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Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation thrice over a period of 2 years. However, the bleeding was not controlled. When the patient finally visited the Emergency Department, the hemoglobin level was 4.4 g/dL. Transhepatic intrajugular portosystemic shunt was unsuccessful. Subsequently, the patient underwent percutaneous transhepatic variceal obliteration. Twenty hours after this procedure, the patient complained of aphasia, dizziness, headache, and general weakness. Six hours later, the patient became drowsy and unresponsive to painful stimuli. Lipiodol particles used to embolize the coronary and posterior gastric veins might have passed into the systemic arterial circulation, and they were found to be lodged in the brain, kidney, lung, and stomach. There was no abnormality of the portal vein on portal venography, and blood flow to the azygos vein through the paravertebral and hemiazygos systems was found to drain to the systemic circulation on coronary venography. Contrast echocardiography showed no pulmonary arteriovenous fistula. Symptoms improved with conservative management, and the esophageal varices were found to have improved on esophagogastroduodenoscopy.

Thiopurine S-methyltransferase (TPMT) methylates purine analogues, showing TPMT activity in inverse relation to concentrations of active metabolites such as 6-thioguanine nucleotide (6-TGN). With conventional dosing of thiopurines, patients with homozygous variant TPMT alleles consistently suffer from severe myelosuppression. Here, we report a patient with TPMT*3C/*3C who managed successfully with monitoring of thiopurine metabolites. The patient was an 18-year-old male diagnosed with Crohn's disease. The standard dose of azathioprine (AZA) (1.8 mg/kg/day) with mesalazine (55.6 mg/kg/day) was prescribed. Two weeks after starting AZA treatment, the patient developed leukopenia. The DNA sequence analysis of TPMT identified a homozygous missense variation (NM_000367.2: c.719A>G; p.Tyr240Cys), TPMT*3C/*3C. He was treated with adjusted doses of azathioprine (0.1-0.2 mg/kg/day) and his metabolites were closely monitored. Leukopenia did not reoccur during the follow-up period of 24 months. To our knowledge, this is the first case of a patient homozygous for TPMT*3C successfully treated with azathioprine in Korea. While a TPMT genotyping test may be helpful to determine a safe starting dose, it may not completely prevent myelosuppression. Monitoring metabolites as well as routine laboratory tests can contribute to assessing drug metabolism and optimizing drug dosing with minimized drug-induced toxicity.

Purpose: This study was aimed to evaluate the frequency and course of adverse events associated with azathioprine treatment in Korean pediatric patients with inflammatory bowel disease., Methods: Total of 174 pediatric patients (age range, 1 to 19 years) with inflammatory bowel disease who received azathioprine in order to maintain remission at Samsung Medical Center (Seoul, Korea) from January 2002 through December 2012 were included in this study. Medical records of these subjects were retrospectively reviewed regarding the development of adverse events associated with azathioprine treatment., Results: Ninety-eight patients (56.3%) of 174 patients experienced 136 episodes of adverse events, requiring dose reduction in 31 patients (17.8%), and discontinuation in 18 patients (10.3%). The mean dose of azathioprine that had been initially administered was 1.32±0.42 mg/kg/day. Among the adverse reactions, bone marrow suppression developed in 47 patients (27.0%), requiring dose reduction in 22 patients (12.6%) and discontinuation in 8 patients (4.6%). Other adverse events that occurred were gastrointestinal disturbance (15.5%), hair loss (12.1%), pancreatitis (7.5%), arthralgia (6.9%), hepatotoxicity (2.9%), skin rash/allergic reactions (2.9%), headache/dizziness (2.3%), sepsis (0.6%), and oral mucositis (0.6%)., Conclusion: Bone marrow suppression, especially leukopenia was most commonly associated with azathioprine treatment in Korean pediatric inflammatory bowel disease patients. Close observation for possible adverse events is required in this population with inflammatory bowel diseases who are under treatment with azathioprine.

Background: Severe aortic stenosis (AS) patients with late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) or left ventricular (LV) systolic dysfunction are known to have worse outcome. We aimed to investigate whether LGE on CMR would be useful in early detection of subclinical LV structural and functional derangements in AS patients., Methods: 118 patients with moderate to severe AS were prospectively enrolled. Echocardiography and CMR images were taken and the patients were divided into groups according to the presence/absence of LGE and of LV systolic dysfunction (LV ejection fraction (EF) <50%). The stiffness of LV was calculated based on Doppler and CMR measurements., Results: Patients were grouped into either group 1, no LGE and normal LVEF, group 2, LGE but normal LVEF and group 3, LGE with depressed LVEF. There was a significant trend towards increasing LV volumes, worsening of LV diastolic function (E/e', diastolic elastance), systolic function (end-systolic elastance) and LV hypertrophy between the three groups, which coincided with worsening functional capacity (all p-value < 0.001 for trend). Also, significant differences in the above parameters were noted between group 1 and 2 (E/e', 14.6 ± 4.3 (mean ± standard deviation) in group 1 vs. 18.2 ± 9.4 in group 2; end-systolic elastance, 3.24 ± 2.31 in group 1 vs. 2.38 ± 1.16 in group 2, all p-value < 0.05). The amount of myocardial fibrosis on CMR correlated with parameters of diastolic (diastolic elastance, Spearman's ρ = 0.256, p-value = 0.005) and systolic function (end-systolic elastance, Spearman's ρ = -0.359, p-value < 0.001)., Conclusions: These findings demonstrate the usefulness of CMR for early detection of subclinical LV structural and functional deterioration in AS patients.

Dehydroascorbate reductase (DHAR, EC 1.8.5.1) maintains redox pools of ascorbate (AsA) by recycling oxidized AsA to reduced AsA. To investigate whether DHAR affects rice yield under normal environmental conditions, cDNA-encoding DHAR (OsDHAR1) was isolated from rice and used to develop OsDHAR1-overexpressing transgenic rice plants, under the regulation of a maize ubiquitin promoter. Incorporation and expression of the transgene in transgenic rice plants was confirmed by genomic polymerase chain reaction (PCR), semi-quantitative reverse transcription PCR (RT-PCR), western blot, and enzyme activity. The expression levels were at least twofold higher in transgenic (TG) rice plants than in control wild-type (WT) rice plants. In addition, OsDHAR1-overexpression in seven-independent homologous transgenic plants, as compared to WT plants, increased photosynthetic capacity and antioxidant enzyme activities under paddy field conditions, which led to an improved AsA pool and redox homeostasis. Furthermore, OsDHAR1 overexpression significantly improved grain yield and biomass due to the increase of culm and root weights and to enhance panicle and spikelet numbers in the same seven independent TG rice plants during the farming season (2010 and 2011) in South Korea. The OsDHAR protein contained the redox-active site (Cys20), as well as the conserved GSH-binding region, GSH-binding motif, glutathione-S-transferase (GST) N-terminal domain, C-terminal domain interface, and GST C-terminal domain. Therefore, our results indicate that OsDHAR1 overexpression, capable of functioning in AsA recycling, and protein folding increases environmental adaptation to paddy field conditions by the improving AsA pool and redox homeostasis, which enhances rice grain yield and biomass.

