Summary ERG is immunoexpressed in vascular endothelial tumors, blastic extramedullary myeloid tumors, and tumors with ERG-involved translocation, such as prostate carcinoma or Ewing sarcoma. ...Recently, ERG immunoexpression was reported in an epithelioid sarcoma, which is a SMARCB1/INI1-deficient tumor, although epithelioid sarcoma is not associated with chromosomal translocations involving ERG and is categorized as a tumor with uncertain differentiation. SALL4 is essential for a proliferation and stabilization of embryonic stem cells. It was reported that SALL4 expression may aid in distinguishing epithelioid sarcoma from malignant rhabdoid tumor. We analyzed the frequency of ERG and SALL4 expressions in 80 SMARCB1/INI1-deficient tumors, including 45 epithelioid sarcomas (conventional-type, 24; proximal-type, 20), 17 malignant rhabdoid tumors, 5 atypical teratoid/rhabdoid tumors, 6 undifferentiated/unclassified sarcomas, 5 myoepithelial tumors, and 4 extraskeletal myxoid chondrosarcomas. We found that ERG expression was present in 18 of the epithelioid sarcomas (41%), including 13 conventional-type (54%) and 5 proximal-type (25%), whereas all 17 of the malignant rhabdoid tumors exhibited negative immunoreactivity. One atypical teratoid/rhabdoid tumor (20%), 1 myoepithelial carcinoma (20%), 1 undifferentiated/unclassified sarcoma (17%), and no extraskeletal myxoid chondrosarcomas (0%) also showed ERG expression. SALL4 expression was recognized in 5 epithelioid sarcomas (11%), 12 malignant rhabdoid tumors (71%), 2 atypical teradoid/rhabdoid tumors (40%), 4 undifferentiated/unclassified sarcomas (67%), 1 myoepithelial tumor (20%), and none of the extraskeletal myxoid chondrosarcomas (0%). Therefore, the evaluation of ERG and SALL4 immunoexpressions may be a useful diagnostic tool to distinguish epithelioid sarcoma, especially proximal type, from malignant rhabdoid tumor.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Abstract Background Research on the correlation of serum bilirubin level with cardiac function as well as outcomes in heart failure patients with cardiac resynchronization therapy (CRT) has not yet ...been reported. The aim of this study was to analyze the relationship between change in serum bilirubin level and left ventricular reverse remodeling, and also to clarify the impact of bilirubin change on clinical outcomes in CRT patients. Methods We evaluated 105 consecutive patients who underwent CRT. Patients who had no serum total-bilirubin data at both baseline and 3–9 months’ follow-up or had died less than 3 months after CRT implantation were excluded. Accordingly, a total of 69 patients were included in the present analysis. The patients were divided into two groups: decreased bilirubin group (serum total-bilirubin level at follow-up ≤ that at baseline; n = 48) and increased bilirubin group (serum total-bilirubin level at follow-up > that at baseline; n = 21). Results Mean follow-up period was 39.3 months. In the decreased bilirubin group, mean left ventricular end-systolic diameter decreased from 54.5 mm to 50.2 mm ( p = 0.001) and mean left ventricular ejection fraction increased significantly from 29.8% to 37.0% ( p = 0.001). In the increased bilirubin group, there was no significant change in echocardiographic parameters from baseline to follow-up. In Kaplan–Meyer analysis, cardiac mortality combined with heart failure hospitalization in the increased bilirubin group was significantly higher than that in the decreased bilirubin group (log-rank p = 0.018). Multivariate Cox regression analysis revealed that increased bilirubin was an independent predictor of cardiac mortality combined with heart failure hospitalization (OR = 2.66, p = 0.023). Conclusions The change in serum bilirubin is useful for assessment of left ventricular reverse remodeling and prediction of outcomes in heart failure patients with CRT.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Abstract Background The effect of cardiac pacing on sleep-disordered breathing is controversial. We investigated the long-term effect of cardiac pacing on sleep disordered breathing in patients with ...conventional indications for permanent pacemakers Methods Subjects comprised 40 patients (29 men; mean age 69±9 years, mean left ventricle ejection fraction 69±8%, and body mass index 23.6±3.5 kg/m2 ) who were diagnosed with indications for permanent pacemakers (sick sinus syndrome in 23 patients, atrioventricular block in 15, and brady atrial fibrillation in 2). All patients received polysomnographic evaluations before implantation of permanent pacemakers. After implantation of permanent pacemakers, all patients received polysomnographic evaluations during use of the pacemaker settings (AAI/DDD/VVI at 70 beats per minute). Results The mean follow-up period was 35±13 months. Before implantation, the distribution of sleep-disordered breathing was as follows: 93% had apnea hypopnea index >5, 58% had apnea hypopnea index >15, and 20% had apnea hypopnea index >30. The mean apnea hypopnea index for all patients was 20±15, for those with obstructive type apnea was 4.9±5.3, and for those with central type apnea was 3.0±4.5. The mean Epworth Sleepiness Score was 5.9±4.0. No patient received continuous positive airway pressure therapy or any other therapy for sleep-disordered breathing during the follow up period. The mean apnea hypopnea index at 1 week after implantation of permanent pacemakers was 21±14 ( P =0.8) and the mean apnea hypopnea index at end of follow-up was 11±7 ( P <0.0001). Conclusion Long term cardiac pacing significantly reduces the number of episodes of sleep apnea in patients with conventional permanent pacemaker indications.
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FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, UILJ, UL, UM, UPCLJ, UPUK, VSZLJ, ZAGLJ, ZRSKP
Abstract Patients with corrected transposition of great arteries (c-TGA) are generally known to develop atrioventricular block, systemic right ventricular dysfunction, and tricuspid regurgitation ...over time, which are associated with tachyarrhythmia and progressive heart failure. A 76-year-old man had been diagnosed with c-TGA. He developed a cardiopulmonary arrest while playing tennis, and an automated external defibrillator detected ventricular fibrillation (VF). Immediate cardiopulmonary resuscitation and intensive treatment were performed. He fully recovered without neurological sequelae. QRS duration was 172 ms. Echocardiography showed marked dysfunction and dyssynchrony of the systemic right ventricle (systemic right ventricular end-diastolic diameter/end-systolic diameter = 73/60 mm, systemic right ventricular ejection fraction = 34%). For secondary prevention and treatment of progressive heart failure, cardiac resynchronization therapy with defibrillator (CRT-D) implantation was recommended. Venography via the left cubital superficial vein revealed a persistent left superior vena cava (PLSVC) and giant coronary sinus that did not connect with the right superior vena cava (SVC). Because of the acute angle between the PLSVC and great cardiac vein, we selected a right-sided approach via the right SVC. We were finally able to deliver a coronary sinus lead to the lateral vein. CRT-D implantation can be achieved even in patients with c-TGA and PLSVC. < Learning objective: CRT-D implantation can be achieved even in a patient with c-TGA and PLSVC.>
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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