Prediction of major complications after hepatectomy using liver stiffness values determined by magnetic resonance elastography.

BACKGROUND: Liver fibrosis is a risk factor for hepatectomy but cannot be determined accurately before hepatectomy because diagnostic procedures are too invasive. Magnetic resonance elastography (MRE) can determine liver stiffness (LS), a surrogate marker for assessing liver fibrosis, non-invasively. The aim of this study was to investigate whether the LS value determined by MRE is predictive of major complications after hepatectomy. METHODS: This prospective study enrolled consecutive patients who underwent hepatic resection between April 2013 and August 2016. LS values were measured by imaging shear waves by MRE in the liver before hepatectomy. The primary endpoint was major complications, defined as Clavien-Dindo grade IIIa or above. Logistic regression analysis identified independent predictive factors, from which a logistic model to estimate the probability of major complications was constructed. RESULTS: A total of 96 patients were included in the study. Major complications were observed in 15 patients (16 per cent). Multivariable logistic analysis confirmed that higher LS value (P = 0·021) and serum albumin level (P = 0·009) were independent predictive factors for major complications after hepatectomy. Receiver operating characteristic (ROC) analysis showed that the best LS cut-off value was 4·3 kPa for detecting major complications, comparable to liver fibrosis grade F4, with a sensitivity of 80 per cent and specificity of 82 per cent. A logistic model using the LS value and serum albumin level to estimate the probability of major complications was constructed; the area under the ROC curve for predicting major complications was 0·84. CONCLUSION: The LS value determined by MRE in patients undergoing hepatectomy was an independent predictive factor for major complications.

The role of nuclear imaging in pulmonary hypertension.

Pulmonary hypertension (PH) is a disease characterized by a chronic elevation of pulmonary artery pressure from various causes. Pulmonary artery hypertension (PAH) is one of subtype which results in premature death often as a result of right ventricular (RV) dysfunction. In spite of the recent progress in novel cardiac imaging techniques and new drugs for PAH, there remain significant unresolved issues including a need for earlier diagnosis, refinement of risk stratification, and monitoring the effects of treatment. Cardiac and pulmonary imaging with transthoracic echocardiography (TTE) with Doppler, magnetic resonance imaging (MRI), and computed tomography (CT) are done routinely in many clinical centers. However, routine and emerging nuclear techniques may have a pivotal role of assessment of the patient with PH, and is currently the subject of significant research. Potential Roles for Nuclear Imaging in the Evaluation of the PH Patient: (1) Evaluation of cardiac structure and function (RNA) (non-nuclear techniques would include TTE, CT, and MRI). (2) Functional imaging. This includes the use of ventilation-perfusion scintigraphy (V/Q scan) to diagnose chronic thromboembolic pulmonary hypertension (CTEPH), 123l-metaiodobenzylguanidine (MIBG) imaging to evaluate the cardiac sympathetic nervous system (non-nuclear techniques include invasive right heart catheterization and TTE). (3) Measurement of RV perfusion (with gated SPECT studies). (4) Evaluation of cardiac and pulmonary metabolism (PET scans). This review article will summarize the pathophysiology, classification, natural history, and diagnostic approach of PH. Current and emerging nuclear techniques will be discussed under the four themes of evaluation of structure, functional imaging, flow, and metabolism. These will be compared to current and emerging nuclear and non-nuclear diagnostic tests in the evaluation and management of patients with PH. We will also discuss research applications exploring new insights into flow and metabolism in the right heart and lung and the application of new radioligands.

Simple Resistance Exercise helps Patients with Non-alcoholic Fatty Liver Disease.

To date, only limited evidence has supported the notion that resistance exercise positively impacts non-alcoholic fatty liver disease. We evaluated the effects of resistance exercise on the metabolic parameters of non-alcoholic fatty liver disease (NAFLD) in 53 patients who were assigned to either a group that performed push-ups and squats 3 times weekly for 12 weeks (exercise group; n=31) or a group that did not (control; n=22). Patients in the control group proceeded with regular physical activities under a restricted diet throughout the study. The effects of the exercise were compared between the 2 groups after 12 weeks. Fat-free mass and muscle mass significantly increased, whereas hepatic steatosis grade, mean insulin and ferritin levels, and the homeostasis model assessment-estimated insulin resistance index were significantly decreased in the exercise group. Compliance with the resistance exercise program did not significantly correlate with patient background characteristics such as age, sex, BMI and metabolic complications. These findings show that resistance exercise comprising squats and push-ups helps to improve the characteristics of metabolic syndrome in patients with non-alcoholic fatty liver disease.