The aim of this study was to determine the normal values for aortic pulse wave velocity (PWV) and distensibility using cine and phase contrast cardiac magnetic resonance imaging (CMRI) in patients without cardiovascular risk factors. PWV and distensibility are indispensible predictors of global and regional cardiovascular risk. Regional heterogeneity in aortic stiffness plays an important role in the pathogenesis of cardiovascular disease. Contrary to global estimates of aortic PWV that are commonly measured with tonometry, CMRI has emerged as an important method for estimating regional PWV and distensibility. A total of 124 Korean patients, aged 20-79 years and free of cardiovascular risk factors, were categorized by age decade. Using cine and phase contrast sequences, the cross-sectional area for distensibility and average blood flow were measured at four aortic levels: the ascending, upper descending thoracic, lower thoracic and abdominal aorta. Regional PWV was determined in four aortic segments: proximal, descending thoracic, abdominal aorta and across the entire aorta. Distensibility at the four levels of the aorta from the ascending to distal (4.4 ± 2.5, 4.0 ± 1.6, 5.2 ± 1.9, and 3.3 ± 1.7 × 10(-3) mm/Hg, respectively) was higher in women (P < 0.001) and decreased with age. The regional PWV was highest in the descending thoracic aorta and increased with age. The present study is the first to show the heterogeneity in aortic PWV and distensibility, as well to provide normal values for these parameters using CMRI in an Asian sample.

Azathioprine is the most common drug used to maintain clinical remission in inflammatory bowel disease. This drug is also important as a steroid-sparing agent in steroid-dependent and chronically active inflammatory bowel disease. Nevertheless, many questions remain concerning the optimal treatment regimens of azathioprine. The dose of azathioprine has to be reduced or the therapy has to be discontinued frequently because of drug-induced toxicity. In this review, we discuss monitoring of thiopurines, adverse events, malignant complications and how to use azathioprine safely and usefully.

We conducted a study to clarify the incidence, clinical course, and risk factors of de novo allergies after liver transplantation. Ninety-three patients who had been followed longer than one yr and who had no previous allergy history were included. Forty-two patients (45.2%) developed de novo allergy. Of them, food allergy developed in 35 (37.6%). Respiratory allergy was observed in three (3.2%), and a patient (1.1%) had drug allergy. Fifty-two (55.9%) of the 93 patients developed eosinophilia. The median age of patients with de novo allergy was 15 months (IR 11.3-20 months). De novo allergy developed five months after liver transplantation (IR 2.3-9.5 months) and lasted for 16 months (IR 8-34.5 months). Younger age at liver transplantation displayed statistically significant differences in development of allergy between allergy and non-allergy groups. Twenty-nine (69.0%) patients improved from allergy during the follow-up period. No patient with de novo gastrointestinal allergy progressed to any respiratory allergy such as asthma. Older age at transplantation, EBV non-risk, and CMV non-risk had statistical significance in allergy improvement. Younger age at transplant predisposes to the development of allergy, while improvement of allergy is achieved more in older age., (© 2013 John Wiley & Sons A/S.)

Crohn's disease is a chronic relapsing, granulomatous, and inflammatory bowel disorder. Variable extra-intestinal manifestations may occur, which include erythema nodosum, erythema multiforme, pyoderma gangrenosum, and other non-specific skin lesions. Here, we present a case of metastatic penile Crohn's disease without scrotal involvement, which was initially approached as a balanoposthitis with penile cellulitis, and completely treated with infliximab infusion in a short time., (Copyright © 2012 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.)

Various environmental stresses induce reactive oxygen species (ROS), causing deleterious effects on plant cells. Glutathione (GSH), a critical antioxidant, is used to combat ROS. GSH is produced by γ-glutamylcysteine synthetase (γ-ECS) and glutathione synthetase (GS). To evaluate the functional roles of the Oryza sativa L. Japonica cv. Ilmi ECS (OsECS) gene, we generated transgenic rice plants overexpressing OsECS under the control of an inducible promoter (Rab21). When grown under saline conditions (100mM) for 4 weeks, 2-independent transgenic (TGR1 and TGR2) rice plants remained bright green in comparison to control wild-type (WT) rice plants. TGR1 and TGR2 rice plants also showed a higher GSH/GSSG ratio than did WT rice plants in the presence of 100mM NaCl, which led to enhanced redox homeostasis. TGR1 and TGR2 rice plants also showed lower ion leakage and higher chlorophyll-fluorescence when exposed to 10μM methyl viologen (MV). Furthermore, the TGR1 and TGR2 rice seeds had approximately 1.5-fold higher germination rates in the presence of 200mM salt. Under paddy field conditions, OsECS-overexpression in transgenic rice plants increased rice grain yield (TGW) and improved biomass. Overall, our results show that OsECS overexpression in transgenic rice increases tolerance and germination rate in the presence of abiotic stress by improving redox homeostasis via an enhanced GSH pool. Our findings suggest that increases in grain yield by OsECS overexpression could improve crop yields under natural environmental conditions., (Copyright © 2013 Elsevier GmbH. All rights reserved.)

Children have a natural tendency to explore objects with their mouths; this can result in the swallowing of foreign objects. Most ingested foreign bodies pass uneventfully through the gastrointestinal tract. However, some foreign bodies cause obstruction or perforation of the gastrointestinal tract, requiring surgical intervention. Perforation of the gastrointestinal tract may be associated with considerable morbidity and mortality. The most common sites of intestinal foreign body perforation are the ileocecal and rectosigmoid regions. Foreign body perforation of the duodenum is relatively uncommon. We report the first Korean case of duodenal perforation by an ingested 8-cm lollipop stick. Lollipops are popular with the children and fairly accessible to them, as most parents are not aware of their potential harm. Pediatric clinicians should be aware of the risks associated with lollipop stick ingestion. Our report also describes the feasibility and safety of laparoscopic diagnosis and management of pediatric patients with peritonitis induced by the ingestion of foreign bodies.

