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。     

心肌淀粉样变性的MRI与超声心动图诊断价值

目的 探讨心肌淀粉样变性的MRI与超声心动图诊断价值.方法 回顾性分析11例经病理活检证实的心肌淀粉样变性患者的MR和超声心动图检查结果.结果 MRI和超声心动图示心肌淀粉样变性患者中,11例均存在不同程度的左心室、室间隔轻度增厚,5例房间隔轻度增厚,7例左心室心肌质量增加,7例左心房增大,6例左心室射血分数减低,10例舒张功能减低,多伴随心包积液、胸腔积液.8例MRI延迟增强显示特征性的弥漫性、广泛性心内膜下至透壁性延迟强化.6例超声心动图显示心肌回声增强,呈磨玻璃样改变,并可见散在颗粒样斑点状强回声.结论 超声心动图具有较好的初步诊断价值,延迟增强MRI能为心肌淀粉样变性诊断及鉴别诊断提供更为全面、丰富的信息.     

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。     

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.     

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。     

肥厚型心肌病的心电图与MRI表现分析

目的 探讨肥厚型心肌病(hpertrophic crdiomyopathy,HCM)的心电图和MRI表现.方法 搜集17例经临床确诊的HCM患者资料,对其心电图、MRI表现进行总结分析.结果 17例患者心电图均异常,主要表现为ST-T异常、左束支传导阻滞、左心室高电压、异常Q波、T波倒置.MRI以左心室壁非对称性肥厚、室间隔肥厚居多,其次是心尖肥厚;心腔缩小,心肌信号正常;心脏功能异常(7例).ST段压低深度与心尖部室壁厚度呈正相关,Q波深度与室间隔厚度呈正相关,T波深度与心尖部和室间隔的室壁厚度差呈正相关.结论 HCM的心电图、MRI均有一定的特征性,两者结合、相互补充,可为临床诊断该病提供重要的依据.     

心脏MRI对左心室扩大疾病的鉴别诊断价值

目的探讨心脏MRI在引起左心室扩大疾病中的鉴别诊断价值。方法选取并分析35例左心室扩大患者的MRI表现,测量左心室舒张末期最大横径、左心室壁各节段舒张末期厚度,观察心肌延迟强化表现。结果 8例扩张型心肌病(DCM)均表现为左心室壁普遍变薄,其中6例出现室间隔肌壁间线样强化; 12例缺血性心脏病(IHD)表现为肇事血管供血区心肌变薄,且相应区域出现心内膜下及透壁性强化; 7例病毒性心肌炎(VMC)患者,均出现侧壁心外膜侧强化,其中6例可见室间隔肌壁间线样强化; 6例左心室心肌致密化不全(LVNC)中,2例表现为室间隔肌壁间线样强化,4例无延迟强化;酒精性心肌病(ACM)和围产期心肌病(PPCM)各1例,均表现为左心室壁普遍变薄,无延迟强化。结论心脏MRI能够为左心室扩大疾病提供结构及功能信息,钆对比剂增强能够为这些疾病的鉴别提供有效依据。     

合并左心室受累的致心律不齐性右室型心肌病的组织学和MRI表现

目的：探讨终末期致心律不齐性右室型心肌病（ARVC）组织学特点和MRI特征。方法9例患者接受心脏移植，移植后离体心脏行组织病理学检查。7例移植前行MR扫描。结果：病理学检查显示所有心脏双室受累，右心室腔显著扩张7例，大致正常2例。右心室壁均显示严重透壁性肌肉丧失，其中3例几乎完全被脂肪组织替代，6例几乎完全被纤维脂肪组织替代。左心室中重度扩张8例、轻度扩张1例。左心室游离壁受累者7例，室间隔和左心室游离壁同时受累者2例。5例以脂肪细胞浸润为主伴小灶性纤维化；4例以弥漫性纤维化为主，伴灶性脂肪细胞浸润。7例MRI显示左心室射血分数平均（21．66±7．05）％，左心室轻度扩张3例，中度扩张2例，高度扩张2例。右心室腔明显扩大、壁薄者5例，其中3例可见线状高信号脂肪浸润；其余2例右心室形态、大小及信号均无明显异常，仅突出地表现为左心室受累。2例显示心外膜下脂肪信号浸润，选择性累及左心室，心尖和侧、后壁；3例左心室节段性变薄伴运动功能丧失分别累及室间隔、心尖和侧后壁；4例左心室游离壁变薄，厚度不足5mm。5例心肌灌注延迟显像均表现为不同程度的增强，左心室侧后壁强化者4例，其中透壁性和心外膜下各2例；室间隔肌壁间强化者2例；左心室心尖部强化者2例，灶性和透壁性各1例。4例患者右心室壁亦可见透壁性增强，其中累及右心室游离壁者2例，累及右心室心尖和后壁各1例。结论：ARVC合并左心室受累是该组患者的特点，MRI不仅能够准确地反映ARVC继发性的心室扩张及室壁运动的节段性变化，而且能检出心室壁的脂肪浸润以及纤维化等，因此能够在一定程度上反映生理状态下心脏组织学特征。     

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...     

