Exogenous insulin-like growth factor 1 attenuates cisplatin-induced muscle atrophy in mice.

BACKGROUND: A reduction in the skeletal muscle mass worsens the prognosis of patients with various cancers. Our previous studies indicated that cisplatin administration to mice caused muscle atrophy. This is a concern for human patients receiving cisplatin. The insulin-like growth factor 1 (IGF-1)/phosphoinositide 3-kinase (PI3K)/Akt pathway stimulates the rate of protein synthesis in skeletal muscle. Thus, IGF-I can be a central therapeutic target for preventing the loss of skeletal muscle mass in muscle atrophy, although it remains unclear whether pharmacological activation of the IGF-1/PI3K/Akt pathway attenuates muscle atrophy induced by cisplatin. In this study, we examined whether exogenous recombinant human IGF-1 attenuated cisplatin-induced muscle atrophy. METHODS: Male C57BL/6J mice (8-9 weeks old) were injected with cisplatin or saline for four consecutive days. On Day 5, quadriceps muscles were isolated. Mecasermin (recombinant human IGF-1) or the vehicle control was subcutaneously administered 30 min prior to cisplatin administration. A dietary restriction group achieving weight loss equivalent to that caused by cisplatin administration was used as a second control. C2C12 myotubes were treated with cisplatin with/without recombinant mouse IGF-1. The skeletal muscle protein synthesis/degradation pathway was analysed by histological and biochemical methods. RESULTS: Cisplatin reduced protein level of IGF-1 by about 85% compared with the vehicle group and also reduced IGF-1/PI3K/Akt signalling in skeletal muscle. Under this condition, the protein levels of muscle ring finger protein 1 (MuRF1) and atrophy gene 1 (atrogin-1) were increased in quadriceps muscles (MuRF1; 3.0 ± 0.1 folds, atrogin-1; 3.0 ± 0.3 folds, P < 0.001, respectively). The administration of a combination of cisplatin and IGF-1 significantly suppressed the cisplatin-induced downregulation of IGF-1/PI3K/Akt signalling and upregulation of MuRF1 and atrogin-1 (up to 1.6 ± 0.3 and 1.5 ± 0.4 folds, P < 0.001, respectively), resulting in diminished muscular atrophy. IGF-1 showed similar effects in cisplatin-treated C2C12 myotubes, as well as the quadriceps muscle in mice. CONCLUSIONS: The downregulation of IGF-1 expression in skeletal muscle might be one of the factors playing an important role in the development of cisplatin-induced muscular atrophy. Compensating for this downregulation with exogenous IGF-1 suggests that it could be a therapeutic target for limiting the loss of skeletal muscle mass in cisplatin-induced muscle atrophy.

CT-pathologic correlation of non-calcified atherosclerotic arterial plaques: a study using carotid endarterectomy specimens.

OBJECTIVE: Pathologic features of atherosclerotic plaques on CT are not established. We compared CT values among pathologically confirmed plaque constituents and evaluated their ability to distinguish plaque constituents. METHODS: 50 histopathological images of carotid endarterectomy samples from 10 males and 2 females (age 54-74 years, average 65.9 years) were examined. We compared pre-operative CT [pre-contrast (CT-P), early post-contrast phase (CT-E), delayed post-contrast phase (CT-D)] of lipid-rich necrotic core (NC) and fibrous tissue (F) plaque components with pathological images. The ability of features to differentiate plaque components using several discrimination techniques were compared. RESULTS: CT values of NC and F were 36 ± 13, 45 ± 11 (mean ± standard deviation, Hounsfield unit, HU), 41 ± 17, 69 ± 18, and 44 ± 16, 70 ± 13 in CT-P (p < 0.01), CT-E (p < 0.0001), and CT-D (p < 0.0001), respectively. The threshold, sensitivity, and accuracy for distinguishing NC from F were 44 HU, 74%, and 68%; 55 HU, 85%, and 85%; and 63 HU, 92%, and 84% in CTP, CT-E, and CT-D, respectively. CT-P had lower accuracy than CT-E and CT-D (both p < 0.05), but CT-E and CT-D were similar. CT-E and CT-D yielded 90 and 91% sensitivity and accuracy, respectively in linear discrimination analysis. CONCLUSION: In both pre- and post-contrast CT, CT values were lower in NC than F. Although values overlapped, using two-phase post-contrast CTs improved discrimination ability. ADVANCES IN KNOWLEDGE: Our findings may help to establish computer-aided diagnosis of vulnerable atherosclerotic plaques in future.

