Abstract
Background
Although rare, angiosarcoma is the most common type of cardiac primary malignancy. This disease can cause life-threatening complications and the prognosis remains poor. There is ...no standard approach to care, and clinical judgement is exercised on a case-by-case basis. Tumour progression causes serious complications, such as heart failure and vascular disruption.
Case summary
A 64-year-old Japanese woman presenting with a right atrial tumour was referred to our department. Tumour biopsy revealed that the patient suffered from angiosarcoma. We performed a lumpectomy to excise the tumour, but due to tissue adhesions in and around the right atrium, the malignancy could not be completely removed. After 3 years of chemotherapy, the patient was admitted to our hospital with increased chest pain. Emergency coronary angiogram revealed severe stenosis of the ostial right coronary artery. Intravascular ultrasound (IVUS) and computed tomography suggested coronary compression due to cardiac angiosarcoma. In this study, we report a unique case of advanced cardiac angiosarcoma, presenting as unstable angina, which was successfully treated with percutaneous coronary intervention using stent implantation.
Discussion
Due to the rarity of cardiac primary angiosarcoma, many symptoms are misdiagnosed until mechanical complications arise, such as coronary compression. The clinical course and various imaging modalities are useful for differentiating angiosarcomas from coronary stenosis.
Aims: Measurement of protein S (PS) activity in patients taking direct oral anticoagulants (DOACs) using reagents based on a clotting assay results in falsely high PS activity, thus masking inherited ...PS deficiency, which is most frequently seen in the Japanese population. In this study, we investigated the effect of factor Xa (FXa) inhibitors on PS activity using the reagent on the basis of the chromogenic assay, which was recently developed in Japan. Methods: The study enrolled 152 patients (82 males and 70 females; the average age: 68.5±14.0 years) receiving three FXa inhibitors (rivaroxaban, edoxaban, and apixaban). PS activity was measured using the reagents on the basis of the clotting and chromogenic assays. Results: PS activity measured by the clotting assay reagents exhibited falsely high values depending on the plasma concentrations of FXa inhibitors in patients taking either rivaroxaban or edoxaban. However, none of the three FXa inhibitors affected PS activity when measured using the chromogenic assay. Conclusion: In patients taking rivaroxaban or edoxaban, inherited PS deficiency is likely missed because the levels of PS activity measured using the reagents based on the clotting assay are falsely high. However, we report that three FXa inhibitors do not affect PS activity measured by the chromogenic assay. When measuring the levels of PS activity in patients undergoing DOACs, the principles of each reagent should be understood. Furthermore, plasma samples must be collected at the time when plasma concentrations of DOACs are lowest or the DOAC-Stop reagent should be used.
BackgroundSimplicity HTN-3 trial showed the negative result for renal denervation (RDN) in patient with resistant hypertension. One of limitations was that no appreciate evaluation of renal ...sympathetic nerve activity (RSNA) was applied before and after RDN. 123I-MIBG has been used to assess the cardiac sympathetic nerve activity. However, it remains unclear whether 123I-MIBG in kidney would reflect sympathetic nerve activity. In this study, we investigated the relationship renal 123I- MIBG and muscle sympathetic nerve activity (MSNA).Methods31 hypertensive patients were included in this study. MSNA was recorded from the peroneal nerve to evaluate direct sympathetic nerve activity to peripheral vascular bed. Images of 123I-MIBG were taken using the low-medium-energy general purpose collimator. An 111MBq dose of 123I-MIBG was injected intravenously at rest for the MIBG study. 123I-MIBG planar and SPECT studies were performed at 20min (early) and 3hr (delayed) after injection. In the planar study, a region of interest (ROI) was set over the heart and a rectangular ROI on the upper third of the mediastium and kidney cortex. The early and delayed heart-to-mediastinum average count ratios (H/M) and kidney-to-mediastinum average count ratios (K/M) were calculated. Washout rate (WR) of heart and kidney were also calculated.ResultsAverage systolic and diastolic blood pressure was 118±18/67±15 mmHg. Average estimated glomerular filtration rate was 71.5±22.4 ml/min/1.73m2 that was not related to renal 123I-MIBG. As expected, MSNA had significant negative relationship with delayed H/M in cardiac 123I-MIBG image (r=-0.37, p<0.01) and positive relationship with WR of cardiac 123I-MIBG (r=0.45, p<0.01). With regard to kidney, WR of 123I-MIBG of each left and right renal MIBG showed a significant correlation with MSNA (right kidneyr=0.56, p<0.01, left kidneyr=0.60, p<0.01, respectively), but not with early and delayed K/M in renal 123I-MIBG.ConclusionsOur study showed that WR of renal 123I-MIBG was associated with MSNA, indicating that WR of renal 123I-MIBG might reflect RSNA. These results suggest that renal 123I-MIBG might be available to assess sympathetic nerve activity as well as MSNA.
