The kidney in Fabry's disease.

Fabry disease (FD) is an X-linked disease in which mutations of the GLA gene result in a deficiency of the enzyme α-galactosidase A and subsequent progressive, intralysosomal deposition of undegraded glycosphingolipid products, primarily globotriaosylceramide, in multiple organs. Progressive nephropathy is one of the main features of FD and is marked by an insidious development, with an overall rate of progression of chronic kidney disease (CKD) very similar to diabetic nephropathy. Untreated patients usually develop end stage renal disease in their 50s. The decline in renal function in FD is adversely affected by male gender, advanced CKD, hypertension and, in particular, severe proteinuria. Enzyme replacement therapy (ERT) has been shown to slow the progression of Fabry nephropathy. The current consensus is that ERT should be started in all men and women with signs of renal involvement.

Coronary artery calcification and outcomes in diabetic patients with and without chronic kidney disease.

BACKGROUND/AIMS: Presence and progression of coronary artery calcification (CAC) indicate severe atherosclerosis and predict cardiovascular events (CVE) in dialysis (ESRD patients) and nondialysis patients (CKD patients). This study aimed at evaluating the prevalence and progression of CAC and CVE in CKD patients with diabetes in whom atherosclerosis of coronary arteries is the leading cause of CVE. METHODS: This was a retrospective study conducted in both out- and in-patients with chronic kidney disease, stage 2-5. CAC were assessed by the total calcium (TC) score. CT scans were performed at the entry and at the end of the study. RESULTS: Patients (n = 341) were divided into nondiabetic (n = 281) and diabetic patients (n = 60). CAC prevalence and TC score were higher in diabetics and associated with age, diabetes mellitus and pulse pressure. Diabetics with CKD presented a higher percentage of CAC progression and CVE. CONCLUSION: Prevalence, extent, progression of CAC and CVE are higher in diabetics with concomitant CKD. These findings may promote a more aggressive protocol of screening and care in type 2 diabetics with concomitant CKD.

Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: assessing cardiac effects of long-term enzyme replacement therapy.

PURPOSE: Anderson-Fabry disease is a multisystemic disorder of lipid metabolism secondary to X-chromosome alterations and is frequently associated with cardiac manifestations such as left ventricular (LV) hypertrophy, gradually leading to an alteration in cardiac performance. The purpose of this study was to monitor, using magnetic resonance imaging (MRI), any changes produced by enzyme replacement therapy with agalsidase beta at the cardiac level in patients with Anderson-Fabry disease. MATERIALS AND METHODS: Sixteen (ten men, six women) patients with genetically confirmed Anderson-Fabry disease underwent cardiac MRI before starting enzyme replacement therapy (baseline study) and after 48 months of treatment with agalsidase beta at the dose of 1 mg/kg (follow-up study). RESULTS: After 48 months of treatment, a significant reduction in LV mass and wall thickness was observed: 187±59 g vs. 149±44 g, and 16±3 mm vs. 13±3 mm, respectively. A significant reduction in T2 relaxation time was noted at the level of the interventricular septum (81±3 ms vs. 67±7 ms), at the apical level (80±8 ms vs. 63±6 ms) and at the level of the lateral wall (82±8 ms vs. 63±10 ms) (p<0.05). No significant variation was observed in ejection fraction between the two studies (65±3% vs. 64±2%; p>0.05) (mean bias 1.0); however, an improvement was noted in the New York Heart Association (NYHA) class of the majority of patients (12/16) (p<0.05). CONCLUSIONS: In patients with Anderson-Fabry disease undergoing enzyme replacement therapy with agalsidase beta, MRI documented a significant reduction in myocardial T2 relaxation time, a significant decrease in maximal myocardial thickness and in total LV mass. MRI did not reveal significant improvements in LV global systolic function; however, improvement in NYHA functional class was noted, consistent with improved diastolic function.

Comparison between multislice CT and MR imaging in the diagnostic evaluation of patients with pancreatic masses.

