Macrophage activation syndrome triggered by methotrexate-related lymphoproliferative disease in a patient with rheumatoid arthritis.

A 56-year-old woman was treated for rheumatoid arthritis for 17 years with methotrexate (MTX). Night sweats, fever and weight loss made her visit our hospital. Although levofloxacin failed to resolve her fever, she was suspected of having sepsis because of pancytopenia, elevated procalcitonin and a nodular lesion in the lung. After urgent hospitalization, she was diagnosed finally with the methotrexate-related lymphoproliferative disorder (MTX-LPD) associated with macrophage activation syndrome (MAS). Her general condition was improved with MTX withdrawal and 5-day high-dose glucocorticoid administration. Thus, even when the patient was critically ill with MAS, no cytotoxic agents were required to control MTX-LPD.

Diagnosis of infective endocarditis with cardiac CT in an adult.

The diagnosis of infective endocarditis is based on modified Duke's criteria, which includes clinical, biological, and echocardiographic findings. However, computed tomography (CT) has not been mentioned as a diagnostic tool in the criteria. We report a case of infective endocarditis confirmed by cardiac CT in which we could identify vegetations and perivalvular abscess not on transthoracic echocardiography and transesophageal echocardiography. Cardiac CT was feasible imaging modality for evaluating and diagnosing infective endocarditis. Cardiac CT should be recommended in patients with clinical suspicion of infective endocarditis even when transthoracic echocardiography and transesophageal echocardiography is negative for infective endocarditis.

A Homozygous CASQ2 Mutation in a Japanese Patient with Catecholaminergic Polymorphic Ventricular Tachycardia.

A 62-year-old female had suffered from recurrent syncopal episodes triggered by physical and emotional stress since childhood. She had no family history of sudden death. An intensive examination could not detect any structural disease, and exercise stress testing provoked polymorphic ventricular ectopy followed by polymorphic ventricular tachycardia accompanied with syncope leading to a diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT). A genetic analysis with a next generation sequencer identified a homozygous W361X mutation in the CASQ2 gene. Careful history taking disclosed that her parents had a consanguineous marriage. Here we present a Japanese patient with a recessive form of CPVT.

A multicenter trial of extracorporeal cardiac shock wave therapy for refractory angina pectoris: report of the highly advanced medical treatment in Japan.

We have previously demonstrated that cardiac shock wave therapy (CSWT) effectively improves myocardial ischemia through coronary neovascularization both in a porcine model of chronic myocardial ischemia and in patients with refractory angina pectoris (AP). In this study, we further addressed the efficacy and safety of CSWT in a single-arm multicenter study approved as a highly advanced medical treatment by the Japanese Ministry of Health, Labour and Welfare. Fifty patients with refractory AP [mean age 70.9 ± 12.6 (SD) years, M/F 38/12] without the indications of percutaneous coronary intervention (PCI) or coronary artery bypass grafting (CABG) were enrolled in 4 institutes in Japan. Ischemic myocardial regions in the left ventricle (LV) were identified by drug-induced stress myocardial perfusion imaging (MPI). Shock waves (200 shots/spot at 0.09 mJ/mm2) were applied to 40-60 spots in the ischemic myocardium 3 times in the first week. The patients were followed up for 3 months thereafter. Forty-one patients underwent CSWT and completed the follow-up at 3 months. CSWT markedly improved weekly nitroglycerin use [from 3.5 (IQR 2 to 6) to 0 (IQR 0 to 1)] and the symptoms [Canadian Cardiovascular Society functional class score, from 2 (IQR 2 to 3) to 1 (IQR 1 to 2)] (both P < 0.001). CSWT also significantly improved 6-min walking distance (from 384 ± 91 to 435 ± 122 m, P < 0.05). There were no significant changes in LV ejection fraction evaluated by echocardiography and LV stroke volume evaluated by cardiac magnetic resonance imaging (from 56.3 ± 14.7 to 58.8 ± 12.8%, P = 0.10, and from 52.3 ± 17.4 to 55.6 ± 15.7 mL, P = 0.15, respectively). Percent myocardium ischemia assessed by drug-induced stress MPI tended to be improved only in the treated segments (from 16.0 ± 11.1 to 12.1 ± 16.2%, P = 0.06), although no change was noted in the whole LV. No procedural complications or adverse effects related to the CSWT were noted. These results of the multicenter trial further indicate that CSWT is a useful and safe non-invasive strategy for patients with refractory AP with no options of PCI or CABG.

