心肌淀粉样变性的临床表现及MRI特征

目的 探讨心肌淀粉样变性临床表现及MRI特征.方法 回顾性分析5例经组织病理检查确诊的心肌淀粉样变性患者,其中3例经心脏移植,2例经心内膜活检证实.5例患者均经心电图、X线胸片、超声心动图和MR检查.结果 5例均确诊为心肌淀粉样变性,男女比例为4∶1,5例患者均有心电图异常;X线胸片3例有肺淤血,5例心脏增大,3例伴有少至中、大量胸腔积液;超声心动图示心房扩大,左心室壁肥厚,心室舒张受限,部分出现心肌回声增强;MRI表现为左心室壁普遍增厚,但以室间隔增厚为主,左心室射血分数32.5%±15.0%,限制性心室舒张期充盈受限;延迟增强MRI 4例表现为全心心内膜下为主的弥漫性粉尘样强化,同时累及乳头肌,其中3例患者室间隔左、右心室壁心内膜下均出现强化,在室间隔形成"斑马征",1例表现左心室透壁性强化,自内向外强化程度渐弱.结论 MRI"一站式"扫描能够全面显示心肌淀粉样变性心脏结构和功能变化,心肌弥漫性粉尘样强化和"斑马征",在心肌淀粉样变性诊断中具有很高的参考价值.     

心肌淀粉样变性MRI及临床特征分析(附4例报道)

目的综合分析临床和/或病理学确诊的心肌淀粉样变性(cardiac amyloidosis, CA)患者的临床特征、心电图(electrocardiogram, ECG)、心脏超声(echocardiology, UCG)、心脏磁共振(cardiac magnetic resonance, CMR),加强对该疾病综合认识,进一步指导临床治疗及科学研究。方法收集4例经心内膜下活检、肾脏、骨髓穿刺活检及临床随访直接或间接证实的心肌淀粉样变性患者,综合分析其影像学及临床特征。结果 4例患者CMR表现为左心室扩大,左心室壁弥漫性均匀增厚并舒张运动受限,延迟增强呈弥漫性心内膜下粉尘样强化3例;表现为双心室扩大,双心室壁弥漫性均匀性增厚并舒张运动受限,延迟增强呈弥漫性心内膜下强化1例。同时,4例均伴有不同程度的房室瓣返流及心包积液。结论心肌淀粉样变性的MRI表现具有特征性,结合临床表现、ECG、UCG等可做出准确诊断,为临床针对性治疗提供可靠依据。     

淀粉样心肌病的诊断

目的 探讨淀粉样心肌病的诊断方法.方法 分析2例表现为难治性右心衰竭患者,分别采用右心导管和心内膜活检等方法明确诊断.结果 2例患者心脏超声和MRI均提示室间隔增厚、心肌回声和延迟扫描增强,右心导管检查提示右心压力增高,心内膜活检刚果红染色阳性.结论 心脏症状通常是系统性淀粉样变性的第一表现,部分患者表现为难治性心力衰竭,右心导管和心内膜活检可以明确诊断.     

超声心动图诊断模型在鉴别心肌淀粉样变性与其他原因所致心肌肥厚中的应用

目的 采用LASSO回归整合多种超声参数建立诊断模型,探讨其对心肌淀粉样变性(CA)与其他心肌肥厚疾病的鉴别诊断价值.资料与方法 纳入11例CA左心室增厚、27例非CA左心室增厚以及22例心肌正常的受试者(对照组).比较3组间左心室舒张末期内径、左心室收缩末期内径、左心房前后径、左心室射血分数(LVEF)、左心室室壁厚...     

