12例心肌淀粉样变性的临床特点和误诊分析

目的:分析心肌淀粉样变性患者临床特点及误诊原因。方法:对12例心肌淀粉样变性患者的临床资料进行回顾性分析。结果:①误诊率高,首诊误诊率为91.7%。最常误诊为肥厚型心肌病(33.3%),其次为冠心病(25%);②心肌淀粉样变性常联合肾脏和肝功能损害;③超声心动图显示心肌颗粒样闪光回声增强者占41.7%,室间隔增厚和房间隔增厚的检出率分别为83.3%和33.3%;④心电图改变以肢体导联低电压和胸前导联R波递增不良最为常见。结论:心肌淀粉样变性常联合多脏器损害,有超声心动图和心电图的特征性改变。首诊误诊率很高,需对此病提高认识。     

心肌淀粉样变30例临床特征分析

心肌淀粉样变属于少见病,临床对其认识不足,易将其误诊为肥厚型心肌病、扩张型心肌病、冠心病或限制性心肌病等。因此,早期识别和诊断心肌淀粉样变性具有实际价值和意义。本文回顾分析30例心肌淀粉样变性患者的临床表现及心电图、心脏超声和组织活检结果,旨在提高该病的早期诊断水平。     

cTnT和NT-proBNP联合检测在扩张型心肌病诊断中的应用

目的探讨氨基末端脑钠肽前体(NT-proBNP)与心肌钙蛋白T(cTnT)联合检测在扩张型心肌疾病诊断中的价值。方法选取100例扩张型心肌病患者作为观察组,将50例正常健康者作为对照组,分别测定两组对象的cTnT及NT-proBNP水平。结果扩张型心肌病患者cTnT水平为(0.71±0.57)ng/ml,高于对照组〔(0.06±0.08)ng/ml〕,观察组患者血清内NT-proBNP水平(561.61±143.98)pg/mL,显著高于对照组的(146.10±116.12)pg/ml(均P<0.05)。选用NT-proBNP及cTnT联合检测对扩张型心肌病的敏感度与特异性分别为92%与98%,显著优于单项检测(P<0.05)。结论为提高扩张型心肌病的诊断效率,应选用cTnT与NT-proBNP联合检测方案。     

心肌淀粉样变性临床特点及远期预后的影响因素

目的探讨心肌淀粉样变性患者的临床特点及其远期预后的影响因素。方法回顾性分析119例临床确诊心肌淀粉样变性患者的临床资料,电话随访患者的生存状态,分析患者的临床表现、心电图、心脏超声及心脏磁共振特点及其与远期预后的关系,主要观测终点为全因死亡。应用SPSS 17.0统计软件进行数据分析。结果 119例患者首发症状多样,以气短喘憋和胸闷为主,其次为下肢水肿和乏力,心功能多为美国纽约心脏病学会心功能分级Ⅲ～Ⅳ级。患者1年生存率50%,5年生存率仅为25%,全因死亡率的独立影响因素包括脑利钠肽前体(NT-proBNP)、肌钙蛋白T、糖抗原125(CA125)、白蛋白、血氯、免疫球蛋白M水平。结论心肌淀粉样变性患者预后差,1年生存率仅为50%。患者全因死亡率的独立影响因素包括NT-proBNP、肌钙蛋白T、CA125、白蛋白、血氯、免疫球蛋白M水平。     

以体循环淤血为主要表现的淀粉样变性心肌病临床特点分析

目的分析探讨淀粉样变性心肌病的临床特点。方法对7例淀粉样变性心肌病患者行超声心动图、X线胸片、电子计算机断层摄影术(CT)及常规血生化检查,分析归纳其临床特点、超声表现及辅助检查结果。结果7例患者,2例猝死,其中1例患者曾用免疫抑制剂及激素治疗,一度好转,后猝死。结论对55岁以上男性患者,出现体循环淤血的限制型心肌病表现时,超声心动图表现为左心室对称性或非对称性心肌肥厚伴收缩功能障碍,而心电图呈低电压,应高度怀疑淀粉样变性心肌病。     