Ice-binding proteins (IBPs) can bind to the ice crystal and inhibit its growth. Because this property of IBPs can increase the freeze-thaw survival of cells, IBPs have attracted the attention from industries for their potential use in biotechnological applications. However, their use was largely hampered by the lack of the large-scale recombinant production system. In this study, the codon-optimized IBP from Leucosporidium sp. (LeIBP) was constructed and subjected to high-level expression in methylotrophic Pichia pastoris system. In a laboratory-scale fermentation (7 L), the optimal induction temperature and pH were determined to be 25 °C and 6.0, respectively. Further, employing glycerol fed-batch phase prior to methanol induction phase enhanced the production of recombinant LelBP (rLeIBP) by ∼100 mg/l. The total amount of secreted proteins at these conditions (25 °C, pH 6.0, and glycerol fed-batch phase) was ∼443 mg/l, 60 % of which was rLeIBP, yielding ∼272 mg/l. In the pilot-scale fermentation (700 L) under the same conditions, the yield of rLeIBP was 300 mg/l. To our best knowledge, this result reports the highest production yield of the recombinant IBP. More importantly, the rLeIBP secreted into culture media was stable and active for 6 days of fermentation. The thermal hysteresis (TH) activity of rLeIBP was about 0.42 °C, which is almost the same to those reported previously. The availability of large quantities of rLeIBP may accelerate further application studies.

We sought to determine which contrast-enhanced magnetic resonance imaging (CE-MRI) parameter is the best predictor for left ventricular (LV) remodeling and clinical outcomes after ST-segment elevation myocardial infarction (STEMI). In 135 patients undergoing primary percutaneous coronary intervention (PCI) for STEMI, CE-MRI was performed at a median of 7 days after PCI. Echocardiography was performed soon after PCI and at a follow-up visit. LV remodeling was defined as an increase in end-diastolic volume index ≥20 % on follow-up echocardiography. Several CE-MRI parameters such as infarct size, transmurality, microvascular obstruction (MVO), and hemorrhagic infarction were tested using a 17-myocardial segment model. Optimal cut-off values were derived from receiver-operating characteristic curve (ROC) analysis. Twenty-eight patients (21 %) demonstrated LV remodeling. Although the addition of transmural necrotic segment count, infarct size, and MVO segment count to clinical models improved the prediction of LV remodeling in multivariable regression analysis, transmural necrotic segment count had better incremental predictive value than other CE-MRI parameters. The aggregate consideration of infarct size (cut-off ≥25 %), transmural necrotic segment count (≥5), and MVO segment count (≥2) yielded better diagnostic performance than each of the individual parameters in ROC analysis (P < 0.01). In Kaplan-Meier curve analysis, patients with transmural necrotic segment counts ≥5 had a higher incidence of major adverse cardiac event than did those without. The transmural necrotic segment count is the most important predictor of LV remodeling and clinical outcomes. The combination of CE-MRI parameters including infarct size, transmural necrotic segment count, and MVO segment count appeared to increase reliability for predicting LV remodeling.

The aim of this study was to compare the radiation dose and image quality of different adenosine-stress dynamic myocardial perfusion CT protocols using a 128-slice dual-source computed tomography (DSCT) scanner. We included 330 consecutive patients with suspected coronary artery disease. Protocols employed the following dynamic scan parameters: protocol I, a 30-s scan with a fixed tube current (FTC, n = 172); protocol II, a 30-s scan using an automatic tube current modulation (ATCM) technique (n = 108); protocol III, a 14-s scan using an ATCM (n = 50). To determine the scan interval for protocol III, we analyzed time-attenuation curves of 26 patients with myocardial perfusion who had been scanned using protocol I or II. The maximum attenuation difference between normal and abnormal myocardium occurred at 18.0 s to 30.3 s after initiation of contrast injection. Myocardial perfusion images of FTC and ATCM were of diagnostic image quality based on visual analysis. The mean radiation dose associated with protocols I, II, and III was 12.1 ± 1.6 mSv, 7.7 ± 2.5 mSv, and 3.8 ± 1.3 mSv, respectively (p < 0.01). Use of a dose-modulation technique and a 14-s scan duration for adenosine-stress CT enables significant dose reduction while maintaining diagnostic image quality.

Background: Left ventricular (LV) mass is an important prognostic indicator in hypertrophic cardiomyopathy. Although LV mass can be easily calculated using conventional echocardiography, it is based on geometric assumptions and has inherent limitations in asymmetric left ventricles. Real-time three-dimensional echocardiographic (RT3DE) imaging with single-beat capture provides an opportunity for the accurate estimation of LV mass. The aim of this study was to validate this new technique for LV mass measurement in patients with hypertrophic cardiomyopathy., Methods: Sixty-nine patients with adequate two-dimensional (2D) and three-dimensional echocardiographic image quality underwent cardiac magnetic resonance (CMR) imaging and echocardiography on the same day. Real-time three-dimensional echocardiographic images were acquired using an Acuson SC2000 system, and CMR-determined LV mass was considered the reference standard. Left ventricular mass was derived using the formula of the American Society of Echocardiography (M-mode mass), the 2D-based truncated ellipsoid method (2D mass), and the RT3DE technique (RT3DE mass)., Results: The mean time for RT3DE analysis was 5.85 ± 1.81 min. Intraclass correlation analysis showed a close relationship between RT3DE and CMR LV mass (r = 0.86, P < .0001). However, LV mass by the M-mode or 2D technique showed a smaller intraclass correlation coefficient compared with CMR-determined mass (r = 0.48, P = .01, and r = 0.71, P < .001, respectively). Bland-Altman analysis showed reasonable limits of agreement between LV mass by RT3DE imaging and by CMR, with a smaller positive bias (19.5 g [9.1%]) compared with that by the M-mode and 2D methods (-35.1 g [-20.2%] and 30.6 g [17.6%], respectively)., Conclusions: RT3DE measurement of LV mass using the single-beat capture technique is practical and more accurate than 2D or M-mode LV mass in patients with hypertrophic cardiomyopathy., (Copyright © 2013 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Pediatric liver transplantation is the standard of care for treatment of liver failure in children. The aim of this study was to identify the characteristics of pediatric liver transplantation in centers located in Korea and determine factors that influence outcomes. This retrospective study was performed using data from between 1988 and 2010 and included all recipients 18 yr old and younger who underwent pediatric liver transplantation in Korea during that period. Our data sources were hospital medical records and the outcome measure was overall patient survival. Univariate and multivariate statistical analyses were undertaken using the Cox proportional hazards model. Five hundred and thirty-four pediatric liver transplantations were performed in 502 children. Median age and average pediatric end-stage liver disease (PELD) score were 20 months and 18 point, respectively. Biliary atresia (57.7%, 308/534) was the most common cause of liver disease. Eighty-two (15.3%) were deceased donor liver transplantations and 454 (84.7%) were living donor liver transplantations. Retransplantation was performed in 32 cases (6%). Overall, 1-, 5-, and 10-yr patient survival rates were 87.8%, 82.2%, and 78.1%, respectively. In multivariate analysis, independent significant predictors of poor patient survival were chronic rejection and retransplantation. This study presents the epidemiologic data for nearly all pediatric liver transplantation in Korea and shows that the independent prognostic factors in patient survival are chronic rejection and retransplantation.