Risk factors and utility of maximum carotid intima-media thickness as a surrogate marker for coronary artery stenosis.

BACKGROUND: This study investigated the factors associated with coronary artery stenosis in outpatients. Furthermore, the usefulness of maximum carotid intima-media thickness (maximum-IMT) as a surrogate marker of coronary artery stenosis was evaluated. METHODS: We conducted a single-center retrospective study. A total of 601 outpatients (338 males; 263 females; mean age, 69.8±10.0 years) who underwent coronary computed tomography angiography between April 2006 and March 2012 were analyzed. The associations between coronary artery stenosis (≥75%) as determined by coronary computed tomography angiography and clinical and laboratory parameters were evaluated by multivariate logistic regression. Validation of maximum-IMT as measured by ultrasonography as a surrogate marker of coronary artery stenosis was analyzed by receiver operating characteristic (ROC) curve analysis. RESULTS: The estimated glomerular filtration rate (eGFR: mL/min/1.73 m2) (odds ratio [OR] 0.985, p<0.01), diabetes mellitus (OR 1.98, p<0.05), and maximum-IMT (mm) (OR 1.76, p<0.01) were significantly associated with coronary artery stenosis (≥75%). In analysis of each group categorized by identified factors, such as renal impairment (eGFR <60 mL/min/1.73 m2) and diabetes mellitus, the ROC curve of maximum-IMT was significant in the group of patients with diabetes mellitus without renal impairment (p<0.01) (cutoff value of maximum-IMT, 2.0 mm; sensitivity, 0.74; and specificity, 0.54) but not in other groups. CONCLUSION: Renal impairment, diabetes mellitus, and increased maximum-IMT may be significant risk factors of coronary artery stenosis. Maximum-IMT as measured by ultrasonography may be a useful surrogate marker for coronary artery stenosis in patients with diabetes mellitus without renal impairment but not in other patients.

Initial results of carotid artery stenting in Japan.

Carotid artery stenting (CAS) was approved by the government and reimbursement was started in 2008 in Japan, probably the last country in the world. CASCARD is a retrospective registry study performed by cardiologists to assess initial results of CAS in Japan. CAS was indicated for patients with at least one high risk factor for carotid endarterectomy and with >50 % stenosis in symptomatic or >80 % stenosis in asymptomatic patients. Primary endpoint was major adverse events (MAE) including death, myocardial infarction and any stroke at 30 days. Between April 2008 and March 2010, a total of 704 cases were enrolled from 55 centers. The study population with an average age of 74 ± 8 years included 62 % asymptomatic patients, with 23 % of cases with contralateral carotid artery occlusion or significant stenoses. Angioguard filter wire was exclusively used as a primary protection device and successfully passed the lesion in 701 cases (99.6 %). Precise stent was implanted successfully in all cases. MAE at 30 days was 3.7:0.3 % death, 0 % myocardial infarction, and 3.4 % stroke (0.4 % major ipsilateral stroke). Death or any stroke rate at 30 days was 2.7 % in asymptomatic and 5.5 % in symptomatic patients. The CASCARD study showed that the initial results of CAS in Japan are acceptable for both symptomatic and asymptomatic cases.

Upfront thrombus aspiration in primary coronary intervention for patients with ST-segment elevation acute myocardial infarction: report of the VAMPIRE (VAcuuM asPIration thrombus REmoval) trial.