PURPOSE: This study compared the results of multislice computed tomography (MSCT) and high-field magnetic resonance imaging (MRI) in the diagnostic evaluation of pancreatic masses. MATERIALS AND METHODS: Forty patients with clinical and ultrasonographic evidence of pancreatic masses underwent MSCT and MRI. The majority of patients (31/40, 78%) had proven malignant pancreatic tumours (24 ductal adenocarcinoma, six mucinous cystadenocarcinoma, one intraductal papillary mucinous carcinoma), whereas the remaining patients (9/40, 22%) were found to have benign lesions (eight chronic pancreatitis, one serous cystadenoma). Results of the imaging studies were compared with biopsy (n=33) and/or histology (n=7) findings to calculate sensitivity, specificity, accuracy and positive (PPV) and negative (NPV) predictive value for correct identification of tumours and evaluation of resectability of malignancies. RESULTS: Both for tumour identification and resectability, MSCT and MRI had comparable diagnostic accuracy, with no statistically significant differences between them. Tumour identification CT/MRI: accuracy 98/98%, sensitivity 100/100%, specificity 88/88%, PPV 97/97%, NPV 100/100%; tumour resectability CT/MRI: accuracy 94/90%, sensitivity 92/88%, specificity 100/100%, PPV 100/100%, NPV 78/70%. CONCLUSIONS: MRI represents a valid diagnostic alternative to CT in the evaluation of patients with pancreatic masses, both for correct identification and characterisation of primary lesions and to establish resectability in the case of malignancies. New high-field MRI equipment allows optimal imaging quality with good contrast resolution in evaluating the upper abdomen.

Dual-time-point [18F]-FDG PET/CT in the diagnostic evaluation of suspicious breast lesions.

PURPOSE: The authors sought to evaluate whether the reacquisition of images 3 h after administration of radiotracer improves the sensitivity of fluorine-18 fluorodeoxyglucose positron emission tomography computed tomography ([(18)F]-FDG PET/CT) in patients with suspicious breast lesions. MATERIALS AND METHODS: Forty-eight patients with 59 breast lesions underwent an [(18)F]-FDG PET/CT study in the prone position with a dual-time-point acquisition performed in the early phase 1 h after FDG administration (PET-1) and in the delayed phase 3 h after FDG administration (PET-2). Both examinations were evaluated qualitatively and semiquantitatively with calculation of the mean percentage variation of the standard uptake values (Delta% SUV(max)) between PET-1 and PET-2. All lesions with an SUV(max) >or=2.5 at PET-1 or a reduction in SUV between PET-1 and PET-2 were considered benign. The definitive histopathological diagnosis was available for all patients included in the study. RESULTS: The dual-time-point acquisition of [(18)F]-FDG PET/CT displayed an accuracy of 85% for lesions with an SUV(max) >or=2.5 and/or positive Delta% SUV(max), with sensitivity and specificity values of 81% and 100% compared with 69%, 63% (both p<0.001) and 100% (p=n.s.), respectively, for the single-time-point acquisition. Malignant lesions showed an increase in FDG uptake between PET-1 and PET-2, with a Delta% SUV(max) of 10+/-7 (p<0.04). In contrast, benign lesions showed a decrease in SUV between PET-1 and PET-2, with a Delta% SUV(max) of -21+/-7 (p<0.001). CONCLUSIONS: The delayed repeat acquisition of PET images improves the accuracy of [(18)F]-FDG PET/CT in patients with suspicious breast lesions with respect to the single-time-point acquisition. In addition, malignant breast lesions displayed an increase in FDG uptake over time, whereas benign lesions showed a reduction. These variations in FDG uptake between PET-1 and PET-2 are a reliable parameter that can be used for differentiating between benign and malignant breast lesions.

Cardiac performance during exercise in patients with Fabry's disease.