This retrospective-medical-record review was conducted to evaluate effect of infliximab therapy, particularly with a top-down strategy, on the nutritional parameters of children with Crohns disease (CD). 42 patients who were diagnosed with Crohns disease at the Pediatric Gastroenterology center of a tertiary care teaching hospital and achieved remission at two months and one year after beginning of treatment were divided into four subgroups according to the treatment regimen; azathioprine group (n = 11), steroid group (n = 11), infliximab top-down group (n = 11) and step-up group (n = 9). Weight, height, and serum albumin were measured at diagnosis, and then at two months and one year after the initiation of treatment. At 2 months, the Z score increment for weight was highest in the steroid group, followed by the top-down, step-up, and azathioprine groups. At one year, the Z score increment was highest in top-down group, followed by steroid, azathioprine, and step-up group. There were no significant differences between the four groups in Z score increment for height and serum albumin during the study period. The top-down infliximab treatment resulted in superior outcome for weight gain, compared to the step-up therapy and other treatment regimens.

Purpose: To investigate the efficacy of early infliximab use and to follow the progress of pediatric cases of Crohn's disease for 3 years., Methods: We reviewed the medical records of 28 pediatric patients who had been treated with infliximab for Crohn's disease. Eighteen patients (the 'top-down' group) received infliximab and azathioprine for induction and maintenance therapy for the first year, and then were treated with azathioprine for 2 additional years. Ten patients who were refractory to conventional therapy were categorized in the 'step-up' group. All patients were followed for at least 36 months. Treatment efficacy was assessed by the relapse rate using the pediatric Crohn's disease activity index (PCDAI) score in each group at 12, 24, and 36 months. Blood samples were available from 10 patients, and were used to assess antibody to infliximab (ATI)., Results: The relapse rate in 'top-down' group was lower than that in 'step-up' group at 1, 2, and 3 years. But, just the relapse rate at the 2 years was significantly different. At 3 years, the relapse rate according to different characteristic variables (sex, age at diagnosis, involvement, PCDAI at diagnosis) was not significantly different. Only one patient treated with infliximab had an adverse event, consisting of dyspnea and tachycardia. ATI was not detected in the blood samples from 10 patients., Conclusion: Early induction with infliximab at diagnosis ('top-down' therapy) is effective for reducing the relapse rate compared to conventional therapies in pediatric Crohn's disease possibly for up to 3 years.

The clinical utility of cardiac magnetic resonance imaging (CMR) is growing and is being used predominantly as a means of measuring ventricular function. The normal reference range of ventricular function may vary based on age, sex and ethnicity. At present, most CMR reference values for healthy individuals have been reported from Western countries. The intent of this study was to investigate the normal CMR reference range for left ventricular (LV) and right ventricular (RV) parameters in healthy Koreans. Healthy volunteers between the ages of 20-70 years without any history of cardiovascular disease or associated risk factors were prospectively recruited to be a part of this study. A total of 124 patients were recruited for this study. Steady-state free precession pulse sequences were used to obtain the cine images for LV and RV volume analysis. All parameters were analyzed based on age and gender, and normalized to body surface area (BSA). LV volume, mass and cardiac output were significantly greater in males than in females. However, all of these parameters which are associated with BSA and gender differences disappeared when corrected for BSA. RV volume was less in females even after the data was normalized for BSA. LV and RV volumes normalized for BSA gradually decreased with greater age, whereas the ejection fraction increased with age, thus maintaining the stroke volume index and cardiac index. LV and RV volumes, mass and function values for a healthy population largely depend on BSA and should be evaluated after normalization by BSA. LV parameters show no difference based on gender, but RV volume is less in the female. Greater age is associated with less ventricular volume, suggesting the possibility of volume sensitivity in the elderly.

Aims: We validated and compared transluminal attenuation gradient (TAG) and corrected coronary opacification (CCO) of coronary computed tomography angiography (CCTA) with invasively measured fractional flow reserve (FFR)., Background: One of the major limitations of CCTA is the discrepancy between angiographical stenosis and ischaemia-causing stenosis. Recently two new CCTA analysis methods, TAG and CCO, have been attempted to overcome this limitation but without physiological validation., Methods and Results: We measured TAG and CCO of 97 major epicardial coronary arteries from 63 patients who underwent CCTA and followed by invasive coronary angiography and FFR. Diagnostic performance of TAG and CCO was assessed using FFR <0.80 as the reference standard. The overall diagnostic performance of TAG and CCO on a per-vessel basis was moderate and similar (c-statistic = 0.696 vs. 0.637, P = 0.29). The sensitivity, specificity, positive, and negative predictive values of TAG cut-off ≤-0.654 for FFR <0.80 were 47.5, 91.2, 79.2, and 71.2%, and those of CCO cut-off >0.063 were 65.0, 61.4, 54.2, and 71.4%. TAG showed an incremental value to the diagnostic performance of CCTA but CCO did not (c-statistic =0.726 vs. 0.809, P = 0.025; c-statistic =0.726 vs. 0.784, P = 0.09). In net reclassification improvement (NRI) analysis, addition of TAG to CCTA did not result in significant reclassification (NRI = 1.0%, P = 0.41) and addition of CCO to CCTA resulted in negative reclassification (NRI = -9.3%, P = 0.036)., Conclusion: Intracoronary attenuation-based CCTA analyses, TAG and CCO, showed moderate correlation with physiological coronary artery stenosis. The incremental value of TAG or CCO to the evaluation of haemodynamically stenosis by CCTA seemed to be limited.

Hypertrophic cardiomyopathy (HCM) is a disease that typically has heterogeneous hypertrophy and dysfunction of the myocardium. Cardiac magnetic resonance imaging (CMR) can be used to accurately assess ventricular wall thickness and regional fibrosis. We investigated the effects of hypertrophy and fibrosis on the heterogeneity of regional and global myocardial function in HCM. Forty patients who were diagnosed with HCM were consecutively enrolled. Echocardiography and CMR with delayed hyper-enhancement imaging (DHE) was performed for each patient. Left ventricular (LV) regional and global longitudinal strain (SL(R) and SL(G)) were obtained by two-dimensional speckle tracking method on echocardiography. With CMR, regional myocardial wall thickness was measured, and the amount of DHE was calculated semi-quantitatively in each segment. Overall, 720 segments were analyzed. SL(R) was significantly decreased in the hypertrophied segments (thickness > 11 mm) and segments with DHE (P < 0.001). SL(R) was correlated with myocardial wall thickness (r = 0.47, P = 0.001) and amount of regional DHE (r = 0.39, P < 0.001). On multivariate analysis, regional LV wall thickness and amount of DHE were the only independent determinants of SL(R). SL(G) was associated with LV diastolic functional parameters in echocardiography, total DHE volume, and LV mass index. Total DHE volume and LV mass index were independent determinants of SL(G) on multivariate analysis. The extent of regional myocardial fibrosis is associated with regional myocardial function independently of morphological changes of the myocardium, and the correlation extended to global LV function. In this context, DHE may be a useful parameter to discover early myocardial dysfunction independently of LV hypertrophy.