15例心脏淀粉样变性患者的临床特点和预后分析

心脏淀粉样变性是由于淀粉样物质沉积于心脏 ,造成心功能不全和心律失常的一种少见的继发性心肌病 ,预后极差。上海仁济医院住院确诊的淀粉样变性患者 2 5例 ,其中 15例有心脏损害。现对其临床特点及预后进行分析。对象与方法1.对象 :2 5例患者 ,其中 15例 (60 % )有心脏淀粉样变性的表现。全部病人均经肾、胃肠道或腹壁脂肪活检证实。2 .方法 :对 15例患者的临床症状、体格检查、心电图、动态心电图、心脏彩超、心室晚电位全面分析。预后随访采用对出院病人定期信件或电话联系 ,了解其病情进展 ,是否存活 ,死亡原因及方式 (猝死或非猝死 )…     

老年扩张型心肌病20例误诊分析

老年扩张型心肌病临床并不罕见,近年有增加趋势,误诊较多。现将近5年来,我院收治的20例老年扩张型心肌病误诊患者分析如下。1临床资料20例患者中男14例,女6例,平均65岁,误诊时间15d~16个月。误诊情况:误诊为冠心病14例,其中冠心病心绞痛12例,急性心肌梗死2例;风湿性心脏病3例;慢性肺心病2例;高血压性心脏病1例。上述病例全部进行胸片、心电图、超声心动图检查,根据《实用内科学》关于扩张型心肌病的诊断标准[1]而确诊。2讨论老年扩张型心肌病病因迄今未明。主要病理生理改变是心肌重量增加,单侧或双侧心腔扩大,瓣膜关闭不全,心肌收缩力下降,…     

心肌淀粉样变诊断进展

心肌淀粉样变（Cardiacamyloidosis,CA）是系统性淀粉样变性最常见的临床表现之一,临床上常以合并症或者单独发病的形式被发现。淀粉样变性可累及许多器官并最终导致器官衰竭,累及心脏时预后极差,因此有淀粉样变性疾病的患者均应排除CA。CA系淀粉样蛋白质物质沉积在心肌组织内,改变细胞代谢、钙转运、受体调节和细胞水肿等所致的一种限制性心肌病。CA较为少见,且临床表现多样化及缺乏特异性,易将其漏诊或误诊为肥厚型心肌病或其他病因的限制性心肌病。     

心肌淀粉样变性4例临床特点分析

目的探讨心肌淀粉样变性的临床特点及其诊治方法。方法对确诊的4例心肌淀粉样变性患者的临床资料进行回顾性分析。结果 4例临床表现均以充血性心衰、心律失常、低血压为主;均出现血清N末端B型利钠肽原水平增高,3例出现肝肾功能异常,1例行血尿轻链水平检查出现增高。4例心电图均为低电压表现,心脏超声均表现为左室壁增厚。4例腹壁脂肪组织活检刚果红染色均为阳性。1例行硼替佐米及地塞米松化疗者第1疗程结束后复查血轻链水平降至正常,其余3例未行化疗者均于就诊后4~8个月死亡。结论心肌淀粉样变性具有特征性的心电图和心脏超声表现,脂肪组织病理检查可确诊。应主要针对淀粉样变性进行治疗。     

68例Wellens综合征临床分析

目的探讨Wellens综合征(WS)的临床表现、心电图特点及误诊原因。方法回顾性分析2013年1月~2015年7月于解放军第二五四医院心内科行冠状动脉造影(CAG)的68例WS患者的心电图、超声心动图、心肌标记物;统计误诊疾病类型、数量。结果 (1)临床情况:男性47例,女性21例;接受冠状动脉介入术58例,冠状动脉旁路移植术6例,药物治疗4例;3例进展为前壁心肌梗死。(2)病变均位于前降支近中段,狭窄50%者0例,50%~70%者4例(5.9%),70%~90%者12例(17.6%),90%的52例(76.5%)。(3)心肌标记物:肌钙蛋白I(c TNI)升高24例,平均峰值:0.72±0.26 ng/ml;N端脑钠肽前体(NT-pro BNP)平均峰值384.02±192.89 pg/ml。(4)心电图:T波倒置52例(76.5%)、双向30例(44.1%)、T波倒置及正负双向均出现14例(20.6%)。(5)超声心动图:37例室壁运动障碍。(6)误诊:心脏神经官能症5例,应激性心肌病4例,心包炎、肺栓塞、反流性食管炎、咽炎各1例。结论 WS心电图表现为孤立性T波改变,病变位于前降支近中段;部分患者c TNI、NT-pro BNP出现升高;对心电图特点认识不足是误诊的主要原因。     