BACKGROUND: Fatigability and dyspnoea on effort are present in many patients with Fabry's disease. We assessed the determinants of cardiac performance during exercise in patients with Fabry's disease and preserved left ventricular ejection fraction at rest. MATERIALS AND METHODS: Sixteen patients with Fabry's disease and 16 control subjects underwent radionuclide angiography at rest and during exercise, tissue Doppler echocardiography and magnetic resonance imaging at rest. RESULTS: The exercise-induced change in stroke volume was +25 +/- 14% in controls and +5.8 +/- 19% in patients with Fabry's disease (P < 0.001). In 10 patients (group 1), the stroke volume increased (+19 +/- 10%), and in 6 patients (group 2) it decreased (-16 +/- 9%) with exercise. Patients of group 2 were older, had worse renal function, higher left ventricular mass and impaired diastolic function compared to group 1. The abnormal stroke volume response to exercise in group 2 was associated with a decrease in end-diastolic volume (P < 0.001) and a lack of reduction of end-systolic volume (P < 0.01) compared with both controls and group 1. The ratio of peak early-diastolic velocity from mitral filling to peak early-diastolic mitral annulus velocity was the only independent predictor of exercise-induced change in stroke volume (B -0.44; SE 0.119; beta-0.70; P < 0.005). CONCLUSIONS: The majority of patients with Fabry's disease were able to augment stroke volume during exercise by increasing end-diastolic volume, whereas patients with more advanced cardiac involvement may experience the inability to increase cardiac output by the Frank Starling mechanism.

Pathogenesis of hypertrophic cardiomyopathy. Impact of growth factors on left ventricular anatomy.

AIM: The aim of this study was to evaluate the effect of insulin-like growth factor 1 (IGF1) and transforming growth factor beta-1 (TGFbeta-1) on collagen turnover, left ventricular (LV) hypertrophy and on passive diastolic function of the LV in hypertrophic cardiomyopathy (HCM). METHODS: This study group comprised 34 patients with non-dilated HCM. Procollagen I amino-terminal propeptide (PINP) and collagen I carboxy-terminal telopeptide (ICTP) were measured by radioimmunoassay. Matrix metalloproteinase 9 (MMP 9), IGF1 and TGFalfa-1 were determined by enzyme-linked immunosorbent assay. The difference in duration between transmitral forward (A) and pulmonary venous retrograde (Ar) waves, was considered as an estimate of passive diastolic function; the ratio between the peak flow velocity at rapid filling at the mitral level (E) and E' measured by tissue Doppler was considered an estimate of active diastolic function. LV mass was measured and normalized to body surface area (LVMi) by cardiac magnetic resonance imaging. RESULTS: LVMi correlates to E/E' (r=0.597, P=0.019 ) and is inversely related to A-Ar (r=0.453, P=0.015). TGFbeta-1 is directly related to active MMP 9 (r=0.439, P=0.012 ). IGF1 is directly related to PICP-ICTP (r=0.347, P=0.501), that expresses the balance between collagen I synthesis and its degradation. CONCLUSION: The study demonstrated that in HCM, LVMi influences active and passive diastolic dysfunction and that IGF1 stimulates collagen synthesis and TGFbeta-1 is related to LV hypertrophy.

Redefining the Pulvinar Sign in Fabry Disease.

BACKGROUND AND PURPOSE: The pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity. MATERIALS AND METHODS: We retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls. RESULTS: The pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables. CONCLUSIONS: The pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease.

Difficult vascular conditions in kidney transplantation.

The new dialytic and medical therapies have improved the survival of uremic patients and their preservation of a efficacious clinical condition so as to warrant suitability for transplantation, even after a long period of dialysis. In addition, today the use of a "marginal donor" and "marginal kidney" are often used to increase the pool of available organs, so that the surgeon must face more technical difficulties than in the past; anomalies of the donor kidney, harvesting and bench surgery damages, as well as vascular pathologies in the recipient. A review of our 151 renal transplantations from January 1999 to May 2003 showed that it was often possible to overcome these technical difficulties yielding good results. This work sought to demonstrate that neither "marginal donor"/"marginal kidney" used to expand donor pool nor recipient vascular pathologies should be considered transplant contraindications. Knowledge of various technical options and the ability to put them rapidly into practice are necessary to use any organ.

Prognostic significance of extent of disease in bone in patients with androgen-independent prostate cancer.