Aim: To analyse the multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) findings in patients with atresia of the coronary sinus orifice (CSA)., Materials and Methods: MDCT findings of 15 consecutive adult patients with CSAs were retrospectively analysed. The patients underwent contrast-enhanced electrocardiography-gated MDCT (n = 13) or both CT and MRI (n = 2)., Results: The mean size of the coronary sinus (CS) was 14.2 mm (range 5.5-24 mm) and 11 patients (73.3%) showed CS dilatation (diameter ≥12 mm). The mean length of the atretic CS segment was 2.9 mm (range 0-8 mm). Different forms of venous collateral pathways were observed in the CSA patients. Nine (60%) of the 15 CSA patients had communication between the right atrium (RA; n = 6) or LA (n = 5) and CS via intraseptal veins; six patients (40%) had persistent left superior caval veins; communications were also observed between the CS and RA (n = 4) or LA (n = 4); two patients had collateral venous pathways between dilated cardiac veins with RA; two patients had unroofing of the CS as outlet channels. Nine patients (60%) had cardiac anomalies: coronary artery fistula to the pulmonary artery (n = 6) or left ventricular base and CS (n = 1), atrial septal defects (n = 2), and a ventricular septal defect (n = 1)., Conclusion: CSA patients have venous collateral pathways and a high incidence of associated cardiovascular anomalies such as coronary artery fistulae and atrial septal defects., (Copyright © 2012 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Objectives: The aim of this study was to compare the diagnostic performance of coronary computed tomography angiography (CCTA)-derived computed fractional flow reserve (FFR(CT)) and transluminal attenuation gradient (TAG) for the diagnosis of lesion-specific ischemia., Background: Although CCTA is commonly used to detect coronary artery disease (CAD), it cannot reliably assess the functional significance of CAD. Novel technologies based on CCTA were developed to integrate anatomical and functional assessment of CAD; however, the diagnostic performance of these methods has never been compared., Methods: Fifty-three consecutive patients who underwent CCTA and coronary angiography with FFR measurement were included. Independent core laboratories determined CAD severity by CCTA, TAG, and FFR(CT). The TAG was defined as the linear regression coefficient between intraluminal radiological attenuation and length from the ostium; FFR(CT) was computed from CCTA data using computational fluid dynamics technology., Results: Among 82 vessels, 32 lesions (39%) had ischemia by invasive FFR (FFR ≤0.80). Sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratio of TAG (≤ -0.654 HU/mm) for detection of ischemia were 38%, 88%, 67%, 69%, 3.13, and 0.71, respectively; and those of FFR(CT) were 81%, 94%, 90%, 89%, 13.54, and 0.20, respectively. Receiver-operating characteristic curve analysis showed a significantly larger area under the curve (AUC) for FFR(CT) (0.94) compared to that for TAG (0.63, p < 0.001) and CCTA stenosis (0.73, p < 0.001). In vessels with noncalcified plaque or partially calcified plaque, FFR(CT) showed a larger AUC (0.94) compared to that of TAG (0.63, p < 0.001) or CCTA stenosis (0.70, p < 0.001). In vessels with calcified plaque, AUC of FFR(CT) (0.92) was not statistically larger than that of TAG (0.75, p = 0.168) or CCTA stenosis (0.80, p = 0.195)., Conclusions: Noninvasive FFR computed from CCTA provides better diagnostic performance for the diagnosis of lesion-specific ischemia compared to CCTA stenosis and TAG., (Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Takayasu's arteritis (TA) is primary vasculitis. Cardiac involvements in TA is due to the consequences of the vascular lesions as well as the primary pathology of the heart. The disease activity of TA is known to influence the prognosis of TA. We hypothesized that the cardiovascular involvement of TA is related to the disease activity. We evaluated the cardiovascular manifestations of TA, and we assessed their relation to the disease activity of TA. Two hundred four patients were diagnosed with TA from September, 1994 to March, 2009 according to the diagnostic criteria of the 1990 American College of Rheumatology. Their clinical features and the laboratory, angiographic and echocardiographic findings were retrospectively reviewed. The group with active disease activity was defined as satisfying one of the following criteria: i) an elevated ESR or CRP level, ii) thickened arterial wall with mural enhancement on CT or MR angiography, and iii) carotidynia at the time of the initial diagnosis. One hundred thirty nine patients (69.2%) were classified as the active group. The cardiovascular signs and symptoms were not generally different between the active and inactive groups. The active TA patients had more frequent involvement of the ascending aorta and the aortic arch and its main branches than did the inactive group. The active group showed a higher incidence of significant aortic valve regurgitation and pulmonary hypertension, and a higher level of NT-proBNP. These findings suggest that disease activity plays an important role for the cardiovascular manifestations of TA. The TA patients with higher activity have more cardiovascular morbidity compared to the TA patients with low disease activity., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

To define causes and pathological mechanisms underlying differences in clinical outcomes, we compared the findings of contrast-enhanced magnetic resonance imaging (CE-MRI) between ST-segment elevation myocardial infarction (STEMI) and non-ST-segment elevation myocardial infarction (NSTEMI). In 168 patients undergoing early invasive intervention for STEMI (n = 113) and NSTEMI (n = 55), CE-MRI was performed a median of 6 days after the index event. Infarct size was measured on delayed-enhancement imaging, and area at risk (AAR) was quantified on T2-weighted images. The median infarct size was significantly smaller in the NSTEMI group than in the STEMI group (10.7% [5.6-18.1] vs. 19.2% [10.3-30.7], P < 0.001). Although there was a trend toward a greater myocardial salvage index ([AAR - infarct size] × 100/AAR) in the NSTEMI group compared to the STEMI group (48.2 [30.4-66.8] vs. 40.5 [24.8-53.5], P = 0.056), myocardial salvage index was similar between the groups in patients with anterior infarction (39.6 [20.0-54.9] vs. 35.5 [23.2-53.4], P = 0.96). The NSTEMI group also had a significantly lower extent of microvascular obstruction and a smaller number of segments with >75% of infarct transmurality relative to the STEMI group (0% [0-0.6] vs. 0.9% [0-2.3], P < 0.001 and 3.0 ± 2.3 vs. 4.6 ± 2.9, P = 0.001, respectively). Myocardial hemorrhage was detected less frequently in the NSTEMI group than the STEMI group (22.6% vs. 43.8%, P = 0.029). In the multivariate analysis, baseline Thrombolysis In Myocardial Infarction flow grade 3 and hemorrhagic infarction were closely associated with ST-segment elevation (OR 0.32, 95% CI 0.13-0.81, P = 0.017; OR 5.66, 95% CI 1.77-18.12, P = 0.003, respectively). In conclusion, in vivo pathophysiological differences revealed by CE-MRI assessment include more favorable infarct size, AAR, myocardial salvage and reperfusion injury in patients with NSTEMI compared to those with STEMI undergoing early invasive intervention.

