心肌致密化不全专家讲座.pptx

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,概述,心肌致密化不全(noncompaction ventricular myocardium,NVM)又称海绵状心肌(spongy myocardium)，临床上较为少见。近年来国内外对该病逐步认识，年心肌病分类为遗传性心肌病,心肌致密化不全,是一个罕见先天性心肌病，本病是因为胚胎时期疏松心肌组织致密化过程障碍造成小梁化心肌连续存在所致一个先天畸形,解剖学上表现为无数突出肌小梁和深陷小梁间隐窝。,临床表现缺乏特异性，常见表现为心功效不全、心律失常和栓塞。,多见于左心室，也可见于右心室及双心室，可单发或呈家族聚集性。孤立性心肌致密化不全或与其它先天性心脏畸形并存,超声心动图是首选诊疗方法。,心肌致密化不全专家讲座,第1页,病理&病理生理,心脏扩大，心肌重量增加，冠状动脉通畅。主要病变为受累心室腔内多发异常粗大肌小梁和交织深陷隐窝，可不一样程度地累及心室壁内2/3,非致密心肌肌束显著肿大，细胞核异形，纤维组织主要出现在心内膜下，其间可见炎症细胞浸润；外层致密心肌厚度变薄，肌束走行及形态基本正常，细胞核大小均匀,心肌致密化不全专家讲座,第2页,病理&病理生理,进行性心力衰竭系收缩功效及舒张功效受损引发,慢性缺血可能是收缩功效受损主要原因，多个异常隆起肌小梁对血液需求和心脏血供不匹配造成了慢性缺血，及微循环障碍。,致密化不全心肌范围和慢性缺血程度以及伴发疾病决定了病程进展,舒张功效减低是因为粗大肌小梁引发室壁主动弛张障碍和室壁僵硬度增加，顺应性下降,心肌致密化不全专家讲座,第3页,病理&病理生理,心肌致密化不全大部分孤立存在，有时也合并有其它先天性心脏病，如房间隔缺损、室间隔缺损，其它复杂紫绀性先天性心脏病，或冠状动脉畸形、心脏瓣膜畸形等,心肌致密化不全能够合并各种心律失常，包含预激综合征、阵发性室上性心动过速、室性二联律、完全性左束支传导阻滞、完全性房室传导阻滞,机制可能与心肌肌束不规则相互联结及局部冠脉微循环障碍引发电生理紊乱相关。,在一些儿童患者中，能够观察到一些面部畸形，如前额突出、斜视、小面脸、低耳垂等,心肌致密化不全专家讲座,第4页,病理&病理生理,A section has been taken from the interventricular septum,B Low-power view of histologic section showing a deep myocardial recess in close proximity to the epicardial adipose tissue(hematoxylin and eosin stain;Original magnification,40),C Low-power view of histologic section showing multiple trabeculations and myocardial projections,with extensive fibrosis(Klatskin trichrome stain;original magnification,10),D Low-power view of histologic section showing a thickened endocardium overlying the myocardial recesses,with areas of patchy fibrosis(Klatskin stain;original magnification,40),心肌致密化不全专家讲座,第5页,病理&病理生理,Microphotograph of a transverse section at the level of both ventricles of a heart that shows extensively developed trabeculae that fill the ventricular lumen.Note the form of the more compacted ventricular septum,心肌致密化不全专家讲座,第6页,心肌致密化不全专家讲座,第7页,心肌致密化不全专家讲座,第8页,临床特点,儿童多见，本病在成人发病率高于预期，有家族倾向,即使心肌致密化不全是先天性心肌病，但心脏病症状出现常在成年,临床表现无特异性：,1、心功效不全 患者可表现为胸闷、心悸、气急、咳嗽、水肿等。,心肌致密化不全专家讲座,第9页,2、心律失常 NVM 病人绝大部分作心电图检验均显示有异常，表现为 ST-T 改变、电轴偏移、传导阻滞、预激综合征、房性室性心律失常等，其中以室性心律失常和传导阻滞较多见，部分心律失常能够是致命性 3、,栓塞 包含脑血管意外、短暂脑缺血发作、肺栓塞、肠系膜梗死等,心肌致密化不全专家讲座,第10页,临床特点,心肌致密化不全专家讲座,第11页,诊疗,临床表现及心电图无特异性表现，心室造影、超高速CT、磁共振成像对心肌致密化不全诊疗有一定帮助,超声心动图当前依然是诊疗心肌致密化不全可靠方法,心肌致密化不全专家讲座,第12页,诊疗-,CT&MRI,心肌致密化不全专家讲座,第13页,诊疗-,超声诊疗标准,左室或右室腔内可探及无数突出增大肌小梁，错综排列，小梁间见大小不等深陷间隙,彩色多普勒可探及间隔内有血流与心腔相通,病变以近心尖部1/3节段最为显著，可涉及室壁中段，普通不累及基底段室壁，从室间隔中部到心尖部肌小梁逐步增多，占据大部分心尖区心腔，小梁外侧近心外膜有薄层靠近于正常心肌厚度致密心肌回声，而室间隔及左室后壁基底部心肌结构基本正常,受累心腔增大，运动显著减弱，收缩和舒张功效均减低，并可合并各种其它畸形,心肌致密化不全专家讲座,第14页,超声诊疗,A,Apical four chamber,B,parasternal short axis views demonstrating the prominent trabeculations and deep intertrabecular recesses localized to the regions of hypertrophy in the mid and apical anterior and anterolateral segments,C,Short axis view further depicting the trabeculations and intertrabecular recesses in the anterior and anterolateral segments,心肌致密化不全专家讲座,第15页,超声诊疗,心肌致密化不全专家讲座,第16页,超声诊疗-,三维超声,心肌致密化不全专家讲座,第17页,超声诊疗,Two-dimensional