ABSTRACT A 58-year-old man, in whom an implantable cardiac defibrillator (ICD) had been implanted for Brugada syndrome, suffered rapidly progressive general paralysis. Various diagnostic imaging ...techniques were performed, but the cause could not be determined. Magnetic resonance imaging (MRI) scanning was performed. A 1.5-Tesla MRI system was used, and the ICD was programmed to ODO mode and all tachycardia detection was turned off. MRI was performed safely under electrocardiogram and pulse oximeter monitoring, and appropriate precautions were taken in preparation for an emergency. ICD parameters did not change in post-imaging investigations. MRI revealed an apparent tumor in the patient's medulla and upper cervical spinal cord, which was diagnosed as high-grade astrocytoma. When performing MRI procedures in patients with an ICD under urgent conditions, it is necessary to have complete knowledge of the procedure and to make careful preparations.
Muscle atrophy F-box (MAFbx/atrogin-1), an E3 ubiquitin ligase, is a crucial mediator of skeletal muscle atrophy and cardiac hypertrophy in response to pressure overload and exercise. The role of ...MAFbx in the regulation of cardiac remodeling after myocardial infarction (MI) remains unclear. Permanent coronary ligation of the left coronary artery was performed on MAFbx knockout (KO) and wild-type (WT) mice and MAFbx expression in the WT mice was shown to be significantly increased in the left ventricles after MI. The mortality rate due to post-MI cardiac rupture was significantly decreased in MAFbx KO mice compared to that in the WT mice. DNA microarray and mRNA expression analyses revealed that the upregulation of genes involved in inflammatory processes and cell motility of leukocytes and neutrophils, including Mmp9, Il1b, Cxcl2, and Nlrp3, was significantly attenuated in MAFbx KO mice 1 day after MI. MAFbx downregulation inhibited nuclear factor-κB (Nfkb) activation after MI. Flow cytometry results demonstrated that the myocardial infiltration of neutrophils was suppressed in MAFbx KO mice 1 day after MI. Nlrp3 and Il1b protein levels were decreased in MAFbx KO mice compared with those in the WT mice. MAFbx downregulation significantly attenuated Tnfa-induced Cxcl2, Il1b, and Nlrp3 expression in cardiomyocytes. We conclude that MAFbx plays an important role in the mediation of excessive inflammation, including neutrophil infiltration, inflammasome formation, and production of proinflammatory cytokines through the activation of Nfkb, promoting cardiac rupture after MI.
•Muscle atrophy F-box (MAFbx/atrogin-1) is upregulated in the heart after MI.•Loss of MAFbx reduces cardiac rupture after MI.•Loss of MAFbx attenuates upregulation of proinflammatory cytokines and Mmp9 after MI.•Neutrophil infiltration and inflammasome activity during MI are suppressed in MAFbx KO.•Nfkb activation following MI is suppressed in MAFbx KO.
Renal denervation is effective for modulating augmented sympathetic nerve activity (SNA) in heart failure with reduced ejection fraction (HFrEF). We have demonstrated that renal ...iodine123-metaiodobenzylguanidine (123I-MIBG) scintigraphy is associated with muscle sympathetic nerve activity (MSNA) in patients with hypertension. However, it is unclear whether renal 123I-MIBG scintigraphy is useful for assessment of SNA in HFrEF.
The study population consisted of 24 HFrEF patients and 11 healthy subjects as controls. Patients with HFrEF underwent 123I-MIBG scintigraphy and hemodynamics using a Swan-Ganz catheter (SGC). HFrEF was defined as echocardiography with left ventricular ejection fraction (LVEF) < 50%. MSNA was measured from the peroneal nerve for direct evaluation of SNA. Renal 123I-MIBG scintigraphy was performed simultaneously with cardiac scintigraphy. The early and delayed kidney-to-mediastinum ratio (K/M), early and delayed heart-to-mediastinum ratio (H/M), and washout rate (WR) were calculated.
LVEFs were 35% ± 11% in patients with HFrEF and 63% ± 10% in the controls (p < 0.01). The WR of cardiac 123I-MIBG showed no relation to MSNA, but was related to stroke volume (r = 0.45, p < 0.05). In contrast, the WR of renal 123I-MIBG scintigraphy (average of both sides) showed a strong correlation with MSNA (BI, r = 0.70, p < 0.01; BF, r = 0.66, p < 0.01); however, no significant correlations were detected between renal 123I-MIBG scintigraphy and SGC results.
The WR of renal 123I-MIBG scintigraphy may reflect MSNA. Further studies are needed to clarify the relationship between renal 123I-MIBG imaging and renal SNA.
•Cardiac and renal WR were higher in the HFrEF group than the controls.•The increase in WR of renal 123I-MIBG is accompanied by an increase in MSNA.•Cardiac MIBG images showed significant correlation with impaired hemodynamics, but not renal MIBG images.
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