肌钙蛋白T与N端B型尿钠肽前体对急性肺栓塞患者危险分层的意义

目的探讨联合检测生化标记物肌钙蛋白T（cTnT）和N端B型尿钠肽前体（NT—proBNP）水平对急性肺栓塞（APE）患者进行危险分层及预后判断的临床意义。方法根据血浆cTnT和NT—proBNP水平将59例APE患者分为3组：1组（14例）,cTnT〈0．1ng／ml,NT—proBNP〈100pg／ml;2组（28例）,cTnT≥0．1ng／ml或NT—proBNP≥100pg／ml;3组（17例）,cTnT≥0．1ng／ml且NT-proBNP≥100pg／ml,分析cTnT和（或）NT—proBNP升高对APE患者危险分层与临床预后的关系。结果三组间动脉血PaO2、P（A—a）O2比较差异有统计学意义（P〈0．01）。进行两两比较,1、3组动脉血PaO2、P（A—a）O2分别与其他各组相比差异有统计学意义（P〈0．01）。1组、2组、3组预后不良者分别为0（0％）、7例（25．0％）、9例（52．9％）,差异有统计学意义（P〈0．01）。59例APE患者中临床不良事件发生组与无临床不良事件组比较,PaO2、cTnT、NT—proBNP水平差异均有统计学意义（P〈0．01）。结论联合检测cTnT和NT—proBNP在APE患者早期危险分层、指导临床决策及预后判断中具有重要价值。     

Do clinical diagnoses correlate with pathological diagnoses in cardiac transplant patients? The importance of endomyocardial biopsy

BACKGROUND:

Heart transplantation remains the last treatment option for patients with end-stage cardiac disease. Such diseases include ischemic cardiomyopathy, nonischemic cardiomyopathy and other conditions such as arrhythmogenic right ventricular dysplasia, cardiac sarcoidosis and cardiac amyloidosis.

OBJECTIVE:

To review the changes that have occurred over time in the etiology of heart disease in patients requiring heart transplantation, and to compare the clinical and histological diagnoses of explanted hearts from patients with progressive cardiac disease.

METHODS:

The pathological findings of 296 surgically excised hearts over a 20-year period (January 1987 to July 2006) at one institution were examined. Patients were separated into groups based on year of heart transplantation. The tissue was examined to determine the underlying cardiac pathology leading to congestive heart failure. Patient records were reviewed for preoperative clinical diagnoses and other relevant data, including pretransplant endomyocardial biopsy (EMB) results, information regarding left ventricular assist devices and, finally, evidence of disease recurrence in the grafted heart.

RESULTS:

A shift in the underlying etiology was found in patients who underwent heart transplantation from 1992 to 1996, and 1997 to 2001. Between 1987 and 1997, the majority of transplant cases consisted of ischemic cardiomyopathies. From 1997 to 2001, the majority of patients had nonischemic cardiomyopathies, and this trend continued to 2006. A majority of patients with ischemic and hypertrophic cardiomyopathy were diagnosed correctly (96.5% and 82%, respectively) before transplantation. Most patients diagnosed post-transplant with lymphocytic (viral, 15%), hypersensitive/eosinophilic (25%) and giant cell (100%) myocarditis, arrhythmogenic right ventricle dysplasia (100%), cardiac sarcoidosis (83%) and iron overload toxicity-associated cardiomyopathy (100%) had been misdiagnosed in pre-transplantation investigations. Investigations before transplantation did not include an EMB. Of all 296 patients, 51 patients (17%) were misdiagnosed. Excluding the patients with ischemic cardiomyopathy, 46 of 152 patients (30%) were misdiagnosed before transplantation.