apical 4 chamber and parasternal short axis images at the level of the ventricles show dilatation of both ventricles,multiple trabeculae and intertrabecular recesses in inferior,lateral,anterior walls,middle and apical portions of the septum and apex of the left ventricle.A mild pericardial effusion can be observed,心肌致密化不全专家讲座,第18页,超声诊疗,Transthoracic two-dimensional study with color and continuous wave Doppler shows left ventricular noncompaction associated with patent ductus arteriosus(PDA).Trabeculae and deep recesses with penetration of color can be observed in the left ventricle.Continuous wave Doppler from a suprasternal approach at the level of the great vessels registers systolic-diastolic flow through the ductus arteriosus.,心肌致密化不全专家讲座,第19页,超声诊疗,Transthoracic 2-D echocardiogram in apical4 chamber and parasternal short axis at the level of both ventricles demonstrate dilatation,deep trabeculae and intertrabecular recesses in the inferior,lateral,anterior walls,middle and apical portions of the septum and apex of the left ventricle.The right ventricle also shows evidence of noncompaction,心肌致密化不全专家讲座,第20页,超声诊疗,Two-dimensional parasternal and color Doppler images at the level of both ventricles that show the noncompacted:compacted,wall ratio and how the color enters the intertrabecular recesses,Nilda Espinola-Zavaleta etal,Non-compacted cardiomyopathy:clinical-echocardiographic study,，,Cardiovascular Ultrasound,4:35,心肌致密化不全专家讲座,第21页,超声发觉-,IN 53 CASES,LVEDD,58 11.38(normal:50 mm),LVESD,45 13.35(normal:1000),Diastolic function,Impaired relaxation 14(26.4%)(E/A 1.0),Restrictive pattern 26(49.1%)(E/A 1.5),Normal 13(24.5%)(E/A=1.01.49),Thrombus,3(5.7%),Left ventricle 2,Left atrium 1,Pericardial effusion,3(5.7%),Valvular regurgitation,Mild mitral 15(28%),Moderate-Severe mitral 23(43%),Moderate aortic 1(1.9%),Mild tricuspid 17(32%),Moderate-Severe tricuspid 17(32%),Isolated ventricular noncompaction,39(74%),Ventricular noncompaction associated with other congenital anomalies,14(26%),Localization of ventricular noncompaction,Left ventricle 33(62%),Both ventricles 20(38%),Ratio of Noncompacted to Compacted Wall,3.4 0.87,心肌致密化不全专家讲座,第22页,超声发觉-,IN 53 CASES,Nilda Espinola-Zavaleta etal,Non-compacted cardiomyopathy:clinical-echocardiographic study,，,Cardiovascular Ultrasound,4:35,心肌致密化不全专家讲座,第23页,判别诊疗,（1）扩张性心肌病：可见心腔内突起肌小梁，但突起程度轻；,（2）缺血性心肌病：缺血区心肌运动异常，但没有异常突起肌小梁，另外 NVM因为没有心外膜冠脉病变，冠脉造影多正常；,（3）肥厚性心肌病：其心室肌增厚可类似 NVM，但没有深陷小梁间隐窝。,心肌致密化不全专家讲座,第24页,治 疗,心肌致密化不全治疗与扩张型心肌病治疗相同，主要是针对并发症加以治疗,利尿、扩血管、强心有利于改进心功效,双心室起搏,心脏移植,因为有频发室性心律失常和较高心源性猝死危险性，患者应每年行动态心电图检验,心肌致密化不全专家讲座,第25页,恶性心律失常患者可植入除颤器。,预防栓塞事件不论是否发觉心脏内血栓，NVM 患者宜行长久抗凝治疗,患者预后取决于病变分布范围，范围较小者能够终生无症状，范围较大患者能够较早就发生进行性心力衰竭，甚至死亡,心肌致密化不全专家讲座,第26页,人类窦状隙仅出现在胚胎头两个月。,在个体发生早期,心肌小梁呈网状,小梁无血管及毛细血管,由与心房或心室相通小梁间隙或窦状隙供血。,在正常伴随心肌发育,疏松小梁网逐步致密化,小梁间隙变为毛细血管。小梁类型逐步转变为致密化心肌,伴同它供血重构,心肌致密化不全专家讲座,第27页,