Triglyceride deposit cardiomyovasculopathy: a rare cardiovascular disorder.

Triglyceride deposit cardiomyovasculopathy (TGCV) is a phenotype primarily reported in patients carrying genetic mutations in PNPLA2 encoding adipose triglyceride lipase (ATGL) which releases long chain fatty acid (LCFA) as a major energy source by the intracellular TG hydrolysis. These patients suffered from intractable heart failure requiring cardiac transplantation. Moreover, we identified TGCV patients without PNPLA2 mutations based on pathological and clinical studies. We provided the diagnostic criteria, in which TGCV with and without PNPLA2 mutations were designated as primary TGCV (P-TGCV) and idiopathic TGCV (I-TGCV), respectively. We hereby report clinical profiles of TGCV patients. Between 2014 and 2018, 7 P-TGCV and 18 I-TGCV Japanese patients have been registered in the International Registry. Patients with I-TGCV, of which etiologies and causes are not known yet, suffered from adult-onset severe heart disease, including heart failure and coronary artery disease, associated with a marked reduction in ATGL activity and myocardial washout rate of LCFA tracer, as similar to those with P-TGCV. The present first registry-based study showed that TGCV is an intractable, at least at the moment, and heterogeneous cardiovascular disorder.

Sex differences in impact of coronary artery calcification to predict coronary artery disease.

OBJECTIVE: To assess sex-specific differences regarding use of conventional risks and coronary artery calcification (CAC) to detect coronary artery disease (CAD) using coronary CT angiography (CCTA). METHODS: The Nationwide Gender-specific Atherosclerosis Determinants Estimation and Ischemic Cardiovascular Disease Prospective Cohort study is a prospective, multicentre, nationwide cohort study. Candidates with suspected CAD aged 50-74 years enrolled from 2008 to 2012. The outcome was obstructive CAD defined as any stenosis ≥50% by CCTA. We constructed logistic regression models for obstructive CAD adjusted for conventional risks (clinical model) and CAC score. Improvement in discrimination beyond risks was assessed by C-statistic; net reclassification index (NRI) for CAD probability of low (<30%), intermediate (30%-60%) and high (≥60%); and risk stratification capacity. RESULTS: Among 991 patients (456 women, 535 men; 65.2 vs 64.4 years old), women had lower CAC scores (median, 4 vs 60) and lower CAD prevalence (21.7% vs 37.0%) than men. CAC significantly improved model discrimination compared with clinical model in both sexes (0.66-0.79 in women vs 0.61-0.83 in men). The NRI for women was 0.33, which was much lower than that for men (0.71). Adding CAC to clinical model had a larger benefit in terms of moving an additional 43.3% of men to the most determinant categories (high or low risk) compared with -1.4% of women. CONCLUSIONS: The addition of CAC to a prediction model based on conventional variables significantly improved the classification of risk in suspected patients with CAD, with sex differences influencing the predictive ability. TRIAL REGISTRATION NUMBER: UMIN-CTR Clinical Trial: UMIN000001577.

Radiologic-Pathologic Correlation of Primary and Secondary Cardiomyopathies: MR Imaging and Histopathologic Findings in Hearts from Autopsy and Transplantation.