Eye movements during emotion recognition in faces.

When distinguishing whether a face displays a certain emotion, some regions of the face may contain more useful information than others. Here we ask whether people differentially attend to distinct regions of a face when judging different emotions. Experiment 1 measured eye movements while participants discriminated between emotional (joy, anger, fear, sadness, shame, and disgust) and neutral facial expressions. Participant eye movements primarily fell in five distinct regions (eyes, upper nose, lower nose, upper lip, nasion). Distinct fixation patterns emerged for each emotion, such as a focus on the lips for joyful faces and a focus on the eyes for sad faces. These patterns were strongest for emotional faces but were still present when viewers sought evidence of emotion within neutral faces, indicating a goal-driven influence on eye-gaze patterns. Experiment 2 verified that these fixation patterns tended to reflect attention to the most diagnostic regions of the face for each emotion. Eye movements appear to follow both stimulus-driven and goal-driven perceptual strategies when decoding emotional information from a face.

The role of F(18)-fluorodeoxyglucose positron emission tomography in guiding diagnosis and management in patients with known or suspected cardiac sarcoidosis.

Cardiac sarcoidosis (CS) has gained significant interest in recent years with the emergence of advanced imaging modalities such as MRI and F(18)-fluorodeoxyglucose-positron emission tomography (FDG-PET) as modalities to aid in the diagnosis of this condition. CS remains a difficult condition to diagnose, particularly in cases of isolated cardiac involvement and it can present with a broad spectrum of clinical syndromes. Furthermore, the appropriate management of these patients remains controversial. FDG-PET has a potential role not only in diagnosis of CS but also in directing further therapies, facilitating the decision to start immunosuppression and monitoring the response to it. In this article, we discuss when to consider FDG-PET, outline the current optimal patient preparation and scanning protocols and then, using case examples, discuss the use of FDG-PET in follow-up of patients with known or suspected CS. We also outline how PET can influence management decisions in these patients.

Genetic association of Fc receptor-like 3 polymorphisms with susceptibility to primary biliary cirrhosis: ethnic comparative study in Japanese and Italian patients.

A functional variant in the Fc receptor-like 3 (FCRL3) gene is associated with the susceptibility to several autoimmune diseases. In this study, we examined whether the FCRL3 is associated with susceptibility to primary biliary cirrhosis (PBC) by comparing the two different ethnic groups, Japanese and Italians. We enrolled 232 patients with PBC and 230 controls in Japanese, and 216 PBC and 180 controls in Italians. Minor allele frequency of fcrl3_3 (-169 T>C) in the patients with PBC and controls was 0.20 and 0.09 in Japanese and 0.24 and 0.21 in Italians, respectively. We found a significant association of fcrl3_3 with PBC only in Japanese (P = 9.64 × 10(-7) ). These findings support the presence of common FCRL3-related pathological pathways in several autoimmune diseases, especially in Asians.

Replicated association of 17q12-21 with susceptibility of primary biliary cirrhosis in a Japanese cohort.

To examine the genetics of susceptibility to primary biliary cirrhosis (PBC), genome-wide association studies GWAS have been performed in patients of European ancestry and have shown the significant associations of IL12-related pathways, SPIB, IRF5-TNPO3, and 17q12-21. We tested whether these findings could be extended to a Japanese cohort, 303 Japanese PBC and 298 controls. We failed to detect significant associations at IL12A (rs574808, rs1075498) and IL12RB2 (rs3790567). There was no genetic variance at IRF5-TNPO3 (rs10488631) in Japanese. A single nucleotide polymorphism (SNP) at SPIB (rs3745516) reached nominal significance, but the corrected P value did not reach significance. For the 17q12-21 region, two SNPs had nominally significant associations [GSDMB (rs2305480, P = 0.022) and ZPBP2 (rs11557467, P = 0.021)] and we noted a significant P value at a SNP in IKZF3 (rs939327, P = 0.0024, P(c) = 0.017) after correction for multiple comparisons. Thus, these results indicate a haplotype on 17q12-21 with a similar association in Japanese and European PBC.

The short-term role of corticosteroid therapy for pulmonary arterial hypertension associated with connective tissue diseases: report of five cases and a literature review.