The etiology of acute pericarditis is often thought to be autoimmune, and Graves' disease has been reported in a few series to manifest as acute pericarditis. Since the etiology of recurrent pericarditis is known to be more associated with autoimmune causes, recurrent acute pericarditis may be a potential cardiovascular complication of Graves' disease. We report a case of recurrent acute pericarditis that was presumed to be associated with Graves' disease which was controlled after management of the problem of the thyroid.

Purpose: This study was designed to evaluate the validity of the pediatric end-stage liver disease (PELD) score as a prognostic index of native liver survival in biliary atresia before Kasai portoenterostomy., Methods: Medical records of 63 patients who underwent Kasai portoenterostomy at our hospital were reviewed retrospectively. Rate of jaundice clearance and cumulative survival with native liver were calculated. Patients who were alive and did not require transplantation until the last follow up were included in the native-liver survival group. And patients who underwent any event, defined as death without transplantation, transplantation or listing for transplantation, were included in the native-liver failure group. Cox analysis was done., Results: 10-year survival rate with native liver was 41.9%. PELD scores of ≥ 15 and an age at operation of ≥ 60 days were found to be significant risk factors of failure(P = 0.007, OR = 2.851 and P = 0.010, OR = 3.222, respectively). Furthermore, patients with both risk factors showed significantly higher risk of failure (P = 0.001, OR = 8.809)., Conclusion: Since failure rate is higher for patients with high PELD score and older age, these factors should be helpful in estimating the prognosis. And with high-risk patients, cautious monitoring and consultation should be made whether the liver fails and requires transplantation.

Whether the left ventricular (LV) mass index (LVMI) and LV volumetric parameters are associated independently with natriuretic peptide levels is unclear in hypertrophic cardiomyopathy (HCM). Therefore, we investigated which parameters have an independent relationship with N-terminal pro-B type natriuretic peptide (NT-proBNP) levels in HCM patients using echocardiography and cardiac magnetic resonance imaging (CMR). A total of 103 patients with HCM (82 men, age 53 ± 12 years) were evaluated. Echocardiographic evaluations included left atrial volume index (LAVI) and early diastolic mitral inflow E velocity to early annular Ea velocity ratio (E/Ea). LVMI, maximal wall thickness and LV volumetric parameters were measured using CMR. The median value of NT-proBNP level was 387.0 pg/ml. The mean NT-proBNP level in patients with non-apical HCM (n = 69; 36 patients with asymmetric septal hypertrophy, 11 with diffuse, and 22 with mixed type) was significantly higher than in those with apical HCM (n = 34, P < 0.001). NT-proBNP level was negatively correlated with LV end-diastolic volume (LVEDV) (r = -0.263, P = 0.007) and positively with LVMI (r = 0.225, P = 0.022) and maximal wall thickness (r = 0.495, P < 0.001). Among the echocardiographic variables, LAVI (r = 0.492, P < 0.001) and E/Ea (r = 0.432, P < 0.001) were correlated with NT-proBNP. On multivariable analysis, non-apical HCM, increased maximal wall thickness and LAVI were independently related with NT-proBNP. Severity of LV hypertrophy and diastolic parameters might be important in the elevation of NT-proBNP level in HCM. Therefore, further evaluation of these parameters in HCM might be warranted.

To evaluate the ability to identify right ventricular (RV) dysfunction, and to predict adverse outcomes of chest computed tomography (CT), we compared CT and echocardiography in acute pulmonary embolism patients. We analyzed 56 patients diagnosed by CT with acute pulmonary embolism, who underwent echocardiography within 48 h of CT scan from January 2004 to December 2008. From the CT scan, the ratio of RV diameter to left ventricular diameter (RVd/LVd), the presence of septal bowing and embolus location were determined. RVd/LVd (P < 0.001), septal bowing (P < 0.001) and proximal embolism (P = 0.016) were associated with echocardiographic RV hypokinesia. The odds ratio for adverse clinical outcomes was 19.2 for the combination of three CT parameters (RVd/LVd > 1, septal bowing, and proximal embolism), and 13.4 for RV hypokinesia (each P = 0.001). The positive predictive value (PPV) for adverse clinical outcomes for echocardiographic RV hypokinesia was 55.0%, and the negative predictive value (NPV) was 96.2%. The three-parameter combination predicted adverse clinical outcomes with a PPV of 54.5%, and a NPV of 94.1%. CT parameters including RV dysfunction were significantly associated with poor outcomes. Rapid risk stratification of patients with acute pulmonary embolism based on chest CT appears to be comparable with echocardiography, is clinically reliable, and may be useful in guiding management strategy.

Good aerobic fitness is associated with favorable cardiovascular outcomes. However, it is not well known whether aerobic fitness correlates to the degree of coronary atherosclerosis, which affects cardiovascular prognosis. The aim of the present study was to investigate the relation between aerobic fitness and coronary atherosclerosis. A total of 8,565 apparently healthy men underwent routine health screening, including both cardiopulmonary function testing and coronary calcium scoring. The subjects with clinical cardiovascular disease or abnormal exercise electrocardiographic findings were excluded. A treadmill exercise test was done using the modified Bruce protocol, and the Agatston coronary artery calcium (CAC) score was measured using multidetector computed tomography. Advanced CAC was defined as a score > 75th percentile according to the age group. The mean age was 51 ± 7 years, the average maximum oxygen uptake was 32 ± 5 ml/kg/min, and 34% had a positive CAC score. On univariate analysis, age, blood pressure, lipid profile, body mass index, hemoglobin A1c, fasting glucose, calculated 10-year risk for coronary disease, and maximum oxygen uptake were significantly associated with advanced CAC. In the multiple logistic regression model, the subjects in the highest quartile of the maximum oxygen uptake for age were less likely to have advanced CAC for age compared to those in the lowest quartile (odds ratio 0.60, 95% confidence interval 0.48 to 0.73), with adjustment for age, hypertension, hemoglobin A1c, current smoking, body mass index, and regular exercise habit. In conclusion, greater aerobic fitness was associated with less prevalent advanced coronary atherosclerosis in an asymptomatic male population. The degree of subclinical coronary artery disease might be 1 of the mechanisms connecting aerobic fitness and cardiovascular outcome., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: We sought to determine whether a 600-mg loading dose of clopidogrel reduces myocardial infarct size compared with a 300-mg dose using contrast-enhanced magnetic resonance imaging in patients undergoing primary percutaneous coronary intervention (PCI) for ST-segment elevation myocardial infarction (STEMI)., Methods: In 198 patients undergoing primary PCI for STEMI, contrast-enhanced magnetic resonance imaging was performed a median of 7 days after the index event. Infarct size was measured on delayed-enhancement imaging, and area at risk (AAR) was quantified on T2-weighted images., Results: Baseline characteristics were not significantly different between the 600-mg clopidogrel loading group (n = 117) and the 300-mg group (n = 81). The median infarct size was significantly smaller in the 600-mg group than in the 300-mg group (17.3% [8.9%-26.2%] vs 21.7% [12.9%-30.0%], P = .03). Myocardial salvage index ([AAR - infarct size] × 100/AAR) was greater in the 600-mg group than in the 300-mg group (47.7 [33.7-60.9] vs 32.0 [23.6-51.5], P < .01). Patients in the 600-mg group also had a significantly lower extent of microvascular obstruction and smaller number of segments with >75% of infarct transmurality than did those in the 300-mg group. After propensity score matching, the 600-mg group had smaller infarct size and greater myocardial salvage index compared with the 300-mg group. In multivariate analysis, the use of a 600-mg clopidogrel loading dose significantly reduced the risk of a large infarct (odds ratio 0.53, 95% CI 0.29-0.98, P = .04)., Conclusions: In patients undergoing primary PCI for STEMI, a 600-mg loading dose of clopidogrel reduced myocardial infarct size and improved myocardial salvage compared with a 300-mg loading dose., (Copyright © 2012 Mosby, Inc. All rights reserved.)