Cardiac magnetic resonance (MR) imaging with late gadolinium enhancement (LGE) is used to detect and assess the myocardial damage seen with a variety of cardiomyopathies. Gadolinium-based contrast material accumulates in the expanded interstitial space of the myocardium. Areas with LGE correspond to replacement fibrosis, fibrofatty change, epithelioid granuloma, inflammatory cell infiltration, cardiomyocyte necrosis, and amyloid deposition-conditions that represent a focal increase in interstitial space. Areas without LGE correspond to interstitial or plexiform fibrosis, mildly degenerated cardiomyocytes, inflammatory cell infiltration, and diffuse amyloid deposition-conditions that represent diffuse increases in interstitial space. LGE MR imaging cannot depict these diffuse changes and does not enable quantitative evaluation of this increased interstitial space because on inversion-recovery MR images, the inversion time is adjusted to null the signal from normal-appearing or the least enhancing regions of the myocardium. Thus, the absence of LGE does not always indicate normal myocardial tissue. The use of current T1 mapping techniques enables one to overcome these drawbacks of LGE imaging, detect diffuse myocardial abnormalities, and perform quantitative analysis of the interstitial space. The authors describe the histopathologic and corresponding cardiac MR imaging findings of hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, cardiac sarcoidosis, giant cell myocarditis, and cardiac amyloidosis-mainly those seen on LGE MR images-as assessed by using whole-heart specimens obtained from autopsy or transplantation. ©RSNA, 2017.

Evaluation of organized thrombus in distal pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension using cone-beam computed tomography.

PURPOSE: Balloon pulmonary angioplasty (BPA) is an emerging treatment for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) patients. However, the approach to use to identify distal thrombi suitable for BPA has not yet been established. The purpose of this work was therefore to evaluate distal chronic thromboembolic lesions for BPA using cone-beam computed tomography (CBCT). MATERIALS AND METHODS: Thirty-two patients (men/women: 9/23) with CTEPH who underwent CBCT before BPA were enrolled. We assessed representative forms of chronic thromboembolic lesions in 94 segmental and/or 208 subsegmental branches according to CBCT and compared the results to the findings of selective angiography during BPA. RESULTS: We classified CTEPH lesions into five subtypes as follows: type 1a (11.1 %), webs; type 1b (14.4 %), web with severe narrowing of the subsegmental artery; type 2, (58.2 %) web and slits; type 3 (2.4 %), slits; and type 4 (13.9 %), pouch defect with incomplete obstruction of subsegmental branches or complete occlusion. In our study, 92.6 % of the CTEPH lesions diagnosed by CBCT were highly consistent with the findings of selective angiography during BPA. CONCLUSION: CBCT clearly revealed and classified distal lesions in CTEPH patients. The CBCT findings for distal lesions were highly consistent with those of selective angiography during BPA. CBCT could be a useful modality to detect target lesions before BPA.

Late gadolinium enhancement in cardiac amyloidosis: attributable both to interstitial amyloid deposition and subendocardial fibrosis caused by ischemia.

Gadolinium contrast agents used for late gadolinium enhancement (LGE) distribute in the extracellular space. Global diffuse myocardial LGE pronounced in the subendocardial layers is common in cardiac amyloidosis. However, the pathophysiological basis of these findings has not been sufficiently explained. A 64-year-old man was admitted to our hospital with leg edema and nocturnal dyspnea. Bence Jones protein was positive in the urine, and an endomyocardial and skin biopsy showed light-chain (AL) amyloidosis. He died of ventricular fibrillation 3 months later. 9 days before death, the patient was examined by cardiac magnetic resonance (CMR) imaging on a 3-T system. We acquired LGE data at 2, 5, 10, and 20 min after the injection of gadolinium contrast agents, with a fixed inversion time of 350 ms. Myocardial LGE developed sequentially. The myocardium was diffusely enhanced at 2 min, except for the subendocardium, but LGE had extended to almost the entire left ventricle at 5 min and predominantly localized to the subendocardial region at 10 and 20 min. An autopsy revealed massive and diffused amyloid deposits in perimyocytes throughout the myocardium. Old and recent ischemic findings, such as replacement fibrosis and coagulative myocyte necrosis, were evident in the subendocardium. In the intramural coronary arteries, mild amyloid deposits were present within the subepicardial to the mid layer of the left ventricle, but no stenotic lesions were evident. However, capillaries were obstructed by amyloid deposits in the subendocardium. In conclusion, the late phase of dynamic LGE (at 10 and 20 min) visualized in the subendocardium corresponded to the interstitial amyloid deposition and subendocardial fibrosis caused by ischemia in our patient.