儿童支原体肺炎所致心脏损害的超声诊断价值

目的:探讨儿童支原体肺炎所致心脏损害的超声诊断价值。方法:选取支原体肺炎所致心脏损害与正常对照者各68例作为研究对象,分析其超声心动图表现。结果:支原体肺炎组心脏有不同程度超声改变:左房轻度扩大43例,占63.2%;左室轻度扩大32例,占47%;室间隔中下段轻度变薄,回声增强,运动幅度减低29例,占42.6%;心包粘连增厚心包积液43例,占63.2%,支原体肺炎并心肌损害组心脏收缩及舒张功能比对照组减低,差异有统计学意义(P0.01)。经过抗肺炎支原体感染及保护心肌治疗后,分别在第一个月、第二个月复查,心功能恢复正常。结论:超声心动图可及时发现支原体肺炎合并心肌损害辅助诊断,并有利于随访观察,指导治疗,判断预后。     

心脏磁共振成像在左心室肥厚病变鉴别诊断中的价值

目的探讨对比增强心血管磁共振成像(CE-CMR)在肥厚型心肌病、高血压及主动脉瓣狭窄性心脏病、心肌淀粉样变性及扩张型心肌病失代偿期鉴别诊断中的作用。方法回顾性分析2010年1月～2016年5月收治56例左室肥厚患者的MRI表现,所有病例均行CE-CMR检查,定量测量各节段舒张末期室壁厚度,观察心肌延迟强化的部位、程度及范围。结果 36例肥厚型心肌病,其中27例表现为非对称性左室肥厚,9例表现为对称性左室肥厚;20例高血压及主动脉瓣狭窄性心脏病、心肌淀粉样变性及扩张型心肌病失代偿期均表现为左室壁弥漫性增厚。延迟增强MRI 36例肥厚性心肌病组共有28例患者可见左心室壁延迟强化,其中25例为局限右心室游离壁与室间隔的连接处、或心室最厚处的斑片状强化;13例高血压及主动脉瓣狭窄组有6例可见左心室壁斑片状延迟强化;6例心脏淀粉样变性组均有左心室壁弥漫延迟强化;1例扩张性心肌病失代偿期表现为室壁间条片状强化。结论对比增强心血管磁共振成像能够为左室肥厚病变提供有价值的资料,钆对比剂延迟强化能够为病变提供有效的鉴别诊断信息。     

心脏MRI在诊断肥厚型心肌病中的应用价值

目的：分析肥厚型心肌病的MRI与超声心动图征象，探讨MRI对肥厚型心肌病的诊断价值。方法：对31例拟诊为肥厚型心肌病患者行心脏MRI、超声心动图检查，对比2种检查方法对心肌肥厚程度的测量结果。结果：31例中，均发现心室壁不同程度肥厚。其中，非对称性室间隔肥厚17例（54.8%），左心室普遍肥厚3例（9.7%），室间隔增厚并累及右心室心尖部2例（6.5%），左心室游离壁局限性肥厚3例（9.7%），心尖肥厚型6例（19.4%），可见典型"黑桃尖"表现。梗阻型肥厚型心肌病2例，收缩期二尖瓣前叶向前运动；伴感染性心内膜炎1例；伴左心室心尖部室壁瘤1例。全部患者均行心肌对比增强扫描，20例（64.5%）肥厚心肌内可见点状、片状心肌中层延迟强化，5例非肥厚心肌内亦可见延迟强化。1例累及右心室心尖部及3例心尖肥厚型心肌病超声心动图诊断为阴性，室壁厚度测量结果低于MRI（P<0.05）。结论：MRI诊断肥厚型心肌病具有很高的准确性，超声心动图诊断心尖肥厚型心肌病敏感性低；对于临床怀疑肥厚型心肌病，特别是心尖肥厚型心肌病的患者可采用心脏MRI明确诊断。     

心内膜弹力纤维增生症的超声诊断

目的:探讨心内膜弹力纤维增生症(endomyocardial fibroelastosis,EFE)的超声心动图诊断及鉴别诊断价值。方法:搜集EFE 15例,均行超声心动图检查,分析其超声表现及血流动力学变化。结果:二维超声示心内膜回声增强、增厚,厚度一般大于3.5mm,左房室内径增大11例,左右房室均增大3例,房室内径正常1例。二尖瓣轻度返流4例,中度3例,重度5例,无返流3例。11例治疗后房室腔缩小,左室功能(EF)改善,4例因病情较重死亡。结论:心内膜增厚、回声增强伴左房室腔扩大为EFE二维超声特征性表现,超声心动图对于EFE患儿的早期诊断、疗效评价具有重要的临床价值。     