Pulmonary arterial hypertension (PAH) is a life-threatening complication in connective tissue diseases (CTD). It remains controversial whether immunosuppressive therapy is useful for PAH associated with CTD (PAH-CTD). The Dana Point algorithm does not refer such treatments in patients with PAH-CTD due to the lack of evidence. However, some case reports have shown the potential efficacy of immunosuppression for PAH-CTD. Here we report five cases of PAH-CTD treated with corticosteroids and discuss the current management of PAH-CTD with immunosuppressive agents. Our cases consisted of three active systemic lupus erythematosus (SLE), a quiescent SLE and an active polymyositis. WHO functional classes at baseline were class III in three cases and class II in two. Median follow-up period was 44 (28-92) weeks. PAH was diagnosed by right heart catheterization in all cases (median pulmonary arterial pressure was 45 (29-49) mmHg). All patients received 1 mg/kg of prednisolone (PSL) for 2-4 weeks, followed by appropriate dose reduction. Methylprednisolone pulse therapy was performed in patients resistant to the high dosage of PSL. Four patients received vasodilators in combination. The therapy as above improved WHO functional class 4 weeks after the initiation of PSL in all the patients. Two patients required dose increase or additional administration of vasodilators due to the dose reduction of PSL. Corticosteroid therapy may be effective for PAH-CTD at least in the short term, even in low general activity of CTD or moderate PAH. Our experience suggests that corticosteroid therapy, by itself or in conjunction with standard vasodilators, is effective for PAH-CTD patients.

EUS for portal hypertension: a comprehensive and critical appraisal of clinical and experimental indications.

Endoscopic ultrasonography (EUS) has significantly improved our understanding of the complex vascular structural changes that occur in portal hypertension and their clinical and prognostic significance. EUS in combination with color Doppler technique enables us to study the hemodynamic changes in the portal venous system noninvasively, and to determine objectively the effect of different pharmacological agents on portal hypertension. EUS has also found some role in the treatment and follow up of esophageal and gastric varices. It may play a clinical role in the diagnosis of gastric, duodenal, and rectal varices. Recently reported EUS-based devices that measure variceal wall tension and intravariceal pressure noninvasively could have an impact on the identification of patients at high risk of variceal bleeding with the aim of initiating prophylactic treatment, and in the assessment of patients' responses to drug therapy of portal hypertension. EUS is occasionally very helpful in the clinical management of portal hypertension. It is an interesting and important research tool for many experimental indications that are not routinely applied in clinical practice at this time.

Fatal hepatitis B virus reactivation in a chronic myeloid leukemia patient during imatinib mesylate treatment.

Reactivation of chronic hepatitis B virus (HBV) infection in patients undergoing chemotherapy is well-documented, but reactivation during imatinib mesylate treatment has not been reported. This study reports a 54-year-old man, without prior liver dysfunction but with chronic HBV infection, in whom fatal HBV reactivation occurred during treatment of chronic myeloid leukemia (CML) with imatinib mesylate. He developed fulminant hepatitis followed by marked elevation of HBV DNA polymerase, probably from the lymphocytopenic and immunosuppressive status induced by imatinib mesylate. Imatinib mesylate is widely used to treat CML patients. Although therapy with imatinib mesylate is generally well tolerated, the case presented here suggests that viral reactivation should be considered, even when using imatinib mesylate to treat CML.

Radiofrequency thermal balloon coronary angioplasty: a new device for successful percutaneous transluminal coronary angioplasty.

OBJECTIVES: The purpose of this study was to evaluate the effects of thermal balloon percutaneous transluminal coronary angioplasty using radiofrequency energy in the treatment of patients with failed coronary angioplasty and complex lesions. In addition, we evaluated restenosis after radiofrequency thermal balloon applications. BACKGROUND: The efficacy of coronary angioplasty is limited by the relatively low success rate in complex lesions and the high frequency of restenosis. Few reports have studied the combined effects of pressure and laser thermal energy. This study describes a new device for coronary angioplasty using radiofrequency thermal energy. METHODS: Thirty-two patients with failed conventional coronary angioplasty or complex lesions were treated with radiofrequency thermal balloon coronary angioplasty. Radiofrequency energy was delivered up to 11 times in exposures ranging from 30 to 60 s in duration. This combined effect allowed the vascular wall to be heated to temperatures ranging from 60 to 70 degrees C. Follow-up coronary angiography was performed, on average, 6 months after the procedure. RESULTS: Successful radiofrequency coronary angioplasty was achieved in 28 (82%) of 34 lesions. There was one abrupt coronary artery occlusion (3%) and no death, perforation or dissection. Angiographic restenosis occurred in 14 (56%) of 25 lesions. CONCLUSIONS: In patients with failed coronary angioplasty and difficult complex lesions, radiofrequency coronary angioplasty could potentially improve angioplasty success rates and may have important implications for bailout cases with abrupt occlusion. However, restenosis remains a significant problem.