Comparison of cardiovascular mortality in the Great East Japan and the Great Hanshin-Awaji Earthquakes　- a large-scale data analysis of death certificates.

BACKGROUND: Large earthquakes have been associated with cardiovascular disease (CVD) mortality. In Japan, the 1995 Great Hanshin-Awaji (H-A) Earthquake was an urban-underground-type earthquake, whereas the 2011 Great East Japan (GEJ) Earthquake was an ocean-trench type. In the present study, we examined how these different earthquake types affected CVD mortality. METHODS AND RESULTS: We examined death certificate data from 2008 to 2012 for 131 municipalities in Iwate, Miyagi, and Fukushima prefectures (n=320,348) and from 1992 to 1996 for 220 municipalities in Hyogo, Osaka, and Kyoto prefectures (n=592,670). A Poisson regression model showed significant increases in the monthly numbers of acute myocardial infarction (AMI)-related deaths (incident rate ratio [IRR] GEJ=1.34, P=0.001; IRR of H-A=1.57, P<0.001) and stroke-related deaths (IRR of GEJ=1.42, P<0.001; IRR of H-A=1.33, P<0.001) after the earthquakes. Two months after the earthquakes, AMI deaths remained significant only for H-A (IRR=1.13, P=0.029). When analyzing the standardized mortality ratio (SMR) after the earthquakes using the Cochran-Armitage trend test, seismic intensity was significantly associated with AMI mortality for 2 weeks after both the GEJ (P for trend=0.089) and H-A earthquakes (P for trend=0.005). CONCLUSIONS: Following the GEJ and H-A earthquakes, there was a sharp increase in CVD mortality. The effect of the disaster was sustained for months after the H-A earthquake, but was diminished after the GEJ Earthquake.

CT Findings of Risk Factors for Persistent Type II Endoleak from Inferior Mesenteric Artery to Determine Indicators of Preoperative IMA Embolization.

PURPOSE: To identify the computed tomography (CT) findings of persistent type II endoleak from the inferior mesenteric artery (IMA) which indicate the need for preoperative IMA embolization. MATERIALS AND METHODS: Included were 120 patients (96 males, 49-93 years old, mean: 77.7) who underwent endovascular aortic aneurysm repair (EVAR) between June 2007 and October 2010. The relationship between persistent type II endoleak and CT findings of IMA orifice was examined. RESULTS: CT showed no type II endoleak from IMA in 106 patients (89%; Group N), and transient type II endoleak from IMA in 10 patients (8.3%; Group T). CT showed persistent type II endoleak from IMA in 4 patients (3.3%; Group P) and three of them underwent reintervention. Univariate Cox-Mantel test analysis indicated that stenosis (p = 0.0003) and thrombus (p = 0.043) in IMA orifice were significant factors for persistent type II endoleak. The ratios of patients with proximal IMA more than 2.5 mm diameter in Groups N, Y, and P were 26/106 (24%), 5/10 (50%) and 4/4 (100%), respectively. CONCLUSION: Indicators for embolization of IMA prior to EVAR for the prevention of type II endoleak appear to be: (1) more than 2.5 mm in diameter and (2) no stenosis due to calcification or mural thrombus in IMA orifice.

Organized thrombus in pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension; imaging with cone beam computed tomography.

PURPOSE: To assess the usefulness of cone-beam CT (CBCT) during pulmonary angiography for the evaluation of organized thrombus at segmental or subsegmental arteries in patients with chronic thromboembolic pulmonary hypertension (CTEPH). MATERIALS AND METHODS: The segmental and/or subsegmental pulmonary arteries of 13 patients with CTEPH were evaluated by CBCT. We classified representative forms of organized thrombus into 4 types (type 1: webs, type 2: web and slits, type 3: slits, and type 4: narrowing or complete occlusion), and the distribution and frequency of the organized thrombus were evaluated. The relative detectability of these lesions using CBCT was compared with that in contrast-enhanced CT pulmonary angiography (CTPA). RESULTS: Type 1 lesions were most frequently observed in both segmental (30/65 = 46 %) and subsegmental branches (72/156 = 46 %). Type 2 lesions were relatively less frequent than type 1, but subsegmental branches were frequently involved (29/156 = 19 %). Type 3 lesions observed as a thin flap in 9/156 subsegmental branches (6 %). Comparing with CTPA, all 40 lesions in segmental branches were detectable in CTPA, whereas only 62 lesions among 90 lesions (69 %) in subsegmental branches could be observed by CTPA. CONCLUSION: CBCT is found to be useful for the treatment planning of balloon pulmonary angioplasty distal to segmental arteries.