PURPOSE: To evaluate the prognostic significance of a bone scan index (BSI) based on the weighted proportion of tumor involvement in individual bones, in relation to other factors and to survival in patients with androgen-independent prostate cancer. PATIENTS AND METHODS: Baseline radionuclide bone scans were reviewed in 191 assessable patients with androgen-independent disease who were enrolled onto an open, randomized trial of liarozole versus prednisone. The extent of skeletal involvement was assessed by scoring each scan using the BSI and independently according to the number of metastatic lesions. The relationship of the scored bone involvement to other known prognostic factors was explored in single- and multiple-variable analyses. RESULTS: In single-variable analyses, the pretreatment factors found to be associated with survival were age (P = .0446), performance status (P = .0005), baseline prostate-specific antigen (P = .0001), hemoglobin (P = .0001), alkaline phosphatase (P = .0002), AST (P = .0021), lactate dehydrogenase (P = .0001), and treatment (P = .0098). The extent of osseous disease was significant using both the BSI (P = .0001) and the number of lesions present (P = .0001). In multiple-variable proportional hazards analyses, only BSI, age, hemoglobin, lactate dehydrogenase, and treatment arm were associated with survival. When the patient population was divided into three equal groups, with BSI values of < 1.4%, 1.4% to 5.1%, and > 5.1%, median survivals of 18.3, 15.5, and 8.1 months, respectively, were observed (P = .0079). CONCLUSION: The BSI quantifies the extent of skeletal involvement by tumor. It allows the identification of patients with distinct prognoses for stratification in clinical trials. Further study is needed to assess the utility of serial BSI determinations in monitoring treatment effects. The BSI may be particularly useful in the evaluation of agents for which prostate-specific antigen changes do not reflect clinical outcomes accurately.

A new parameter for measuring metastatic bone involvement by prostate cancer: the Bone Scan Index.

In this report, we describe a method for quantitative bone scan interpretation (the Bone Scan Index or BSI) in advanced prostate cancer. The BSI estimates the fraction of the skeleton that is involved by tumor, as well as the regional distribution of the metastases in the bones. The purpose of this report is to describe the development and validation of this method in terms of reproducibility and the application of BSI for determining extent of disease and monitoring disease progression. We analyzed 263 bone scans from 90 patients being studied under four protocols at Memorial Sloan-Kettering Cancer Center for progressive, androgen-independent prostate cancer (AIPC), who had bone scans as a part of their work-up. We determined: (a) the intraobserver and interobserver variability of the BSI; (b) the comparison between a change in BSI and prostate-specific antigen (PSA); (c) the regional distribution of bony metastases in early stage D prostate cancer (<3% skeletal involvement); and (d) the rate of growth of bony metastases from prostate cancer. A cube root transformation of the percentage of involvement of the entire skeleton was used to stabilize the variance over the entire span of values (0-60% tumor involvement). The range of interobserver variability between readers was 0.2-0.5 times the cube root of the BSI (69 scans, 18 patients). Intraobserver variability was minimal when the same reader read the same scans after a 2-year interval, showing a correlation coefficient of 0.97 (reader 1) and 0.99 (reader 2), P < 0.001. There was a parallel rise in the BSI and the PSA in 24 patients (105 scans) treated for AIPC with hydrocortisone followed by suramin at PSA relapse (Pearson's moment correlation, 0.71). In a group of 27 patients with limited bone involvement by AIPC (i.e., <3% BSI), the distribution of early metastases was not random within the skeleton but was distributed in the central skeleton in a manner that matched the distribution of the normal adult bone marrow. Also, in a group of 21 patients (62 scans), the change in BSI as a function of time after diagnosis was explored graphically. The progression of bone scan changes in AIPC, from early involvement (<3%) to late involvement, was fitted to a Gompertzian equation. It showed a rapid exponential growth phase, with an estimated tumor doubling time of 43 days when the BSI was 3.3%. The change in BSI rapidly approached a more gradual slope as the percentage of skeletal involvement increased. The BSI provides a reproducible new parameter for quantitative assessment of bone involvement by AIPC. These results suggest that the BSI will be useful for stratifying patients entering treatment protocols for extent of tumor involvement of bone. Although further study is necessary, serial bone scan BSI appears capable of quantifying both the progression of bony involvement by tumor as well as the response to treatment.

