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27y/oFemalewitharrhythmiaandabnormalEKGECGgatedaxialspin-echoT1WI.36Arrhythmogenicrightventriculardysplasia36致心律失常性右心室发育不良(ARVD)•又称致心律失常性右室心肌病(ARVC)•以右心室心肌进行性脂肪或纤维脂肪组织替代为特征,以右心室形态与功能异常为主,伴有心脏电生理改变及遗传特征的心肌疾患•通常脂肪从心外膜向心肌层浸润,严重者可全层替代,导致心肌变薄,呈“羊皮纸样”改变。右室流出道、心尖部和下壁为其好发部位,称之为“心肌发育不良三角区”。病变晚期可累及左心室,但室间隔较少受累•临床表现为右心室扩大、心律失常和猝死MR表现•右心室扩张和室壁变薄早期右心室流出道扩张,典型者右心室腔明显扩张,室壁普遍变薄,严重者厚度不足2mm,呈“羊皮纸”样改变。•室壁运动功能异常MRI电影序列于右心室心尖部和下壁可见单个或多个瘤样凸出。有时候右室流出道和/或右室游离壁三尖瓣下区域有特征性的局部皱缩,在收缩期表现更加明显,称之为“手风琴征”。•基于脂肪或/和纤维脂肪浸润的组织特定性无论在T1WI还是T2WI上,脂肪组织均表现为高信号,且信号强度可被抑脂序列所抑制,通常以右心室游离壁及右心室流出道最明显。左室壁脂肪浸润最常见的部位为心尖和侧后壁。在对比剂灌注延迟扫描序列上,纤维组织因强化亦表现为高信号。25y/omanwithCooley’sanemia.T2-weightedTSE-BBimageBTFEimage37Myocardiumironoverload37Ironoverloadcardiomyopathy•IOCreferstoasecondaryformofcardiomyopathyresultingfromtheaccumulationofironinthemyocardium.•Itoccursmainlyduetogeneticallydetermineddisordersofironmetabolism(e.g.cardiomyopathyinhaemochromatosis(血色病),thalassaemia(地中海贫血)ormultipletransfusions.CardiacMRI•CMR-derivedT2*relaxationtimeiscurrentlythemainstayforthequantitativeassessmentofcardiacirondeposition.•Measuredinafull-thicknessareaofinterestintheinterventricularseptum,T2*ishighlyrepresentativeofglobalmyocardialiron.•Avalueof20msisconsideredtobethethresholdformyocardialsiderosis(铁沉着病).59y/omanwithhypertension.38Myocardialbridging.381127846225m/o,M2y/oboy,Progressiveabdominalfullnessnotedinrecent2weeks43Lymphoma43043506393m/o,M3m/oboy,Progressiveabdominaldistensionnotedforonemoremonths.DelayedCT(+)DelayedCT(+)CT(-)44Hemangioendothelioma44肝上皮样血管内皮瘤•肝上皮样血管内皮瘤是介于血管瘤和血管肉瘤之间的一种具有潜在恶性的低度恶性间质肿瘤,具有上皮样细胞和血管内皮细胞组织学特征,生长缓慢,转移率低,其中最常见肺转移•发病率低,发病以女性为主,可能与口服避孕药、孕激素失调有关•多数AFP正常•组织学特征:浸润肝窦及肝内静脉系统,肿瘤围绕肝静脉、门静脉或小静脉并使其狭窄或闭塞。CT表现•多见于肝右叶边缘结节或肿块影,可单发或多发,平扫为低密度,中心更低密度,可有钙化;动脉期周边强化,静脉期及延迟期强化范围扩大,而中央低密度区无强化,肝静脉或门静脉行向这些病灶时逐渐变细并终止于这些病灶边缘,形成类似棒棒糖样的外观•棒棒糖征主要包括两个结构:一是静脉期呈低密度边界清楚的肿块,代表棒棒糖中的糖果;二是在组织学上闭塞的静脉,包括肝静脉和门静脉,代表棒棒糖中的棒子MR表现•T1WI:结节多表现为低信号,T1增强呈边缘强化,正常肝实质强化较肿瘤强化明显,肿瘤中心部分与CT扫描类似呈低信号•T2WI:肿瘤多表现为不均匀高信号,肿瘤内信号成分较CT更复杂,中心部分信号减低可能与瘤内出血、凝固性坏死及钙化有关AnenhancedCTimageshowingnumerousvariablesizehypodenselargehepaticnodules.Thelesioninsegment6(arrow)demonstrateswell-definedhypodenseareawiththeperipheralenhancementand“cut-off”oftheRHVatitsedge;alollipopsign.Notethecentralhypodensity(a)Anout-of-phaseMRIimageshowsmultipleperipheralliverlesions.Thesmallerlesionintheleftliverlobeisuniformlyhypointensewithatributaryoftheleftportalveinendingatisedge(arrow).Thelargesubcapsularlesionintherightliverlobeismorecomplexandheterogeneous.Centralnecrosisandmarkedcapsularretractionareevident.(b)AT2-weightedimageofthelivershowsanotherlollipopsigncomposedofahyperintensesubcapsularlesionandahepaticveintributary(arrow).
本文标题:病例讨论1.
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