanner before ERT and after 13 ± 1 months of ERT. All patients underwent cardiac MR imaging and the RV/LV cardiac morphology and function were analyzed. RESULTS: At baseline examination the values were as follows: RV mass 31 ± 6 g/m (2), end-diastolic volume (EDV) 88 ± 13 ml/m (2), end-systolic volume (ESV) 39 ± 9 ml/m (2), stroke volume (SV) 49 ± 7 ml/m (2) and ejection fraction (EF) 56 ± 5 %. The RV mass and EDV decreased significantly after 13 ± 1 months on ERT (mass 27 ± 7 g/m (2), p < 0.05, EDV 76 ± 24 ml/m (2), p < 0.05), with no significant change of ESV (33 ± 13 ml/m (2)), SV (43 ± 12 ml/m (2)) and EF (57 ± 7 %). The LV mass (102 ± 26 g/m (2) vs. 94 ± 27 g/m (2), p < 0.05), EDV (76 ± 13 ml/m (2) vs. 66 ± 22 ml/m (2), p < 0.05) and ESV (29 ± 9 ml/m (2) vs. 23 ± 9 ml/m (2), p < 0.05) decreased significantly while the EF (64 ± 7 % vs. 66 ± 5 %; p < 0.05) increased significantly. CONCLUSION: Besides the known beneficial effect on the LV, ERT improves RV mass and EDV.

[Functional cardiac MRI for assessment of aortic valve disease]. / Aortenklappenstenose im MRT mit Dynamik und 3D.

Aortic valve disease shows a rising incidence with the increasing mean age of Western populations. The detection of hemodynamic parameters, which transcends the mere assessment of valve morphology, has an important future potential concerning classification of the severity of disease. MRI allows a non-invasive and a spatially flexible view of the aortic valve and the adjacent anatomic region, left ventricular outflow tract (LVOT) and ascending aorta. Moreover, the technique allows the determination of functional hemodynamic parameters, such as flow velocities and effective orifice areas. The new approach of a serial systolic planimetry velocity-encoded MRI sequence (VENC-MRI) facilitates the sizing of blood-filled cardiac structures with the registration of changes in magnitude during systole. Additionally, the subvalvular VENC-MRI measurements improve the clinically important exact determination of the LVOT area with respect to its specific eccentric configuration and its systolic deformity.

Comparison of flat-panel-detector-based CT and multidetector-row CT in automated volumetry of pulmonary nodules using an anthropomorphic chest phantom.

This study evaluates the accuracy and reproducibility of an experimental flat-panel-detector-based CT scanner (fp-CT) in comparison with those of a 64-slice multidetector row CT (MDCT) in automated pulmonary nodule volumetry. An anthropomorphic chest phantom with 31 spherical nodules (nodule diameters of 2.94-10.01 mm; volumes of 13.24-524.97 mm(3)) was scanned both with an amorphous silicon-based fp-CT scanner, using various tube current and kilovoltage settings, and with a conventional MDCT scanner. Automated nodule volumetry was performed using dedicated software. CT image data were evaluated twice by two independent radiologists. Intra- and inter-observer variations of volumetric measurements were determined and tested using the Kruskal-Wallis test and analysis of variance (fn-ANOVA). The percentage measurement errors (PME) were calculated and differences tested using Wilcoxon signed ranks and Friedman tests. Intraobserver variation was significantly higher for MDCT than for fp-CT (range: p = 0.043-0.045). The measured nodule volumes were significantly greater on fp-CT than on MDCT scans (p<0.001). The PME was significantly greater in fp-CT than in MDCT scans (PME range, 12.35-13.35% for fp-CT scan protocols and 16.87-19.02% for MDCT scan protocols; p<0.0001). The PME increased significantly with reduction of nodule size, and this increase was significantly higher on MDCT than on fp-CT scans (p = 0.0001). The absolute PME was significantly different for nodules of less than 5 mm in diameter (p = 0.0001-0.0033) than for larger nodules. Flat-panel-detector-based CT has advantages over MDCT in accurately determining the volume of pulmonary nodules below 5 mm in diameter.