Background: Recently developed digital tomosynthesis has shown improved detection of pulmonary lesions with a radiation dose comparable to conventional CR but with a much lower radiation dose than CT., Purpose: To compare the diagnostic performance of digital tomosynthesis (DT) with that of chest radiography (CR) for the detection of aortic arch calcification (AAC)., Material and Methods: The study included 100 patients who underwent multidetector computed tomography (MDCT), DT, and CR (DT and CR were obtained within one week of CT examination). We evaluated and compared the diagnostic performances of DT and CR for the detection of AAC with MDCT as the reference standard. The extent (four grades 0-3) of AAC on DT and CR was also compared with CT calcium score. Inter-observer agreement was analyzed by using kappa statistics., Results: On DT, overall accuracy for AAC was superior to that of CR (94% and 71%, respectively, P < 0.01). Inter-observer agreement was good with DT and CR (kappa values = 0.74 and 0.62, respectively) for the presence of AAC, and good with DT and moderate with CR (kappa value = 0.64 and 0.53, respectively) for AAC grading. The overall correlation coefficient on AAC grading between DT and CT (calcium score) was superior to that between CR and CT (0.90 and 0.60, respectively)., Conclusion: DT is superior to CR for detection and extent evaluation of AAC.

Background and Purpose: Cardiac evaluation is routinely conducted in patients with ischaemic stroke because embolisms originating from the heart are an important cause of stroke. We compared the prevalence of cardioaortic sources of cerebral embolism (CSCE) in patients with ischaemic stroke detected by transthoracic echocardiography (TTE) and/or multidetector cardiac computed tomography (MDCT). Additionaly, we investigated the frequency and severity of asymptomatic coronary artery disease (CAD) in patients who underwent MDCT., Methods: We prospectively recruited patients with non-atrial fibrillation-related stroke who had no history of cardiac disease. Patients underwent cardiac evaluation using TTE alone (N=243, during the early phase of this study--TTE period), TTE and MDCT (N=62, midphase--transitional period) and MDCT alone (N=138, late phase--MDCT period). CSCE were defined according to the original and revised TOAST classifications. CAD was defined as 50% or more stenosis of the coronary artery., Results: Patients' characteristics did not differ amongst groups; however, CSCE were more frequently detected in the MDCT period than in the TTE period (18.1% vs. 6.6%, P<0.001). In the transitional period, there were 16 patients (25.8%) who were found to be CSCE by MDCT but not TTE, whilst three patients (4.8%) were found to be CSCE by TTE but not MDCT (P=0.004). Amongst the patients who underwent MDCT, 72 (36.0%) had asymptomatic CAD, invasive coronary angiography was needed in 21 (10.5%) and 10 (5.0%) patients finally underwent coronary intervention., Conclusion: Multidetector cardiac computed tomography proved to be complementary to TTE for the identification of CSCE and can detect asymptomatic CAD, one of the major causes of vascular death in patients with stroke, in patients with non-atrial fibrillation-related stroke., (© 2011 The Author(s). European Journal of Neurology © 2011 EFNS.)

Among the cardiovascular manifestations in the Marfan syndrome (MFS), aortic dissection stands out as a major cause of early mortality. The aim of this study was to test the hypothesis that patients with the MFS who experience aortic dissection differ in clinical features and outcomes from those with aortic dissection not related to the MFS. Data from patients diagnosed with aortic dissection from December 1994 to March 2009 at 1 of the major medical centers in Korea were reviewed. The clinical presentations, dissection characteristics, and outcomes of patients with and those without the MFS in a Korean population were compared. Of 445 patients with aortic dissection, 46 (10%) had the MFS. Compared to non-MFS patients, those with the MFS developed aortic dissection at younger ages (33 ± 10 vs 57 ± 13 years, p <0.001) and were less frequently hypertensive (11% vs 73%, p <0.001). During the follow-up period, patients with the MFS more often developed aortic dilatation and expansion of the dissection (39% vs 18%, p = 0.003) and showed a higher rate of reoperation (30% vs 9%, p <0.001). In conclusion, in Korean patients, aortic dissection with the MFS had different characteristics and poorer outcomes than aortic dissection without the MFS. These findings underscore the importance of accurate diagnosis and surveillance of this condition in the MFS., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Immune dysregulation, Polyendocrinopathy, Enteropathy X-linked (IPEX) syndrome is a unique example of primary immunodeficiency characterized by autoimmune manifestations due to defective regulatory T (Treg) cells, in the presence of FOXP3 mutations. However, autoimmune symptoms phenotypically resembling IPEX often occur in the absence of detectable FOXP3 mutations. The cause of this "IPEX-like" syndrome presently remains unclear. To investigate whether a defect in Treg cells sustains the immunological dysregulation in IPEX-like patients, we measured the amount of peripheral Treg cells within the CD3(+) T cells by analysing demethylation of the Treg cell-Specific-Demethylated-Region (TSDR) in the FOXP3 locus and demethylation of the T cell-Specific-Demethylated-Region (TLSDR) in the CD3 locus, highly specific markers for stable Treg cells and overall T cells, respectively. TSDR demethylation analysis, alone or normalized for the total T cells, showed that the amount of peripheral Treg cells in a cohort of IPEX-like patients was significantly reduced, as compared to both healthy subjects and unrelated disease controls. This reduction could not be displayed by flow cytometric analysis, showing highly variable percentages of FOXP3(+) and CD25(+)FOXP3(+) T cells. These data provide evidence that a quantitative defect of Treg cells could be considered a common biological hallmark of IPEX-like syndrome. Since Treg cell suppressive function was not impaired, we propose that this reduction per se could sustain autoimmunity., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Background: The aim of this study was to investigate the effects of proton pump inhibitors on symptomatic inflammatory esophagogastric polyps (IEPs) in a pediatric cohort and to determine the optimal duration of treatment., Methods: The 11 patients with IEPs were managed with lansoprazole. Follow-up endoscopies were performed at 2 and 6 months after the start of medication. Medication was discontinued when the clinical symptoms completely resolved and the polyp size was reduced by more than 50% compared to the initial size., Results: The initial polyp size was 13.7 ± 3.3 mm. After 2 months of medication, the polyp size was reduced to 8.0 ± 5.8 mm. At 6 months, the polyp size was 4.7 ± 2.2 mm. The mean duration of medication was 4.8 ± 2.1 months. The duration of medication and the change in the polyp size appeared to have a linear correlation (p < 0.001). According to the formula used to calculate polyp size, the optimal duration of treatment was more than 7 months for complete resolution of the polyps., Conclusions: Proton pump inhibitor was effective for the treatment of IEPs. About 5 months of lansoprazole was adequate to treat IEPs in children. The optimal duration for complete resolution of the polyp might be more than 7 months., (Copyright © 2012 S. Karger AG, Basel.)