OBJECTIVES: This study evaluated safety and efficacy of upfront thrombus aspiration during primary percutaneous coronary intervention (PCI) in patients with ST-segment elevation myocardial infarction (STEMI). BACKGROUND: Distal embolization during primary PCI results in reduced myocardial perfusion and poor clinical outcomes. METHODS: The VAMPIRE (VAcuuM asPIration thrombus REmoval) study was a prospective, randomized, controlled multicenter trial conducted in 23 institutions. Patients (N = 355) presenting within 24 h of STEMI symptoms onset were randomized to primary PCI with (n = 180) or without (n = 175) upfront thrombus aspiration using Nipro's TransVascular Aspiration Catheter (Osaka, Japan). RESULTS: The TransVascular Aspiration Catheter reached the lesion in 100% of cases. It successfully crossed the target obstruction in 86% without any delay in procedure time or time to reperfusion; whereas macroscopic thrombi were removed in 75% of the cases. Procedure success was similar between groups (98.9% vs. 98.3%). There was a trend toward lower incidence of slow or no reflow (primary end point-defined as a Thrombolysis In Myocardial Infarction flow grade <3) in patients treated with aspiration versus conventional primary PCI (12.4% vs. 19.4%, p = 0.07). Rate of myocardial blush grade 3 was higher in the aspiration group (46.0% vs. 20.5%, p < 0.001). Aspiration was most effective in patients presenting after 6 h of symptoms onset (slow flow rate: 8.1% vs. 37.6%, p = 0.01). CONCLUSIONS: This study suggested the safety of primary PCI with upfront thrombectomy using a novel device in patients with STEMI. The study showed a trend toward improved myocardial perfusion and lower clinical events in patients treated with aspiration. Patients presenting late after STEMI appear to benefit the most from thrombectomy.

Characterization and morphology of atherosclerotic plaque of coronary arteries: utility of electron-beam tomography to detect non-calcified plaque: a comparison with conventional coronary angiography and intravascular ultrasound.

BACKGROUND: Electron-beam tomography (EBT) may provide useful information about characterization and morphology of atherosclerotic plaque of coronary arteries. MATERIALS AND METHODS: Twenty-six subjects (20 male, 6 female) with suspected coronary heart disease had two routine (r) and one enhanced (e) EBT scans to detect non-calcified plaque (NCP) in the coronary arterial lumen, and were compared with conventional coronary angiograms (CAG) and intravascular ultrasound (IVUS). RESULTS: Three had the sites, which did not have high CT values suggesting calcification in rEBT, nor which was not enhanced by contrast material in eEBT. One had the site with positive CT values that were supposed to be the proliferation intima or organized thrombus and at the corresponding site mixed plaque was observed in the IVUS image. The other two had the site with negative CT values that were supposed to be fat tissue with significant stenosis in CAG. We also made the cross-sectional images of the vessel and the morphology of the NCP, which projected into the lumen, could be evaluated. CONCLUSIONS: We could detect the NCP, differentiate fat tissue from soft tissue and evaluate the morphology of the plaque in EBT.

Endoluminal perspective volume rendering of coronary arteries using electron-beam computed tomography.

Remarkable progress has been made in the treatment of coronary heart diseases because of a variety of new interventional devices, but as each new device or procedure has suitability for a particular type of patient or purpose, patient selection is increasingly important. Endoluminal perspective volume renderings of the coronary arteries of a 70-year-old male with old myocardial infarction and recurrent chest pain were carried out using electron-beam computed tomography. Conventional coronary angiography had revealed significant stenosis of the distal portion of the left anterior descending branch, and subsequent conventional balloon angioplasty had failed to expand the stenotic site. Perspective volume rendering images can distinguish differences in objects and evaluate the cross sectional area of the lumen and the morphology of calcification. In the present patient, a huge mass of calcified plaque occupied most of the lumen at a site corresponding to the angiographic site of stenosis. According to this finding, rotational atherectomy was indicated and had a good outcome. The qualitative information for characterizing and determining the morphology of atherosclerotic plaque provided by perspective volume rendering may be useful in selecting the appropriate intervention.