心肌致密化不全1例

患者男,72岁。因"痛风",要求心脏超声心动图检查:显示右心房、右心室及主、肺动脉内径正常,左心室内径增大,并见粗大乳头肌束,心尖部增厚,并见此处心肌多个隐窝,搏幅减低(图1)。主动脉瓣回声增强,瓣叶增     

孤立性心肌致密化不全的MRI诊断

目的探讨孤立性心肌致密化不全(INVM)的MRI表现及其对该病诊断的价值。方法12例INVM患者均经超声心动图诊断。采用MRI不同脉冲序列，分析INVM心室结构、运动功能及心肌灌注的MRI表现。结果(1)左心室单独受累9例，双室受累3例，未见右室单独受累者。左心室心尖段及前壁中间段为最常见受累部位(12例)；(2)心肌增厚并能分层，非致密化心肌信号不均匀，由多发、粗大、交错排列的肌小梁及小梁隐窝组成，致密心肌变薄，信号均匀；(3)小梁隐窝内血栓2例；(4)心肌灌注成像首过期，6例14段(14／99段)可见灌注缺损；延迟期左心室心肌强化4例23段(23／99段)，其中1例伴右室心尖心肌强化；(5)左心室普遍收缩功能不良4例，节段性功能不良5例。结论MRI能清晰显示INVM的形态学改变、心室节段性及整体运动功能异常、心肌血流灌注及纤维化的程度和范围，对INVM诊断及预后评估具有重要价值。     

Cardiac amyloidosis: MR imaging findings and T1 quantification, comparison with control subjects

In cardiac amyloidosis an interstitial deposition of amyloid fibrils causes concentric thickening of the atrial and ventricular walls. We describe the results of tissue characterization of the myocardium by T1 quantification and MRI findings in a patient with cardiac amyloidosis. The T1 time of the myocardium was elevated compared to that in individuals without amyloidosis. The T1 time of the myocardium was 1387 +/- 63 msec (mean value obtained from four measurements +/- standard deviation [SD]) in the patient with cardiac amyloidosis, while the reference value obtained from the myocardium of 10 individuals without known myocardial disease was 1083 +/- 33 msec (mean value +/- SD). In combination with other MR findings suggestive of amyloidosis, such as homogeneous thickening of the ventricular and atrial walls, thickening of the valve leaflets, restrictive filling pattern, and reduction of systolic function, T1 quantification may increase diagnostic confidence.     

肥厚型心肌病心功能异常的磁共振电影成像分析

目的 应用磁共振电影成像（ｃｉｎｅ－ＭＲＩ）方法探讨肥厚型心肌病的左心室功能的变化。方法 １２例肥厚型心肌病患者,行ＭＲ快速自旋回波（ＴＳＥ）多层面平扫和ｃｉｎｅ－ＭＲＩ检查,用ＡＲＧＵＳ专用心功能分析软件分析射血分数、心肌增厚率、心腔（左心室）容积及时间－容积变化曲线等参数。结果 （１）肥厚型心肌病主要累及左心室前、侧壁及室间隔,肥厚心肌的心肌增厚率显著低于正常心肌（ｔ＝１５．１,Ｐ〈０．０１）     

Quantitative assessment of myocardial T2 relaxation times in cardiac amyloidosis

Purpose

To evaluate cardiac MRI (CMR) in the diagnosis of cardiac amyloidosis by comparing the T2 relaxation times of left ventricular myocardium in a pilot patient group to a normal range established in healthy controls.

Materials and Methods

Forty‐nine patients with suspected amyloidosis‐related cardiomyopathy underwent comprehensive CMR examination, which included assessment of myocardial T2 relaxation times, ventricular function, resting myocardial perfusion, and late gadolinium enhancement (LGE) imaging. T2‐weighted basal, mid, and apical left ventricular slices were acquired in each patient using a multislice T2 magnetization preparation spiral sequence. Slice averaged T2 relaxation times were subsequently calculated offline and compared to the previously established normal range.