Prevalence of dural ectasia in Loeys-Dietz syndrome: comparison with Marfan syndrome and normal controls.

BACKGROUND AND PURPOSE: Dural ectasia is well recognized in Marfan syndrome (MFS) as one of the major diagnostic criteria, but the exact prevalence of dural ectasia is still unknown in Loeys-Dietz syndrome (LDS), which is a recently discovered connective tissue disease. In this study, we evaluated the prevalence of dural ectasia in LDS according by using qualitative and quantitative methods and compared our findings with those for with MFS and normal controls. MATERIAL AND METHODS: We retrospectively studied 10 LDS (6 males, 4 females, mean age 36.3 years) and 20 MFS cases (12 males, 8 females, mean age 37.1 years) and 20 controls (12 males, 8 females, mean age 36.1 years) both qualitatively and quantitatively using axial CT images and sagittal multi-planar reconstruction images of the lumbosacral region. For quantitative examination, we adopted two methods: method-1 (anteroposterior dural diameter of S1> L4) and method-2 (ratio of anteroposterior dural diameter/vertebral body diameter>cutoff values). The prevalence of dural ectasia among groups was compared by using Fisher's exact test and the Tukey-Kramer test. RESULTS: In LDS patients, the qualitative method showed 40% of dural ectasia, the quantitative method-1 50%, and the method-2 70%. In MFS patients, the corresponding prevalences were 50%, 75%, and 85%, and in controls, 0%, 0%, and 5%. Both LDS and MFS had a significantly wider dura than controls. CONCLUSIONS: While the prevalence of dural ectasia varied depending on differences in qualitative and quantitative methods, LDS as well as MFS, showed, regardless of method, a higher prevalence of dural ectasia than controls. This finding should help the differentiation of LDS from controls.

Myocardial impairment detected by late gadolinium enhancement in hypertrophic cardiomyopathy: comparison with 99mTc-MIBI/tetrofosmin and 123I-BMIPP SPECT.

PURPOSE: Myocardial fibrosis is considered to be an important factor in myocardial dysfunction and sudden cardiac death in hypertrophic cardiomyopathy (HCM). The purpose of this study was to compare myocardial fibrosis detected by late gadolinium enhancement (LGE) on cardiac MRI with myocardial perfusion and fatty acid metabolism assessed by single photon emission computed tomography in HCM. MATERIALS AND METHODS: We retrospectively evaluated 20 consecutive HCM patients (female, 7; mean age, 53.4 years) who underwent LGE, technetium-99m methoxyisobutylisonitrile/tetrofosmin (99mTc-MIBI/tetrofosmin), and iodine-123 beta-methyl-iodophenylpentadecanoic acid (123I-BMIPP) imaging. We calculated the myocardium-to-lumen signal ratio (M/L) for LGE in 17 segments based on the American Heart Association statement. Scoring of 99mTc-MIBI/tetrofosmin (PI) and 123I-BMIPP (BM) was performed for each segment using a 5-point scale (0, normal; 4, highly decreased). RESULTS: Nineteen of 20 patients (95%) and 153 of 340 segments (45%) showed LGE. M/Ls were 0.42±0.16, 0.55±0.17, and 0.65±0.24 in PI0/BM0, PI0/BM1-4 and PI1-4/BM1-4, respectively. All M/Ls were significantly higher than that of a normal control (0.34±0.14) (p<0.001). CONCLUSIONS: Myocardial fibrosis in HCM can occur despite normal perfusion and fatty acid metabolism, and is more strongly associated with disorders of fatty acid metabolism than with perfusion abnormalities. M/L may be a useful indicator of disease severity.