Interferon-gamma and monoclonal antibody 131I-labeled CC49: outcomes in patients with androgen-independent prostate cancer.

To assess the tumor targeting, safety, and efficacy of monoclonal antibody 131I-labeled CC49 in patients with androgen-independent prostate cancer, 16 patients received 75 mCi/m2 of the radiolabeled antibody after 7 days of IFN-gamma pretreatment. Sequential tumor biopsies in three patients showed a median 5-fold (range, 2-6-fold) increase in the proportion of cells staining positively for the TAG-72 antigen, whereas one showed a decrease in staining. Fourteen patients received 131I-labeled CC49, whereas 2 showed a disease-related decrease in performance status, precluding antibody treatment. The antibody localized to sites of metastatic androgen-independent prostate cancer in 86% (12 of 14; 95% confidence interval, 57-95%) of cases. Both osseous and extraosseous sites were visualized, and in six (42%) patients, more areas were visible when the radioimmunoconjugate was used than were apparent when conventional scanning techniques were used. The localization of the conjugate in the marrow cavity was usually a site not visualized by the radionuclide bone scan, in which the isotope localizes primarily to the tumor-bone interface. The dose-limiting toxicity was thrombocytopenia because five (36%) patients showed grade IV and seven (50%) showed grade III effects. In addition, six (42%) patients, four of whom were hospitalized, showed a flare in baseline pain, and four showed a decrease in pain. No patient showed a >50% decline in prostate-specific antigen, although radionuclide bone scans remained stable in four cases for a median of 4 months. The results are consistent with dosimetry estimates showing that the delivered dose to tumor was subtherapeutic and suggest that approaches that exclusively target the bone tumor interface or the marrow stroma may be unable to completely eradicate disease in the marrow cavity. For CC49, improving outcomes would require repetitive dosing, which was precluded by the rapid development of a human antimouse antibody response.

A study on two different CAD systems for mammography as an aid to radiological diagnosis in the search of microcalcification clusters.

OBJECTIVE: The aim of the present study was to evaluate the efficacy of two different computer aided detection (CAD) systems for mammography in improving radiological diagnosis in the search of microcalcification clusters. The CAD systems used are: the SecondLooktrade mark (CADx Medical Systems, Canada) commercial system and the CALMA (computer assisted library in MAmmography) research CAD system. Three radiologists were asked to read mammographic images with and without the support of the CAD systems. MATERIAL AND METHODS: Three radiologists with respectively 3, 5 and 7 years of practice in mammogram reading in an Italian public hospital analysed a dataset composed of 120 digitized mammograms of healthy subjects with no lesion (proven by a radiological follow up of at least 3 years) and 70 images of patients with malignant cluster of microcalcification (proven by histopathological examination) both with no CAD support as well as with the help of the SecondLooktrade mark system. After 3 months they were asked to observe the same digitized mammograms with the assistance of the CALMA system. The radiologists worked independently and were unaware of the final diagnosis. The values of the area A(z) under the ROC curve, diagnostic sensitivity, specificity, positive and negative predictive values, and diagnostic accuracy were evaluated with and without the support of the CAD systems. The reading time and qualitative evaluations of each radiologist were also reported. RESULTS: With the support of the two CAD systems an improvement in A(z) area was obtained ranging from 0.01 to 0.04. Sensitivity increased from +8.6 to +15.7% and specificity decreased from 0.8 to 4.2%. CONCLUSION: In our study, not conditioned by the dataset, the CAD systems as second reader produced an increase in overall sensitivity of up to 15.7%, with a little decrease in specificity of up to 4.2%. Based on these results both CAD systems might be used in the current practise to improve the sensitivity values of conventional reading (radiologist alone). The results of this study show that no significant differences exist in term of A(z), sensitivity and specificity between CALMA and CADx.