Results

Twelve of the 49 patients were confirmed to have cardiac amyloidosis by biopsy. There was no difference in mean T2 relaxation times between the amyloid cases and normal controls (51.3 ± 8.1 vs. 52.1 ± 3.1 msec, P = 0.63). Eleven of the 12 amyloid patients had abnormal findings by CMR, eight having LGE involving either ventricles or atria and four demonstrating resting subendocardial perfusion defects.

Conclusion

CMR is a potentially valuable tool in the diagnosis of cardiac amyloidosis. However, calculation of myocardial T2 relaxation times does not appear useful in distinguishing areas of amyloid deposition from normal myocardium. J. Magn. Reson. Imaging 2009. © 2009 Wiley‐Liss, Inc.     

Assessment of myocardial viability by MRI.

Assessment of myocardial viability has become an important issue in patients presenting with either acute myocardial infarction or presenting with chronic ischemic left ventricular dysfunction. In patients with viable myocardium recovery of left ventricular function can be anticipatedm, spontaneously in patients with acute myocardial infarction or following revascularization in patients with ischemic cardiomyopathy. In contrast, patients without viable tissue are not likely to improve in left ventricular function. Currently, nuclear imaging techniques and dobutamine stress echocardiography are used for assessment of viability; recent studies with magnetic resonance imaging (MRI) have however demonstrated the potential usefulness of this technique for the assessment of viability. Various parameters, derived from resting MRI, can be used as markers of myocardial viability, including the end-diastolic wall thickness, systolic wall thickening and signal intensity without contrast-enhancement. Other studies have combined the information from resting MRI with the assessment of contractile reserve during dobutamine stimulation. Finally, recent studies have evaluated the use of contrast-enhanced MRI to detect viable myocardium. All of these parameters are potentially useful and MRI provide an alternative approach for the assessment of viable myocardium.     

Multiphasic cardiac magnetic resonance imaging: normal regional left ventricular wall thickening

Magnetic resonance imaging (MRI) is a completely noninvasive method for visualizing cardiovascular anatomy but has had limited use for assessment of cardiac function. The authors evaluated the use of gated MRI for the quantification of regional myocardial contraction. Nine normal subjects underwent gated MRI of five transverse sections (7 mm thickness) through the left ventricle at five intervals in the cardiac cycle using a new technique called rotating gated sequence. All five sections were examined, and the section that best demonstrated the midportion of the left ventricle in its maximum dimension was used to obtain measurement. This technique permitted assessment of regional wall thickening of various regions of the left ventricle in different phases of the cardiac cycle. The extent and percentage of wall thickening were calculated from measurements of the septum and anterior and lateral left ventricular wall in end-diastole and end-systole. The calculated mean values for extent and percentage of wall thickening for the septum were 0.40 cm and 40%; for the anterior wall, 0.61 cm and 73%; and for the lateral wall, 0.53 cm and 57%, respectively. A limitation of the current technique in wall thickness measurements is that the transverse MR plane of section is not perpendicular to the long axis of the left ventricle. Consequently, such oblique sections through the left ventricle may give inaccurate absolute wall thickness measurements but can provide reliable estimate of regional wall thickening dynamics. The ability to define left ventricular wall thickness and function without contrast media provides a noninvasive technique for the detection of segmental left ventricular myocardial dysfunction in ischemic heart disease.     

磁共振成像诊断陈旧性心肌梗塞的初步评价

目的：探讨ＭＲＩ在评价陈旧性心肌梗塞的形态和功能中的应用价值。材料和方法：对７例陈旧性心肌梗塞进行ＭＲＩ检查，观察心脏形态学变化、心室壁运动、二尖瓣和主动脉瓣开闭功能，测量左室功能。结果：左室壁限局性变薄６例，其中３例在变薄的左室壁邻近部位见血流信号异常增高，左室壁节段性运动减弱，１例室壁瘤形成。左室射血分数降低。１例见二尖瓣有轻度返流。增强扫描（２例）显示梗塞心肌与正常心肌的增强有明显差异。结论：ＭＲＩ能为临床提供心肌的局部和整体功能、梗塞心肌的信号改变、心脏的瓣膜功能及心肌灌注等信息     

MRI in the assessment of non ischemic myocardial diseases

Our purpose is to discuss MRI findings in non-ischemic myocardial disease (NIMD). Emphasis is placed on the typical locations and patterns of delayed enhancement. Clinicopathological and MRI features of amyloidosis, arrythmogenic right ventricular dysplasia, left ventricular non-compaction, myocarditis, hypertrophic cardiomyopathy, and dilated cardiomyopathy are discussed. Currently, cardiac MRI is the best imaging method for diagnosis and follow-up of NIMD. In particular, the radiologist should be familiar with the different patterns of delayed enhancement on DE-CMR since they play an important role in the detection and differential diagnosis of NIMD.     