[Heart transplantation and ventricular assit systems].

Since the organ transplantation law was passed, we performed 50 heart transplantation at National Cerebral and Cardiovascular Center. Of those, 2 patients have been doing well over 13 years and 10 years survival rate was 93.4%.During those years, we performed 139 applications of left ventricular assist systems( LVAS). Initially, extracorporeal LVASs had been used. Now, 2 implantable LVAS were approved by medical insurance as bridge to transplant in 2011. Now, our 1st option as bridge to transplantation(BTT) is implantable LVAS.

Lung perfused blood volume images with dual-energy computed tomography for chronic thromboembolic pulmonary hypertension: correlation to scintigraphy with single-photon emission computed tomography.

OBJECTIVE: The purpose of this study was to evaluate the feasibility and diagnostic utility of lung perfused blood volume (LPBV) images generated by dual-energy computed tomography (CT), as compared with pulmonary perfusion scintigraphy, for patients with chronic thromboembolic pulmonary hypertension. METHODS: Patients (n = 51) with chronic pulmonary thromboembolic embolism were examined by dual-source CT in dual-energy mode. Lung perfused blood volume images were generated by analysis of the iodine content of the lung parenchyma using dual-energy data. Pulmonary perfusion defects were evaluated on a segment-by-segment basis in images obtained by both LPBV and pulmonary scintigraphy. Findings suggestive of chronic thromboembolic pulmonary hypertension in CT pulmonary angiography were analyzed. RESULTS: All examinations were acquired without complications, and the contrast enhancement of the pulmonary artery was sufficient for diagnosis of vascular thromboses. In the LPBV images, in 76 (8.3%) of 918 segments, it was difficult to assess perfusion because of artifacts. The agreement between the 2 modalities was good (κ = 0.70). The sensitivity of LPBV in detecting perfusion defects was 96%; the specificity was 76%; the positive predictive value was 94%, and the negative predictive value was 29%. CONCLUSIONS: Lung perfused blood volume imaging by dual-energy CT is feasible for the evaluation of pulmonary perfusion and is comparable to pulmonary scintigraphy. It is possible to evaluate vessels and pulmonary perfusion with CT pulmonary angiography and LPBV images and to assess pulmonary perfusion more definitively in diagnosing chronic pulmonary thromboembolic embolism.

High-intensity signals in carotid plaques on T1-weighted magnetic resonance imaging predict coronary events in patients with coronary artery disease.

OBJECTIVES: The purpose of this study was to determine whether high-intensity carotid plaques visualized by a noncontrast T1-weighted imaging technique, magnetization-prepared rapid acquisition with gradient echo (MPRAGE), predict future coronary events in patients with clinically stable coronary artery disease (CAD). BACKGROUND: Coronary plaque vulnerability to rupture can be assessed by examining for the presence of atherosclerosis and measuring intima media thickness (IMT) in surrogate vessels such as the carotid arteries. We previously showed that MPRAGE successfully identifies vulnerable carotid plaques as high-intensity signals. It remains unclear, however, if the presence of carotid high-intensity plaques (HIP) is associated with an increased risk of coronary events. METHODS: We examined the signal intensity of carotid plaques in 217 patients with clinically stable CAD using MPRAGE with magnetic resonance imaging and measured IMT with ultrasonography. A carotid HIP was defined as a signal >200% that of the adjacent muscle. All patients were divided into 2 groups according to the presence or absence of HIP, namely, the HIP group (n = 116) and the non-HIP group (n = 101), and were followed up for as long as 72 months. RESULTS: The presence of HIP was significantly associated with cardiac events compared to the non-HIP group (log-rank p < 0.0001). Furthermore, multivariate Cox regression analysis identified the presence of HIP as the strongest independent predictor of cardiac events (hazard ratio: 3.15; 95% confidence interval: 1.93 to 5.58, p < 0.0001) compared with IMT (hazard ratio: 1.62, 95% confidence interval: 0.97 to 2.44, p = 0.055) and other coronary risk factors. CONCLUSIONS: Characterization of carotid plaques using magnetic resonance imaging with MPRAGE provides more clinically relevant information for the risk assessment of CAD patients than IMT.