Electrophysiologic effects of ischemic preconditioning on QT dispersion during coronary angioplasty.

OBJECTIVES: The aim of this study was to examine the effect of ischemic preconditioning on the manner of ventricular repolarization by assessing the change in QT dispersion during coronary angioplasty. BACKGROUND: QT interval dispersion reflects regional variations in ventricular repolarization and cardiac electrical instability. Previous studies have suggested that increased QT dispersion is associated with an increased incidence of malignant ventricular arrhythmias, whereas brief episodes of myocardial ischemia can render the heart more resistant to subsequent ischemic episodes, a phenomenon called ischemic preconditioning. METHODS: To assess the effects of ischemic preconditioning on myocardial repolarization by examining the change in QT dispersion during coronary angioplasty, we studied 47 consecutive patients (39 men and 8 women; mean age 57 +/- 16 years). QT dispersion was measured after each balloon inflation during coronary angioplasty. Statistical analysis was performed by using repeated measurement of analysis of variance. RESULTS: There were significant differences in QT dispersion as the number of balloon inflations increased (mean +/- SD 52 +/- 14, 42 +/- 11, 36 +/- 9, 31 +/- 10 and 29 +/- 11 ms, respectively [p < 0.01], for the first, second, third, fourth and fifth balloon inflations). The magnitude of decrease in QT dispersion was significant in the first and second balloon inflations, then became insignificant with later inflations. CONCLUSIONS: These data indicate that the gradual decrease in QT dispersion provoked by coronary artery occlusion and reperfusion during coronary angioplasty may be associated with electrophysiologic effects of ischemic preconditioning on myocardium in the human heart.

Comparison of intratumoral distribution of 99mTc-MIBI and deoxyglucose in mouse breast cancer models.

UNLABELLED: The aims of this study were to evaluate the distribution of 99mTc-methoxyisobutylisonitrile (MIBI) in 3 animal models of breast cancer, the effect of radiotherapy on 99mTc-MIBI uptake, and the relationship between uptake and microvessel density. METHODS: We used syngeneic, subcutaneously transplanted FM3A, MM48, and Ehrlich mouse breast cancer. 99mTc-MIBI and FDG were injected intravenously, and tumor uptake was measured 30 min later. Double-tracer macroautoradiography (ARG) images were prepared with 99mTc-MIBI and 2-deoxy-D-[1-14C]-glucose (14C-DG), analyzed quantitatively, and compared with histology. The radiotherapeutic effects of 20 Gy x-ray irradiation were monitored by measuring tumor volume, tumor uptake, and ARG findings using 99mTc-MIBI and FDG in FM3A tumors. Microvessel density was quantified by immunohistochemical staining for CD34 and compared with ARG using 99mTc-MIBI in FM3A tumors. RESULTS: FM3A, MM48, and Ehrlich tumors showed different growth rates and radiosensitivities. Uptake of FDG, but not of 99mTc-MIBI, correlated significantly with growth rates. Compared with 14C-DG, 99mTc-MIBI accumulated more in cancer cells and less in infiltrating fibroblasts and macrophages in all tumor models. Irradiation significantly decreased 99mTc-MIBI uptake, but a rapid increase was noted at recurrence on day 7. Changes in FDG uptake were not significant at recurrence. Microvessel density in tumor tissue correlated significantly with 99mTc-MIBI uptake on ARG. CONCLUSION: Accumulation of 99mTC-MIBI in cancer cells is preferential and can be used as a sensitive marker to examine the response to radiotherapy. Angiogenesis seems to enhance accumulation of 99mTc-MIBI in tumors. These characteristics may be favorable for tumor imaging using 99mTC-MIBI.

Effect of a serotonin receptor antagonist on interleukin-6-induced pulmonary hypertension in rats.