CONCLUSIONS:

Although cardiac transplantation is a viable treatment option for patients with a variety of cardiac diseases, accurate diagnosis of patients before transplantation remains a priority. Accurate diagnosis of particular diseases (sarcoidosis, myocarditis, iron toxicity-associated cardiomyopathy and others) allows for proper treatment before transplantation, which may slow down disease progression and improve patient outcomes. Furthermore, it is important to accurately diagnose patients with diseases such as sarcoidosis, amyloidosis and particular types of myocarditis because these can readily recur in the grafted heart. The risk for recurrence must be known to practitioners and, most importantly, to the patient. We strongly recommend the use of EMB if a nonischemic cardiomyopathy is suspected, because the results may alter the diagnosis and modify the treatment strategy.     

老年冠心病漏诊和误诊临床分析

目的分析老年冠心病误诊的原因,以减少误诊。方法采用回顾方法分析2007年1月至2009年12月17例发生冠心病漏诊和误诊患者的临床资料。结果 17例患者中冠心病误诊为消化道疾病5例,咽炎1例,心神经官能症1例,颈椎病1例;其他疾病误诊为冠心病的有带状疱疹3例,胆囊炎2例,心尖部肥厚性心肌病1例,主动脉夹层动脉瘤1例,早期复极综合征2例。结论老年冠心病患者临床表现不典型,同时身患多种疾病,需全面分析、仔细鉴别,及时进行相关检查,减少误诊。     

Osteopontin: a novel predictor of survival in patients with systemic light-chain amyloidosis

Abstract

Background: Troponin-T (cTnT) and NT-proBNP provide prognostic information in light-chain amyloidosis (AL). Thus, these biomarkers are widely used in clinical routine for risk stratification. Recently, plasma level of osteopontin (OPN), a secreted phosphoglycoprotein expressed by a variety of cell types, has been reported as a risk predictor in various cardiovascular diseases.

Methods: OPN was determined retrospectively in 150 consecutive patients newly diagnosed with AL amyloidosis. All patients were evaluated according to a routine protocol including electrocardiography, echocardiography and laboratory testing.

Results: Mean OPN was 591?±?37?ng/mL. Cardiac involvement was established in 83 (55.3%). Median OPN plasma level were associated with number of organs involved, renal function, eligibility for high-dose melphalan chemotherapy and autologous stem cell transplantation, and severity of cardiac amyloidosis. Median follow-up was 19.2 months. 1-year all-cause-survival was 83.4%. The cut-offs discriminating 1-year all-cause-mortality for NT-proBNP, troponin T, and OPN were 2544?ng/L, 0.035?µg/L, and 426.8?ng/mL, respectively. Outcome was worse in patients with biomarkers above the individual ROC derived cut-off. A significant improvement of survival was observed in patients with cTNT >0.035?µg/L or NT-proBNP >2544?ng/L and OPN below ROC-derived cut-off of 426.8?ng/mL as compared to patients with OPN above 426.8?ng/L. No further discrimination was achieved by OPN in the cohorts of low troponin T or low NT-proBNP, respectively. Separate multivariate models identified OPN (cut-off 426.8?ng/mL) and troponin T (cut-off 0.035?µg/L) as independent predictors of all-cause-mortality.

Conclusions: These data demonstrated that OPN appears to be a valuable marker in the clinical routine for evaluation of patients with AL amyloidosis, especially if it is used in combination with cTNT and/or NT-proBNP.     

A comparison of troponin T and troponin I as predictors of cardiac events in patients undergoing chronic dialysis at a Veteran's Hospital: a pilot study.