Dynamic late gadolinium enhancement simply quantified using myocardium to lumen signal ratio: normal range of ratio and diffuse abnormal enhancement of cardiac amyloidosis.

PURPOSE: To detect abnormal myocardial tissue in patients with diffuse myocardial disease, we propose a simple technique of late gadolinium enhancement (LGE) using routine myocardial imaging modalities. MATERIALS AND METHODS: We retrospectively reviewed LGE images from 51 patients with normal myocardium and 10 patients with pathologically proven cardiac amyloidosis (CA). We obtained sequential LGE images from patients at 2, 5, 10, and 20 minutes after injection of Gd-DTPA (0.15 mmol/kg) with a fixed inversion time of 300 msec. We evaluated the signal intensity ratio of the myocardium to the left ventricular lumen (M/L) in one long and two short axial sections within 463 and 120 segments of normal myocardium and CA, respectively. Visually unenhanced and enhanced regions of myocardium were evaluated in each segment of patients with CA. RESULTS: Among normal myocardium, M/L (means ± standard deviation; SD) was stable with time (2, 5, 10, and 20 min: 0.34 ± 0.03, 0.31 ± 0.05, 0.34 ± 0.07, and 0.42 ± 0.11, respectively). The calculated M/L of unenhanced (0.60 ± 0.20, 0.68 ± 0.19, 0.76 ± 0.20, and 1.09 ± 0.25, respectively) and enhanced myocardium (0.77 ± 0.27, 0.99 ± 0.29, 1.20 ± 0.40, and 1.45 ± 0.54, respectively) in patients with CA was significantly greater than that seen for the normal myocardium at each time and increased over time. CONCLUSION: In patients with CA, diffuse myocardial abnormalities can be demonstrated using M/L, and this technique may be useful for the characterization of other myocardial diseases.

Assessment of calcified carotid plaque volume: comparison of contrast-enhanced dual-energy CT angiography and native single-energy CT.

OBJECTIVE: The purpose of this study was to compare estimates of calcified plaque volume in the carotid arteries based on contrast-enhanced dual-energy CT angiograms with volume estimates based on native CT scans. CONCLUSION: Detection of hard plaque in the carotid arteries with dual-energy CT angiography was successful in all patients. Estimates of calcified plaque volume based on dual-energy CT angiograms correlated well with those based on native single-energy CT scans but at a lower attenuation threshold (130 HU as opposed to 180 HU) were underestimates compared with those on the native CT scans.

High prevalence of vertebral artery tortuosity of Loeys-Dietz syndrome in comparison with Marfan syndrome.

PURPOSE: Loeys-Dietz syndrome (LDS) is a connective tissue disease caused by mutations in the genes encoding the transforming growth factor-beta receptor (TGFBR). LDS is associated with aneurysms or dissections of the aorta similar to Marfan syndrome (MFS) as well as arterial tortuosity and aneurysms in the peripheral arteries. The purpose of this study was to evaluate the arterial diseases of LDS to differentiate it from MFS. MATERIALS AND METHODS: A total of 10 LDS patients with an identified mutation in TGFBR (6 male, 4 female; mean age 36.3 years) and 20 MFS patients with an identified mutation in fibrilin-1 who were age- and sex-matched to the LDS subjects (12 male, 8 female; mean age 37.1 years) were reviewed. The prevalence of vertebral arterial tortuosity (VAT) and peripheral aneurysm (PAN) was studied using computed tomography angiography. RESULTS: In all, 9 of the 10 LDS patients had VAT, and five PANs were observed in 3 patients. In contrast, 8 (40%) of the MFS patients had VAT, and 1 patient had a PAN. LDS had a higher prevalence of VAT (P = 0.017) by Fisher's exact test. CONCLUSION: The VAT was highly prevalent among LDS patients. Thus, the presence of VAT has the potential to differentiate LDS from MFS.