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Background Fabry cardiomyopathy is characterized by left ventricular (LV) hypertrophy and regional fibrosis. Recent high-end echocardiography studies of selected LV sections suggest an interrelation between regional fibrosis, impaired function, and hypertrophy possibly changing under specific enzyme replacement therapy (ERT). Methods Magnetic resonance imaging (MRI) was used for a region dependent study of cardiac function, morphology and late enhancement (LE) in 25 Fabry patients before and after 12months of ERT in comparison to 43 healthy volunteers. Results Fabry patients presented with LV increased wall thickness (EDWT) and reduced wall thickening (WT) with a focus on basal and midventricular regions corresponding to areas of LE. The degree of hypertrophy and hypokinesia were the highest if LE was detectable. A significant decrease of the EDWT under ERT was observed in LE negative patients accompanied by a decline of hypokinesia with regional differences. Conclusions Regional differences of LV hypertrophy and wall motion were detected corresponding to the distribution of myocardial fibrosis (LE). Functional impairment was closely restricted to fibrotic regions while morphologic changes slightly exceeded the areas of fibrosis. ERT resulted in regional improvements whereby absence of fibrosis was connected to a better outcome.

The authors report the case of a patient with acute lower limb ischemia (category IIa) after occlusion of the popliteal artery due to fracture of a long indwelling stent. The patient refused surgical therapy for religious reasons, and an interventional revascularization was performed as acute rescue therapy. After reentry into the distal popliteal artery was achieved, the artery was dilated, and the fragmented stent was crushed, followed by implantation of two helical nitinol stents with high radial force and a third self-expandable nitinol stent. Sufficient primary technical success was achieved, and stent patency was present at midterm follow-up.

Purpose To determine sensitivity, specificity and inter-observer variability of different whole-body MRI (WB-MRI) sequences in patients with multiple myeloma (MM). Methods and materials WB-MRI using a 1.5 T MRI scanner was performed in 23 consecutive patients (13 males, 10 females; mean age 63 ± 12 years) with histologically proven MM. All patients were clinically classified according to infiltration (low-grade, n = 7; intermediate-grade, n = 7; high-grade, n = 9) and to the staging system of Durie and Salmon PLUS (stage I, n = 12; stage II, n = 4; stage III, n = 7). The control group consisted of 36 individuals without malignancy (25 males, 11 females; mean age 57 ± 13 years). Two observers independently evaluated the following WB-MRI sequences: T1w-TSE (T1), T2w-TIRM (T2), and the combination of both sequences, including a contrast-enhanced T1w-TSE with fat-saturation (T1 ± CE/T2). They had to determine growth patterns (focal and/or diffuse) and the MRI sequence that provided the highest confidence level in depicting the MM lesions. Results were calculated on a per-patient basis. Results Visual detection of MM was as follows: T1, 65% (sensitivity)/85% (specificity); T2, 76%/81%; T1 ± CE/T2, 67%/88%. Inter-observer variability was as follows: T1, 0.3; T2, 0.55; T1 ± CE/T2, 0.55. Sensitivity improved depending on infiltration grade (T1: 1 = 60%; 2 = 36%; 3 = 83%; T2: 1 = 70%; 2 = 71%; 3 = 89%; T1 ± CE/T2: 1 = 50%; 2 = 50%; 3 = 89%) and clinical stage (T1: 1 = 58%; 2 = 63%; 3 = 79%; T2: 1 = 58%; 2 = 88%; 3 = 100%; T1 ± CE/T2: 1 = 50%; 2 = 63%; 3 = 100%). T2w-TIRM sequences achieved the best reliability in depicting the MM lesions (65% in the mean of both readers). Conclusions T2w-TIRM sequences achieved the highest level of sensitivity and best reliability, and thus might be valuable for initial assessment of MM. For an exact staging and grading the examination protocol should encompass unenhanced and enhanced T1w-MRI sequences, in addition to T2w-TIRM.

OBJECTIVES: The present study investigated changes in cardiac energy metabolism and function in patients with dilated cardiomyopathy (DCM) before and after exercise training (ET) with phosphorus-31 magnetic resonance spectroscopy (MRS) in combination with magnetic resonance imaging (MRI). BACKGROUND: Exercise training might have a beneficial role on myocardial function and oxidative metabolism in DCM, but it is unclear whether the additional load on the failing heart leads to deterioration of cardiac energy metabolism. METHODS: Twenty-four patients were randomized to an exercise (age 53 +/- 12 years) or a control (age 56 +/- 6 years) group. Supervised ET was performed for 2 months, followed by 6 months of self-regulated training. At baseline and 2 and 8 months, maximal exercise testing along with quantitative MRS and MRI studies were performed. RESULTS: The effectiveness of ET was demonstrated by a 17% increase in peak oxygen uptake (p < 0.05). Exercise training improved left ventricular (LV) end-systolic volume (p < 0.05) and LV ejection fraction (30 +/- 15% vs. 37 +/- 15%; p < 0.01) but not right ventricular parameters. The improvement in cardiac function was not accompanied by changes in cardiac high-energy phosphate concentrations; phosphocreatine, adenosine triphosphate, and the phosphocreatine/adenosine triphosphate ratio were all unchanged after training. CONCLUSIONS: The observation that LV function improved and LV energy metabolism remained unchanged suggests that the beneficial effect of ET on LV function is achieved without adversely affecting metabolism. These findings lend further support for the use of ET as an adjunct therapy in DCM.