OBJECTIVE: The purpose of this study was to prospectively evaluate the usefulness of the cardiac troponins as predictors of subsequent cardiac events in patients with chronic renal failure undergoing dialysis. BACKGROUND: Cardiac troponin T (cTnT) and I (cTnI) are cardiac markers that are specific for cardiac muscle. They are also excellent prognostic indicators for patients presenting with chest pain. Although cardiac disease is the leading cause of death in dialysis patients, standard methods to diagnose acute coronary syndromes in patients with renal failure are often misleading. METHODS: A six-month prospective study was done in a university-affiliated Veterans Hospital's dialysis clinic. Forty-nine patients undergoing chronic dialysis with no complaints of chest pain were followed for cardiac events occurring in the six months after cardiac troponin measurements. These included unstable angina, acute myocardial infarction and cardiac death. An additional 83 patients with renal failure but who were not undergoing dialysis were also examined. RESULTS: Within six months all three dialysis patients with elevated cTnI at entry into the study suffered an adverse complication (specificity and positive predictive value = 100%). Twenty-five patients had cTnT elevated at >0.10 ng/ml (53%). Patients with diabetes were more likely to have elevated troponin T levels (64% vs. 25%, p = 0.01). All six patients developing cardiac events within three months had elevations of cTnT >0.1 ng/ml (sensitivity = 100%). Whereas the specificity of cTnT was only 56% for a near-term cardiac event, the negative predictive value of cTnT using a cutoff of < or = 0.1 ng/ml was 100%. On restratifying patients using a cutoff value of cTnT of >0.2 ng/ml, only nine of 49 dialysis patients (18%) had elevated levels. In patients with renal failure not undergoing dialysis, only three of 83 (4%) had elevated troponin I or T. None of these patients suffered a cardiac event in the next six months. CONCLUSIONS: This prospective pilot study clearly delineates the troponins as important prognosticators in asymptomatic otherwise "stable" patients on chronic dialysis, especially those with concomitant diabetes mellitus. It also appears that troponins are more likely to be elevated in dialysis patients than other patients with renal failure not on dialysis. The above suggests that the combination of cTnI and cTnT might be very effective in elucidating cardiac risks of patients with renal failure undergoing chronic dialysis.     

Association of troponin T, detected with highly sensitive assay, and outcomes in infective endocarditis

Troponin levels have been correlated with adverse outcomes in multiple disease processes, including congestive heart failure, acute coronary syndromes, sepsis, and, in a few small series, infective endocarditis. We hypothesized that a novel measurement of troponin using a highly sensitive assay would correlate with adverse outcomes when prospectively studied in patients with infective endocarditis. At a single center in the International Collaboration on Endocarditis, 42 patients met the inclusion criteria and underwent testing for cardiac troponin T (cTnT) using both a standard and a highly sensitive precommercial assay. The cTnT levels were associated with the prespecified primary composite outcome of death, central nervous system event, and cardiac abscess. Secondary outcomes included the individual components of the composite outcome and the need for cardiac surgery. A receiver operating characteristic curve was derived and used to identify the optimal cutpoint for cTnT using the highly sensitive assay. cTnT was detectable with the highly sensitive assay in 39 (93%) of 42 patients with infective endocarditis and with the standard assay in 25 (56%) of 42 (p <0.05). Of the 42 patients, 15 experienced the composite outcome, 4 died, 9 had a central nervous system event, and 5 had a cardiac abscess. With the hs-cTnT assay, the median cTnT was greater in the patients who experienced the primary outcome (0.12 vs 0.02 ng/ml, p <0.05). According to the receiver operating characteristic curve analysis (area under the curve of 0.74), cTnT levels of ≥0.08 ng/ml produced optimal specificity (78%) for the primary outcome. The patients with a cTnT level of ≥0.08 ng/ml were more likely to experience the primary outcome (odds ratio 7.0, 95% confidence interval 1.7 to 28.6, p <0.01) and a central nervous system event (odds ratio 9.3, 95% confidence interval 1.3 to 24.1, p = 0.02). In conclusion, cTnT is detectable in 93% of patients with infective endocarditis using a novel highly sensitive assay, with higher levels correlating with poor clinical outcomes.     