[MR imaging and MR spectroscopy for characterization of cardiomyopathies in adolescents - preliminary results]. / MR-Bildgebung und MR-Spektroskopie zur Charakterisierung von Kardiomyopathien bei Jugendlichen - erste Ergebnisse.

PURPOSE: Cardiomyopathy is a rare but life-threatening disease in children and adolescents. Recent studies reported morphological, functional or metabolic alterations of the heart. We discuss a combined MR imaging and (31)P MR spectroscopy (MRS) protocol allowing the analysis of interdependencies between these parameters. Since normal values of cardiac MR parameters in this age group are not available, we included studies of age-matched healthy adolescents. MATERIALS AND METHODS: 2D-CINE was used to assess left ventricular (LV) parameters. Additional 3D-Chemical Shift Imaging (3D-CSI) and Spectral Localization with Optimal Pointspread Function (SLOOP) reconstruction allowed quantification of the cardiac energy metabolism. Patients (n = 4; all male; age 16.8 +/- 2.9 years) were included on the basis of an echocardiographic diagnosis of possible cardiomyopathy. The same protocol was applied to healthy young volunteers (n = 4; 1 female, 3 male; age 15.5 +/- 0.6 years). RESULTS: The patients had a significantly higher LV mass index compared to the control group (147 +/- 41 g/m (2) versus 97 +/- 16 g/m2; p = 0.04). The other LV parameters (including LV EF with 59 +/- 22 % versus 67 +/- 10 %) showed no significant differences. The phosphocreatine to adenosine triphosphate ratio (PCr/ATP-ratio) of the patients was reduced to 1.71 +/- 0.40 versus 2.44 +/- 0.30 (p = 0.01), combined with a tendency towards decreased PCr concentrations of 9.1 +/- 2.5 versus 7.9 +/- 1.0 mmol/kg. CONCLUSION: The combination of (31)P MR spectroscopy and MR imaging allows quantitative determination of morphologic, functional and metabolic alterations in adolescents with suspected cardiomyopathy in one examination procedure. The reduction of energy metabolism combined with unaltered global function may indicate a primary role of metabolism in the pathogenesis of cardiomyopathies in adolescents.

[Interindividual-variability of the analysis of regional myocardial wall function after myocardial infarction and revascularization]. / Interindividuelle-Variabilität der regionalen myokardialen Wandfunktionsanalyse nach Herzinfarkt und Revaskularisierung.

PURPOSE: The gold standard for diagnosis myocardial viability is the functional recovery after revascularization. Aim of the study was to compare (1) qualitative analysis and (2) quantitative wall thickening by cine MRI and (3) circumferential shortening by tagged MRI the analysis of regional wall function of an infarcted area before and after revascularization. MATERIAL AND METHODS: Ten patients (age 60 +/- 11 years) with infarct-associated regional left ventricular wall motion abnormalities were examined by cine and tagged MRI on average two weeks after the myocardial infarction and re-examined three months after revascularization. Eight healthy volunteers served as a control for tagged MRI. Interobserver-variabilities of two observers were calculated using the kappa-statistics for grading of wall motion abnormalities as well as for detection of functional recovery by qualitative analysis, measurement of wall thickening of cine MRI, and measurement of circumferential shortening by tagged MRI, respectively. RESULTS: Grading of wall motion abnormalities revealed interobserver-variabilities of the study and control group of kappa = 0.8 and kappa = 0.84, kappa = 0.02 and kappa = 0.5, and kappa = 0.1 and kappa = 0.17 for qualitative analysis, wall thickening analysis and measurement of circumferential shortening, respectively. The interobserver-variability for the definition of wall motion recovery was kappa = 0.8 for all three methods. CONCLUSION: Qualitative analysis of wall motion abnormalities has the lowest interobserver-variability for the grading of wall motion abnormalities. The interobserver-variabilities of qualitative and quantitative analysis are comparable for the diagnosis of regional wall motion recovery. Thus, qualitative analysis of cine MRI can be used for grading regional wall motion in clinical studies.