STUDY OBJECTIVES: To determine the significance of serotonin in the pathogenesis of interleukin (IL) 6-induced pulmonary hypertension (IL-6-PH) in rats, the plasma serotonin concentrations, and the effects of a specific antagonist of the serotonin (5-hydroxytryptamine [5-HT]) receptor, 1-[o-(m-methoxyphenyl)ethyl]phenoxy]-3-(dimethylamino)-2-propyl hydrogen succinate hydrochloride (MCI) on the degree of pulmonary hypertension (PH) were investigated in MCI-treated IL-6-PH (IL-6-MCI-PH) rats. MEASUREMENTS: The thickness of the media of small pulmonary arteries and the ratio of the weight of the right ventricle free wall (RV) to that of the left ventricle with the septum (LV + S) were measured as indexes of the degree of PH. Serotonin concentrations in plasma and in supernatants of cultured vascular smooth muscle cells (VSMCs) stimulated by IL-6 were measured by high-performance liquid chromatography. The amplification of DNA encoding the 5-HT receptor in the lung specimen and VSMCs was performed by polymerase chain reaction. RESULTS: The degree of PH, as determined by the medial thickness of small pulmonary arteries, was significantly increased in IL-6-PH rats as compared with normal control rats (p<0.05), and that in IL-6-MCI-PH rats was not significantly different from that in normal control rats. The RV/LV + S weight ratio in the IL-6-PH rats was significantly higher than that in normal control rats (p < 0.01). The RV/LV + S weight ratio in IL-6-MCI-PH rats was significantly lower than that in IL-6-PH rats (p < 0.01) and was not significantly different from that in normal control rats. The serotonin concentration was significantly higher in IL-6-PH rats than in normal control rats (p < 0.02), and the serotonin concentration in IL-6-MCI-PH rats was not significantly different from that in the normal control rats. The expression levels of the 5-HT receptor messenger RNA in the lung tissue tended to increase in IL-6-PH rats but was suppressed in IL-6-MCI-PH rats. IL-6 significantly increased the amount of serotonin released from VSMCs (p < 0.02). The expression of the 5-HT receptor messenger RNA was observed with IL-6 stimulation as was observed with serotonin stimulation in VSMCs. CONCLUSIONS: Serotonin receptor antagonists could be considered as potentially useful agents for the treatment of chronic thromboembolic PH, as well as for that of primary PH and PH associated with collagen vascular diseases.

Chronopharmacology of furosemide in the elderly.

The authors have previously reported the time-dependent change in the diuretic effects of furosemide, a loop diuretic agent, in young and middle-aged subjects. The current study was undertaken to examine an influence of aging on this chronopharmacologic phenomenon. Ten milligrams furosemide was given intravenously to 12 elderly subjects (greater than 70 years of age) at 9:00 AM (day trial) or at 9:00 PM (night trial) by a cross-over design. One-hour urine samples were collected for 3 hours after each administration, and urine volume and urinary excretions of sodium and furosemide were determined. Urine volume and urinary sodium excretion increased after furosemide administration. Contrary to the findings in the young and middle-aged subjects, no significant differences were observed in these parameters at any observation period between the day and night trials in the elderly subjects. Urinary furosemide excretion of the day and night trials did not significantly differ. These results suggest that the chronopharmacologic profiles of furosemide are altered in the elderly.

Primary sclerosing cholangitis with marked eosinophilic infiltration in the liver.

A 16-year-old boy was diagnosed as having primary sclerosing cholangitis (PSC), based on retrograde cholangiography showing mixed features of narrowing and dilatation of the common hepatic and intrahepatic bile ducts. However, periductal fibrosis was not observed in the needle biopsy liver specimen. The liver biopsy specimen obtained 11 years previously, at the onset of the disease had disclosed a marked infiltration of eosinophils in the portal tract with eosinophilic catinonic protein immunostaining, with marked eosinophilia (54%) being noted. In Japanese reports, eosinophilia of more than 7% was reported in 13 of 32 (40.6%) PSC patients. However, the early stage of PSC, with marked eosinophilia and eosinophilic infiltration in the liver, such as in the present case, has rarely been reported. The findings in this case suggest that eosinocytes are related to the pathogenesis of PSC.

Analysis of peripheral blood mononuclear cell stimulated with pyruvate dehydrogenase complex, T-cell receptors from patients with primary biliary cirrhosis.