Magnetic resonance imaging for the assessment of myocardial viability

The identification of myocardial viability in the setting of left ventricular (LV) dysfunction is crucial for the prediction of functional recovery following revascularization. Although echocardiography, positron emission tomography (PET), and nuclear imaging have validated roles, recent advances in cardiac magnetic resonance (CMR) technology and availability have led to increased experience in CMR for identification of myocardial viability. CMR has unique advantages in the ability of magnetic resonance spectroscopy (MRS) to measure subcellular components of myocardium, and in the image resolution of magnetic resonance proton imaging. As a result of excellent image resolution and advances in pulse sequences and coil technology, magnetic resonance imaging (MRI) can be used to identify the transmural extent of myocardial infarction (MI) in vivo for the first time. This review of the role of CMR in myocardial viability imaging describes the acute and chronic settings of ventricular dysfunction and concepts regarding the underlying pathophysiology. Recent advances in MRS and MRI are discussed, including the potential for dobutamine MRI to identify viable myocardium and a detailed review of the technique of delayed gadolinium (Gd) contrast hyperenhancement for visualization of viable and nonviable myocardium.     

MR-relaxometry of myocardial tissue: significant elevation of T1 and T2 relaxation times in cardiac amyloidosis

OBJECTIVE: This study evaluates if MR-relaxometry of myocardial tissue reveals significant differences in cardiac amyloidosis (CA) compared with patients with systemic amyloidosis but without cardiac involvement (NCA) and a healthy control group. Therefore, we measured T1 and T2 relaxation times (RT) of the left ventricular myocardium with magnetic resonance imaging at 1.5 T. MATERIAL AND METHODS: Nineteen consecutive patients (14 males, 5 females; mean age, 59 +/- 6.1 years) with histologically proven CA were evaluated. T1-RT and T2-RT were measured by using a saturation-recovery TurboFLASH sequence and a HASTE sequence, respectively. Additionally, morphologic and functional data were acquired. Results were compared with patients with systemic amyloidosis but without cardiac involvement (NCA; 5 males, 4 females, 48.9 +/- 15.4 years) and 10 healthy, age-matched control subjects (5 males, 5 females, 60.4 +/- 6.4 years). RESULTS: MR-relaxometry revealed a significant elevation of T1-RT of the left ventricular myocardium in CA-patients compared with that in NCA-patients and the age-matched control group [mean +/- SD (95% CI) 1340 +/- 81 (1303-1376) msec, 1213 +/- 79 (1160-1266) msec, 1146 +/- 71 (1096-1196) msec, respectively; CA vs. control, P < 0.0001; CA vs. NCA:, P < 0.0003; NCA vs. control, P = 0.07]. T2-RT showed a marginal but significant increase in CA-patients compared with NCA-patients and the control group [mean +/- SD (95% CI) 81 +/- 12 (76-86) msec, 71 +/- 11 (64-79) msec, 72 +/- 9 (65-79) msec, respectively; CA vs. control, P = 0.04; CA vs. NCA, P = 0.04; NCA vs. control, P = 0.91]. T1-RT was best suited to discriminate between the groups as shown by logistic regression. A cut-off value of >or=1273 milliseconds for T1-RT was defined using receiver-operator characteristics-analysis to establish the diagnosis of CA with a high sensitivity (84%) and specificity (>89%). CONCLUSIONS: Measurement of T1 and T2 RT is a novel approach for noninvasive evaluation of CA. MR-relaxometry might improve diagnostic reliability of magnetic resonance imaging for evaluation of cardiac involvement in systemic amyloidosis.