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。     

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.     

法布里病累及心脏的临床及MR特征分析

目的:探讨法布里病（AFD）累及心脏的临床及心脏MR（CMR）特征。方法:回顾性纳入2018年1月至2021年3月阜外医院经基因检测或病理确诊的AFD患者8例（AFD组）,其中男3例、女5例,年龄26~60（50±11）岁;同时纳入性别、年龄匹配的肥厚型心肌病（HCM）患者16例（HCM组）和健康对照者16名（正常组）...     

磁共振心功能电影序列快速多参数对比评估危重症STEMI患者的扫描技术探索

目的 阐述磁共振心功能电影序列(CINE)在危重症急性ST段抬高型心肌梗死(STEMI)患者扫描及后处理对比分析中的应用价值.方法 选取临床确诊的危重症STEMI患者1例,通过心脏磁共振成像(CMR)序列的快速优化组合,重点对比分析心功能成像序列,分享扫描经验和技术要点.结果 CMR全部检查时间仅为18 min,CIN...     

Quantification of myocardial delayed enhancement and wall thickness in hypertrophic cardiomyopathy: Multidetector computed tomography versus magnetic resonance imaging

Objectives

To evaluate the accuracy of multidetector computed tomography (MDCT) in assessing myocardial delayed enhancement and left ventricle wall thickness in hypertrophic cardiomyopathy (HCM) compared with cardiac magnetic resonance (CMR) as the reference standard.

Materials and methods

Eighty consecutive patients (59 male; 53.2 ± 13.0 years) were examined with MDCT, followed by CMR 1 day later. Cardiac CT angiography and a delayed CT were performed. CMR was performed according to a standardized protocol. Left ventricle wall thickness and positions of myocardial delayed enhancement were identified in both CMR and CT images according to the American Heart Association left ventricle 17-segment model. Myocardial delayed enhancement was characterized as “dense” (areas with clear defined borders) or “diffuse” and then quantified using both techniques.

Results

Left ventricle wall thickness determined by MDCT was significantly correlated with CMR (R = 0.88, P < 0.01). Compared with CMR, MDCT accurately diagnosed 74 of 78 (94.9%) patients and 1243 of 1326 (93.7%) segments. For dense myocardial delayed enhancement, MDCT significantly correlated with CMR (R = 0.88, P < 0.01) and slightly underestimated myocardial delayed enhancement (mean, −3.85%; lower and upper limits of agreement, −13.40% and 5.70%, respectively).

Conclusions

MDCT provides reliable quantification of myocardial delayed enhancement and evaluation of left ventricle wall thickness and has a good correlation with CMR in patients with HCM when a comprehensive cardiac CT protocol is used and can be applied for intervention planning.     

Cardiac MRI and CT features of inheritable and congenital conditions associated with sudden cardiac death

Cardiac MRI (CMR) and electrocardiogram (ECG)-gated multi-detector computed tomography (MDCT) are increasingly important tools in the identification and assessment of cardiac-related disease processes, including those associated with sudden cardiac death (SCD). While the commonest cause of SCD is coronary artery disease (CAD), in patients under 35 years inheritable cardiomyopathies such as hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy are important aetiologies. CMR in particular offers both accurate delineation of the morphological abnormalities associated with these and other conditions and the possibility for risk stratification for development of ventricular arrhythmias with demonstration of macroscopic scar by delayed enhancement imaging with intravenous gadolinium.     