Progressive destruction of the intrahepatic bile ducts in patients with primary biliary cirrhosis (PBC) is thought to be mediated by cytotoxic T cells which recognize certain epitopes, such as the pyruvate dehydrogenase complex (PDC). To clarify the T-cell repertoire in PBC, we analyzed T-cell receptor (TCR) Vbeta-chain messages expressed in peripheral blood mononuclear cells (PBMCs) stimulated with PDC and in liver biopsy specimens. PBMCs from 12 PBC patients and 6 healthy controls were examined. The TCR Vbeta repertoires of unstimulated PBMCs and PBMCs stimulated with PDC purified from bovine heart were analyzed, using the reverse transcriptase-polymerase chain reaction (RT-PCR) and single-strand conformation polymorphism (SSCP). Liver biopsy specimens from 5 PBC patients were also analyzed. In the PBC patients, several different T-cell clones, some of which showed the same mobility, were evident in both the PDC-stimulated and unstimulated PBMCs, as demonstrated by SSCP analysis. In addition, TCR clonality of infiltrating lymphocytes in the liver was also observed in PBC patients, showing common clonal T-cell accumulation with that seen in PBMCs stimulated with PDC. These data indicate that common clonal T-cell accumulation specific for PDC may be present in both peripheral PBMCs and the liver of patients with PBC.

Lipo prostaglandin E1 reduces the production of CXC chemokines in endotoxin-induced rat liver injury.

The present study attempted to assess the effect of prostaglandin E1 (PGE1) incorporated into lipid microspheres (Lipo PGE1) on chemokine production in endotoxin-induced rat liver injury. Male Wistar rats weighing 200-250 g were injected with 2 mg lipopolysaccharide (LPS) per kg intravenously. Lipo PGE1 was administered simultaneously at various concentrations (0.002, 0.02, 0.2, 2 µg/kg) in the tail vein. Blood samples and liver specimens were taken from the rats at 1, 3, 8, 12 and 24 h after injection with LPS alone or with LPS and Lipo PGE1. Serum macrophage inflammatory protein-2 (MIP-2) and cytokine-induced neutrophil chemoattractant (CINC) levels were measured by the enzyme-linked immunosorbant assay using the corresponding antibodies. Liver specimens were fixed, and the number of neutrophils that had infiltrated each liver section was determined under a microscope. Serum alanine aminotransferase (ALT) levels were significantly lower in the rats injected with LPS and Lipo PGE1 compared with those in the rats injected with LPS alone, and this difference was expressed in a PGE1 dose-dependent manner. Serum MIP-2 levels were significantly lower at 3 h (141.4+/-95.5 pg/ml) and 8 h (44.9+/-44.7 pg/ml) after injection with LPS and Lipo-PGE1 (2 µg/kg) than at the same times after injection with LPS alone (342.9+/-35.9 and 358.3+/-23.4 pg/ml, respectively). Similarly, serum CINC levels were significantly lower at 8 h (482.7+/-156.0 ng/ml) after injection with LPS and Lipo-PGE1 (2 µg/kg) than at the same time after injection LPS alone (723.3+/-29.0 ng/ml). No significant differences were observed at any time between serum tumor necrosis factor-alpha (TNF-alpha) levels in rats injected with LPS alone and in rats injected with LPS and Lipo-PGE1 (2 µg/kg). The number of neutrophils that had infiltrated the liver was significantly lower at 8 h after injection with LPS and Lipo PGE1 than at the same time after injection with LPS alone. This difference was expressed in a Lipo PGE1 dose-dependent manner. In conclusion, Lipo PGE1 reduces liver injury and serum levels of MIP-2 and CINC, but not TNF-alpha, in rats injected with LPS and also reduces the number of neutrophils that infiltrate in the liver.

Radiofrequency ablation of tachyarrhythmias in patients with Ebstein's anomaly.

We performed radiofrequency catheter ablation in five patients associated with Ebstein's anomaly to cure their refractory tachyarrhythmias. The presenting arrhythmias were four cases of orthodromic circus movement tachycardia using accessory pathways as a requisite limb, including one case of a Mahaim fiber and one of atrial flutter of common variety. All accessory pathways, including the Mahaim fiber, were ablated by RF energy delivered through the catheter placed at the AV annulus rather than the displaced anatomical AV groove. Interestingly, the antegrade or retrograde conduction interval over these accessory pathways was relatively longer than that of usual accessory pathways, and the accessory pathway potential was fractionated in some cases. The location of the atrioventricular node was displaced from the usual position to the postero-inferior area of Koch's triangle in one case. The configuration of the flutter wave was larger than usual in height as well as in width. All tachyarrhythmias were cured by RF catheter ablation. In the case of RF catheter ablation for patients with Ebstein's anomaly, close attention is indispensable in order to accomplish it safely and successfully, because of the anatomical and functional differences peculiar to Ebstein's anomaly.