Noninvasive evaluation of cardiac hemodynamics during exercise in patients with chronic heart failure: effects of short-term coenzyme Q10 treatment.

In patients with chronic heart failure (CHF), the addition of coenzyme Q10 to conventional therapy reduces the hospitalization rate for worsening of heart failure and the incidence of serious cardiovascular complications. The present study was planned to assess the hemodynamic mechanisms underlying this phenomenon. Cardiac hemodynamics was evaluated continuously using an ambulatory radionuclide detector (VEST) which allows a noninvasive monitoring of left ventricular function. Six patients wit CHF (mean ejection fraction (EF): 29%) clinically documented were studied. This study was organized as a randomized double-blind, placebo controlled, cross-over trial. The enrolled patients, after a washout period, underwent the first hemodynamic evaluation with VEST. Subsequently they were randomized to receive placebo or coenzyme Q10 for 4 weeks. At the end of this period they underwent the second VEST study. The third VEST study was performed after a further 4-week period with inverted treatment. Cardiac hemodynamics were evaluated during bicycle exercise. The EF in control conditions (CC) changed from 27 +/- 11%, at rest, to 24 +/- 8%, at peak exercise. During coenzyme Q10 treatment EF showed a significant increase both at rest (33 +/- 13%, P < 0.05 vs CC) and at peak exercise (30 +/- 12%, P < 0.05 vs CC). The same trends were recorded for the stroke volume and the cardiac output. Our results demonstrate that coenzyme Q10 improves cardiac hemodynamic response to exercise in patients with CHF and suggest that noninvasive monitoring of left ventricular function allows a more reliable assessment of therapy efficacy.

Quantitative bone metastases analysis based on image segmentation.

UNLABELLED: Preliminary evidence indicates that the fraction of bone containing metastatic lesions is a strong prognostic indicator of survival longevity for prostate and breast cancer. Our current approach to quantify metastatic bone lesions, called the Bone Scan Index, is based on an inspection of the bone scan, estimating visually the fraction of each bone involved and then summing across all bones to determine the percentage of total skeletal involvement. This approach, however, is time consuming, subjective and dependent on individual interpretation. METHODS: To overcome these problems, a semiautomated image segmentation program was developed for the quantitation of metastases from planar whole-body bone scans. The user is required to insert a seed point into each metastatic region on the image. The algorithm then connects pixels to the seed pixel in all directions until a contrast-dependent threshold is reached. The optimal threshold for cessation of the region growing is determined from phantom studies. On the images, lesion delineation and size measurements were performed by the algorithm. Each delineated lesion is associated with a bone site using pull-down menus. The program then computes the fraction of lesion involvement in each bone based on look-up-tables containing the relationship of bone mass with race, sex, height and age. These look-up-tables were obtained by multiple regression of the skeletal mass measurements in humans. The total fraction of skeletal involvement is then obtained from the individual fractional masses. For individual fractional mass, values given in International Commission on Radiation Protection Publication No. 23 were used. RESULTS: The bone metastases analysis system has been used on 11 scans from 6 patients. The correlation was high (r = 0.83) between conventional (manually drawn region-of-interest) and this analysis system. Bone metastases analysis results in consistently lower estimates of fractional involvement in bone compared with the conventional region-of-interest drawing or visual estimation method. This is due to the apparent broadening of objects at and below the limits of resolution of the gamma camera. CONCLUSION: Image segmentation reduces the delineation and quantitation time of lesions by at least two compared with manual region-of-interest drawing. The objectivity of this technique allows the detection of small variations in follow-up patient scans for which the manual region-of-interest method may fail, due to performance variability of the user. This method preserves the diagnostic skills of the nuclear medicine physician to select which bony structures contain lesions, yet combines it with an objective delineation of the lesion.

Technetium-99m-MIBI versus fluorine-18-FDG in diffuse multiple myeloma.

Experience of scintigraphic detection of bone lesion and active bone marrow involvement of multiple myeloma, especially with sestamibi and FDG-PET scans is in evolution. We report a case of intense sestamibi uptake in bone marrow correlating with the extent of the disease, while FDG-PET scans showed activity only in areas of active disease progression associated with pain. Technetium-99m-sestamibi appears to indicate the extent of the disease, while [18F]FDG-PET scans show sites of active tumor proliferation and may be useful in directing local therapy such as radiation.