Late Gadolinium Enhancement of Left Ventricular Papillary Muscles in Patients with Mitral Regurgitation

ObjectiveArrhythmogenic mitral valve prolapse (MVP) is an important cause of sudden cardiac death characterized by fibrosis of the papillary muscles or left ventricle (LV) wall, and an association between late gadolinium enhancement (LGE) of the LV papillary muscles and ventricular arrhythmia in MVP has been reported. However, LGE of the papillary muscles may be observed in other causes of mitral regurgitation, and it is not limited to patients with MVP. This study was to evaluate the association of LGE of the LV papillary muscles or ventricular wall on cardiac magnetic resonance imaging (CMR) and ventricular arrhythmia in patients with mitral regurgitation.Materials and MethodsThis study included 88 patients (mean age ± standard deviation, 58.3 ± 12.0 years; male, 42%) with mitral regurgitation who underwent CMR. They were allocated to the MVP (n = 43) and non-MVP (n = 45) groups, and their LGE images on CMR, clinical characteristics, echocardiographic findings, and presence of arrhythmia were compared.ResultsLV myocardial wall enhancement was more frequent in the MVP group than in the non-MVP group (28% vs. 11%, p = 0.046). Papillary muscle enhancement was observed in 7 (7.9%) patients. Of the 43 patients with MVP, 15 (34.8%) showed LGE in the papillary muscles or LV myocardium, including 12 (27.9%) with LV myocardial wall enhancement and 4 (9.3%) with papillary muscle enhancement. One patient with bilateral diffuse papillary muscle enhancement experienced sudden cardiac arrest due to ventricular fibrillation. Univariable logistic regression analysis showed that high systolic blood pressure (BP; odds ratio [OR], 1.05; 95% confidence interval [CI], 1.01–1.09; p = 0.027) and ventricular arrhythmia (OR, 6.84; 95% CI, 1.29–36.19; p = 0.024) were significantly associated with LGE of the papillary muscles.ConclusionLGE of the papillary muscles was present not only in patients with MVP, but also in patients with other etiologies of mitral regurgitation, and it was associated with high systolic BP and ventricular arrhythmia. Papillary muscle enhancement on CMR should not be overlooked.     

Magnetic resonance imaging of acute myocardial infarction in dextrocardia with situs solitus (dextroversion)

The case report of an 88-year-old woman with dextroversion and acute anterior wall myocardial infarction is presented. The patient, who had been diagnosed with dextrocardia 3 years prior to this admission, presented with right-sided chest pain. Coronary angiography demonstrated an 80% proximal left anterior descending artery stenosis which was successfully stented. A cardiac MRI was performed to exclude a left atrial thrombus after an inconclusive echocardiogram. The MRI demonstrated findings consistent with dextroversion, with delayed contrast-enhanced viability sequences confirming a near transmural anterior wall myocardial infarct. To our knowledge, this is the first report illustrating the cardiac MRI findings in such a case.     

Myocardial Fibrosis in Hypertrophic Cardiomyopathy Demonstrated by Integrated Cardiac F-18 FDG PET/MR

Hypertrophic cardiomyopathy (HCM) is a common condition defined as a diffuse or segmental left ventricular (LV) hypertrophy with a nondilated and hyperdynamic chamber as well as cardiac arrhythmias. Cardiac MR (CMR) imaging is a key modality for evaluation of HCM. In addition to the assessment of LV wall thickness, LV function and aortic flow, CMR is capable of estimation of late gadolinium enhancement (LGE) in affected myocardium which has been shown to have a direct correlation with incidence and severity of arrhythmias in HCM. In patients with HCM, LGE on CMR is presumed to represent intramyocardial fibrosis. Meanwhile, F-18 FDG myocardial PET has been sporadically studied in HCM, mostly for evaluation of the metabolic status of a hypertrophic myocardial segment, especially after interventions or to demonstrate partial myocardial fibrosis. We presented here the case of a 25-year-old male patient referred for simultaneous F-18 FDG cardiac PET/MR for the evaluation of septal hypertrophy. The PET/MR revealed myocardial fibrosis in the septum associated with FDG-defect and LGE.     

Cardiovascular magnetic resonance reveals similar damage to the heart of patients with becker and limb‐girdle muscular dystrophy but no cardiac symptoms

Cardiac involvement in patients with a sarcoglycanopathy (limb‐girdle muscular dystrophy) has been described previously; however, this is the first cardiovascular magnetic resonance (CMR) study in such a patient demonstrating an interesting pattern of myocardial damage using late gadolinium enhancement (LGE) imaging. Moreover, the wall motion abnormality and the subepicardial pattern of LGE in this patient with a sarcoglycanopathy is in agreement with the findings in another patient with Becker muscular dystrophy. The predominance of LGE in the subepicardial layers of the left ventricular inferolateral wall suggests that such a myocardial damage pattern represents a nonspecific cardiac phenotype in response to exaggerated mechanical stress in this region, at least in patients with a sarcoglycanopathy or dystrophinopathy. J. Magn. Reson. Imaging 2009;30:876–877. © 2009 Wiley‐Liss, Inc.