Usefulness of serum hepatocyte growth factor for the diagnosis of amyloidosis

OBJECTIVE: The diagnosis of amyloidosis still relies on biopsy, but there has been a growing demand for the development of a specific noninvasive diagnostic technique. Hepatocyte growth factor (HGF) acts on a variety of epithelial cells in multiple ways and is predominantly produced by mesenchymal cells and macrophages. In the present study, we measured the serum HGF level in patients with amyloidosis and investigated its usefulness for the diagnosis of this disease. METHODS: The subjects were 18 patients diagnosed as having amyloidosis by biopsy. We also measured serum HGF in 47 patients with chronic glomerulonephritis, 32 patients on hemodialysis, and 24 healthy volunteers. The serum HGF level was measured using an HGF ELISA kit. RESULTS: The serum HGF level of patients with amyloidosis was significantly increased compared with that of healthy volunteers, patients with chronic glomerulonephritis, and hemodialysis patients (2.26+/-2.73 ng/ml versus 0.20+/-0.04 ng/ml, 0.23+/-0.08 ng/ml, and 0.18+/-0.07 ng/ml respectively, p<0.0001). There was no significant difference between amyloid light-chain and amyloid A amyloidosis, but the serum HGF level of amyloidosis patients who died within 1 year of measurement was significantly higher than that of patients who lived for more than 1 year (2.83+/-2.85 ng/ml versus 0.49+/-0.26 ng/ml, p<0.01). CONCLUSIONS: The serum HGF level was significantly elevated in both amyloid light-chain and amyloid A amyloidosis and was a very useful indicator of suspected amyloidosis as well as a potential prognostic indicator. The serum HGF level may become a useful indicator for diagnosing amyloidosis.     

Combined measurements of cardiac troponin T and N-terminal pro-brain natriuretic peptide in patients with heart failure.

BACKGROUND: To examine the prognostic contribution of combined cardiac troponin T (cTnT) and N-terminal pro-brain natriuretic peptide (NT-proBNP) in patients with heart failure (CHF) in the absence of acute coronary syndrome. METHODS AND RESULTS: Between July 2001 and March 2002, 71 consecutive patients (mean age = 68.4+/-1.4 years, 37 men), hospitalised for heart failure, were studied during hospitalisation and follow up until December 2002. Serum cTnT and NT-proBNP were measured on admission. Actuarial rates of adverse cardiac events, including sudden or CHF death, or rehospitalisation for CHF during follow up were compared with patients grouped according to initial serum cTnT and/or NT-proBNP concentrations. The adverse cardiac event-free rate among the 20 patients with cTnT > or 0.01 ng/ml was significantly lower than the 51 patients with cTnT <0.01 ng/ml (P<0.05). Similarly, the adverse cardiac event-free rate among the 36 patients with NT-proBNP > or =1,357 pg/ml (median) was significantly lower than the 35 patients with NT-proBNP <1,357 pg/ml (P<0.01). The 16 patients with high concentrations of both cTnT and NT-proBNP had a lower adverse cardiac event-free rate than the 31 patients with low cTnT and low NT-proBNP upon commencement of the study (P<0.005). CONCLUSION: Measurements of serum cTnT and NT-proBNP were reliable prognostic markers of adverse cardiac event in patients with CHF.     

Minor myocardial damage detected by troponin T is a powerful predictor of long-term prognosis in patients with acute decompensated heart failure

BACKGROUND: The progression of chronic heart failure (CHF) is characterized by frequent exacerbation requiring hospitalization and high mortality. Clinical deterioration is triggered by many factors that could promote ongoing myocytes injury. We sought to determine whether a specific marker of cardiac injury, troponin T (cTnT), is associated with prognosis in acute decompensated heart failure (ADHF). METHODS: One hundred and eighty-four consecutive patients with ADHF were enrolled in the absence of an acute coronary syndrome. A cTnT value> or =0.1 ng/ml in samples drawn at 6, 12 or 24 h after hospital admission was considered abnormal. RESULTS: Increased levels of cTnT were found in 58 patients (31.5%, group 1). There were no significant differences between group 1 and patients with cTnT<0.1 ng/ml (group 2) in terms of demographic and clinical characteristics, although ischemic etiology was more prevalent in group 1 (51.7% vs. 31.7%, p=0.009). During follow-up, the mortality in groups 1 and 2 was 31% and 17.5% (p=0.038, OR=2.13, 95% CI: 1.03-4.69), respectively. The 3-year free-CHF readmission survival in group 1 and 2 was 25% and 53% (log rank test p=0.015). In a Cox proportional hazard model, poor tissue perfusion (HR=2.46, 95% CI=1.31-4.6), previous infarction (HR=1.99, 95% CI=1.02-3.9) and cTnT> or =0.1 ng/ml (HR=1.74, 95% CI=1.05-2.9) emerged as the independent predictors of long-term outcome. CONCLUSIONS: One third of patients with decompensated CHF had elevated levels of cTnT. Troponin T was an independent long-term prognostic marker of morbidity and mortality and it suggests a role of biochemical risk stratification in this setting.