Visualization of myocardial metastasis of carcinoid tumor by indium-111-pentetreotide.

We present a case of metastatic carcinoid tumor metastatic to the heart, presenting as ventricular arrhythmia and diagnosed by 111Inpentetreotide scintiscan despite negative endocardial biopsy. The incidence and diagnosis of carcinoid heart disease is discussed, as well as the complementary role of high-resolution anatomical images (CT, MRI) with functional images (SPECT, PET) to determine the correct diagnosis of this rare condition.

Enhanced detection of viable myocardium by technetium-99m-MIBI imaging after nitrate administration in chronic coronary artery disease.

UNLABELLED: The aim of this study was to assess whether nitrate administration improves the imaging capabilities of 99mTc-MIBI tomography in detecting viable myocardium in coronary artery disease (CAD). METHODS: Thirty-one patients with angiographically proven CAD and chronic LV dysfunction (ejection fraction 39% +/- 9%) underwent two 99mTc-MIBI studies on separate days: one under rest conditions and the other after nitroglycerine (0.005 mg/kg per os) administration. Within 1 wk, all patients also underwent rest-redistribution 201Tl imaging. Eight patients were also studied by echocardiography before and 5 +/- 3 mo after coronary revascularization. RESULTS: On resting 99mTc-MIBI images, 302 segments had normal uptake, 183 segments had moderately reduced uptake and 197 had severely reduced uptake. Of the segments with severely reduced uptake, 54 (27%) had increased uptake after nitroglycerine and were viable on 201Tl images. Of the 143 (73%) segments with severely reduced 99mTc-MIBI uptake and no change after nitroglycerine, 81% were nonviable on 201Tl images. In the eight patients studied before and after revascularization, 87% of segments with reversible 99mTc-MIBI defects and abnormal LV function demonstrated functional recovery after revascularization, whereas 89% of segments with irreversible 99mTc-MIBI defects did not. CONCLUSION: In patients with chronic ischemic LV dysfunction, nitrate administration improved the detection of severely hypoperfused but still viable myocardium on 99mTc-MIBI images.

Myocardial viability index in chronic coronary artery disease: technetium-99m-methoxy isobutyl isonitrile redistribution.

UNLABELLED: The purpose of this study was to evaluate whether an additional redistribution image after a rest 99mTc-MIBI injection enhances detection of viable myocardium and predicts functional recovery after coronary revascularization in patients with chronic coronary artery disease (CAD). METHODS: Thirty-one patients (29 men, mean age 55 +/- 10 yr) with proven CAD and left ventricular (LV) dysfunction (ejection fraction 39% +/- 9%) underwent resting 99mTc-MIBI tomography with initial (1 hr) and delayed (5 hr) images. Within 1 wk of MIBI imaging, all patients underwent rest-distribution 201Tl imaging. Eight patients also underwent two-dimensional echocardiography before and 5 +/- 3 mo after coronary revascularization. RESULTS: On the initial 99mTc-MIBI images, 302 myocardial segments were normal, 183 showed moderate and 197 severe reduction of tracer uptake. Of these 197 segments, 47 (24%) demonstrated increased tracer uptake (> or = 10% versus initial) on delayed images (from 43% +/- 8% to 60% +/- 8%, p < 0.001) and were considered as showing 99mTc-MIBI redistribution. These 47 segments were observed in 20 (65%) patients in whom 201Tl images detected viable myocardium in the same segments. In the eight patients studied before and after revascularization, 83% of segments with 99mTc-MIBI redistribution and abnormal LV function showed functional recovery after revascularization, while 96% of segments without 99mTc-MIBI redistribution did not show functional recovery. CONCLUSION: Resting 99mTc-MIBI redistribution frequently occurs in patients with chronic CAD. Acquisition of 99mTc-MIBI redistribution images enhances detection of viable myocardium and predicts functional recovery after revascularization.