Fragmented QRS (fQRS) on 12-lead electrocardiography reflects conduction delay caused by myocardial fibrosis and dysfunction. Ventricular fibrosis detected by late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is reportedly correlated with worse clinical outcomes in adults with repaired tetralogy of Fallot (TOF). The aim of this study was to assess whether the presence of fQRS is associated with right ventricular (RV) fibrosis or dysfunction in this patient group. In 37 consecutive patients (median age 30 years, median age at repair 6.6 years), the number of leads showing fQRS, defined as the presence of >2 notches on the R/S wave in ≥2 contiguous leads, was counted. RV systolic function, dilatation, and LGE score were measured using LGE CMR. Ventricular LGE was observed mainly at the previous surgical sites: the RV outflow tract (33 of 37), ventricular septal defect patch region (15 of 37), and RV anterior wall (11 of 37). Fragmented QRS was found mostly in the right and mid precordial leads. The fQRS group (n = 20) demonstrated higher RV LGE scores (p <0.001) and lower RV ejection fractions (p = 0.02) and a trend toward larger RV end-diastolic and end-systolic volumes (p = 0.12 and p = 0.06, respectively) compared to the non-fQRS group (n = 17). The number of electrocardiographic leads showing fQRS was positively correlated with RV LGE score (r = 0.75, p <0.001). The presence of fQRS remained independently associated with the presence of supramedian RV LGE score, even after adjusting for relevant parameters. In conclusion, fQRS was closely associated with more extensive RV fibrosis and dysfunction in adults with repaired tetralogy of Fallot., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Aortic arch atheroma (AAA) has been accepted as a possible embolic source in patients with ischemic stroke, especially cryptogenic stroke. However, despite its well-known role as a risk factor for stroke, research focused on the mechanism or characteristics of stroke caused by AAA is insufficient. In this study, we aimed to evaluate clinical and radiological characteristics including lesion pattern on DWI in acute stroke patients associated with vulnerable AAA detected by multidetector row computed tomography (MDCT)., Methods: From September 2008 through May 2011, patients who presented with acute ischemic stroke and underwent MDCT were found in a prospective stroke registry. Patients without evident stroke etiology were included and classified by presence of vulnerable AAA. Vulnerable AAA was defined as (i) at least 6 mm thick adjacent to the aortic wall; (ii) ulcerated plaque, or (iii) soft plaque. Soft plaque was defined as the presence of clearly visualized area of hypoattenuation (<80 Hounsfield units) suggestive of thrombus. The patients without vulnerable AAA were classified as no/simple AAA group. The characteristics of diffusion-weighted MRI (DWI) lesions were analyzed in terms of the number and size of the lesions, and the involved vascular territories., Results: A total of 63 cryptogenic stroke patients were included in this study. Vulnerable AAA was observed in 15 (23.8%) patients. The patients with vulnerable AAA were older than those with no/simple AAA (p = 0.026). DWI analysis revealed that the vulnerable AAA group had a greater chance of having multiple and small lesions in multiple vascular territories that were mainly located in cortical and border-zone regions than the no/simple AAA group. Multiple logistic regression analysis showed that age (odds ratio 1.17; 95% confidence interval 1.02-1.34) and multiple small lesions in multiple vascular territories (odds ratio 33.18; 95% confidence interval 4.26-258.45) were independently associated with vulnerable AAA., Conclusion: Vulnerable AAA is independently associated with a DWI pattern characterized by multiple small scattered lesions in multiple vascular territories in conjunction with age. It may help determine stroke mechanism quickly and easily, and provide more information about the pathomechanism of vulnerable AAA-related stroke., (Copyright © 2012 S. Karger AG, Basel.)

Tuberculosis generally affects the respiratory tract. In developing nations, the pericardium is the most common location of extrapulmonary tuberculosis; however, tuberculous pericarditis rarely appears as a localized mass or tuberculoma. We present here a case of a 62-year-old woman with pericardial tuberculoma. She had a history of effusive tuberculous pericarditis and drainage. Because she had taken regular medication over a period of six months, the pericardial mass with an adjacent lung nodule newly detected on the chest radiogram was initially suspected of being invasive lung cancer. Prior to pathologic confirmation, precise information from imaging tests, including computed tomography, magnetic resonance imaging, and positron emission tomography-computed tomography are helpful when making decisions regarding which methods should be used for surgical approach and treatment. Through imaging, our case showed typical features of pericardial tuberculoma and a favorable clinical course after two months with a change in antituberculous therapy.

ASCI has continued to grow over the past 5 years since its establishment in 2006. Recent record shows total membership of 804 from 34 countries and 1,032 attendants from 32 countries at the last annual congress in Hong Kong in 2011. ASCI publishes ASCI Special Issues twice a year in the International Journal of Cardiovascular Imaging. The Asian CCT and CMR Guideline Working Group of ASCI published guidelines and protocol in the ASCI Special Issue of the International Journal of Cardiovascular Imaging in 2010. ASCI launched the [ASCI](2) (Advanced School for Core Investigators from ASCI) Program with the aim to recruit young ASCI members of high potential and train them to become representative scholars of Asia, which was met with much success in 2010 and 2011. ASCI is trying to widen its scope by collaborating with other cardiovascular imaging societies including the European Society of Cardiovascular Radiology. Multicenter trials by ASCI researchers and cardiac imaging registry in the Asian population are being designed to represent ASCI.

Sweet syndrome is a cutaneous lesion characterized by tender, red inflammatory nodules or papules. We describe a pediatric case of Sweet syndrome presenting 10 days after treatment with azathioprine. As azathioprine is widely used in children with inflammatory bowel disease, clinicians should be aware of this unusual adverse reaction.