Objectives The purpose of the present study was to measure absolute concentrations of phosphocreatine (PCr) and adenosine triphosphate (ATP) in normal, hypertrophied, and failing human heart. Background Conflicting evidence exists on the extent of changes of high-energy phosphate metabolites in hypertrophied and failing human heart. Previous reports using phosphorus-31 magnetic resonance spectroscopy ( 31 P-MRS) have quantified metabolites in relative terms only. However, this analysis cannot detect simultaneous reductions. Methods Four groups of subjects (n = 10 each), were studied: volunteers and patients with hypertensive heart disease (HHD), aortic stenosis, and dilated cardiomyopathy (DCM). Left ventricular (LV) function and mass were measured by cine magnetic resonance imaging. Absolute and relative concentrations of PCr and ATP were determined by 31 P-MRS with spatial localization with optimum pointspread function. Results Left ventricular ejection fraction remained normal in HHD and aortic stenosis, but was severely reduced to 18% in DCM; LV mass was increased by 55%, 79%, and 68% respectively. In volunteers, PCr and ATP concentrations were 8.82 ± 1.30 mmol/kg wet weight and 5.69 ± 1.02 mmol/kg wet weight, and the PCr/ATP ratio was 1.59 ± 0.33. High-energy phosphate levels were unaltered in HHD. In aortic stenosis, PCr was decreased by 28%, whereas ATP remained constant. In DCM, PCr was reduced by 51%, ATP by 35%, and reduction of the PCr/ATP ratio by 25% was of borderline significance (p = 0.06). Significant correlations were observed among energetic and functional variables, with the closest relations for PCr. Conclusions In human heart failure due to DCM, both PCr and ATP are significantly reduced. Ratios of PCr to ATP underestimate changes of high-energy phosphate levels.

The increasing frequency of LV functional MRI studies demands for faster methods and for more comfort for the patient. We tested, whether real-time (RT) non ECG triggered MRI allows a considerable shortening of examination time in high reproducibility. RT and standard ECG-triggered breathhold cine MRI was acquired in 9 volunteers and 21 patients. Differences between both methods were assessed by Bland–Altman analyses including variability studies. Compared to standard cine MRI, RT decreased data acquisition time by more than the factor of ten. RT produced comparable results (e.g. EF in %: +0.67 [−5.63, 6.97]) except for a slight overestimation of LV mass. Interstudy and intraobserver variability of RT cine showed a low variability. Consequently, free-breathing RT cine proved to be a reliable and suitable tool for clinical routine and may be particularly relevant in patients with sub-optimal breath-holding ability and arrhythmia.

Pathologic changes of the intranodal angioarchitecture, as displayed by colour Doppler sonography, were used in recent studies to predict malignant infiltration of superficial lymph nodes. We searched for the underlying histopathologic findings in a prospective study including 100 lymph nodes in 86 patients. In the histopathologic specimens, we evaluated tumour infiltration, distribution of intranodal vessels, hilar structures, thickness of the capsule and tissue alterations (necrosis, sclerosis, lipo-/fibromatosis). Using the only prospectively tested classification, a pathologic angioarchitecture was described as displacement, aberrant course, avascular foci or subcapsular vessels. Testing these four criteria by multivariate regression analysis, the most important correlations were found between the following pairs of sonographic and histopathologic findings: displacement with perinodal tumour spread, aberrant vessels with intranodal sclerosis, avascular foci with intranodal necroses and subcapsular vessels with intranodal necroses. In conclusion, the criteria describing a pathologic angioarchitecture correlate with histopathologic findings that are frequently seen in malignant lymph nodes.

We report a case of a 72-year-old man whose multi-slice cardiac computed tomography revealed an anomalous origin of the right coronary artery between the great arteries.

Using 31P-MR spectroscopy, abnormalities of cardiac energy metabolism have been demonstrated in patients with dilated cardiomyopathy (DCM). However. a detailed analysis of the correlations among energy metabolism, cardiac hemodynamics and myocardial hypertrophy obtained from 31P-MR, right and left heart catheterization and echocardiography has not been presented, 23 patients with DCM (left ventricular (LV) EF 34 ± 3%; NYHA class 2.7 ± 0.1; SE) underwent right and left heart catheterization and echocardiography ± 3 days before/after MR spectroscopy. Coronary artery disease was ruled out by coronary angiography. ECG-triggered. localized 31 P-MR spectra from the anteroseptal myocardium were acquired at rest (prone position) during 30 min on a 1.5 T Philips Gyroscan MR system using ISIS localization, adiabatic pulses. and a 15 sec repetition time. Peak areas were corrected for T1 effects and for blood contamination. and were determined with Lorentzian line fits in the time domain. Linear correlations between creatine phosphate (CP)/ATP ratios and hemodynamic parameters were calculated. Download : Download high-res image (69KB) Download : Download full-size image LV pressures and diameters. cardiac output, stroke volume, pulmonary arterial pressures, right atrial pressure and pulmonary arterial oxygen saturation did not correlate with CP/ATP. Thus, our data demonstrate that in DCM, the extent of high-energy phosphate depletion is related to the extent of mechanical dysfunction as well as to LV wall thickness.