心肌致密化不全課件

1、心室致密化不全診療進(jìn)展,楊萍 內(nèi)科,概述,心肌致密化不全(noncompaction ventricular myocardium,NVM)又稱海綿狀心肌(spongy myocardium)，臨床上較為少見。近年來國內(nèi)外對(duì)該病逐漸認(rèn)識(shí)，2006年心肌病分類為遺傳性心肌病 心肌致密化不全是一種罕見的先天性心肌病，本病是由于胚胎時(shí)期疏松的心肌組織致密化過程障礙導(dǎo)致小梁化的心肌持續(xù)存在所致的一種先天畸形 解剖學(xué)上表現(xiàn)為無數(shù)突出的肌小梁和深陷的小梁間隱窩。 臨床表現(xiàn)缺乏特異性，常見的表現(xiàn)為心功能不全、心律失常和栓塞。 多見于左心室，也可見于右心室及雙心室，可單發(fā)或呈家族聚集性。孤立性心肌致密化不全或

2、與其它先天性心臟畸形并存 超聲心動(dòng)圖是首選的診斷方法。,病理 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 endo

3、cardium overlying the myocardial recesses, with areas of patchy fibrosis (Klatskin stain; original magnification, 40),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

4、of the more compacted ventricular septum,臨床特點(diǎn),兒童多見，本病在成人發(fā)病率高于預(yù)期，有家族傾向 雖然心肌致密化不全是先天性心肌病，但心臟病癥狀的出現(xiàn)常在成年 臨床表現(xiàn)無特異性： 1、心功能不全 患者可表現(xiàn)為胸悶、心悸、氣急、咳嗽、水腫等。,2、心律失常 NVM 病人絕大部分作心電圖檢查均顯示有異常，表現(xiàn)為 ST- T 的改變、電軸偏移、傳導(dǎo)阻滯、預(yù)激綜合征、房性室性心律失常等，其中以室性心律失常和傳導(dǎo)阻滯較多見，部分心律失常可以是致命性的 3、 栓塞 包括腦血管意外、短暫腦缺血發(fā)作、肺栓塞、腸系膜梗死等,臨床特點(diǎn),診斷,臨床表現(xiàn)及心電圖無特異性表現(xiàn)，

5、心室造影、超高速CT、磁共振成像對(duì)心肌致密化不全的診斷有一定幫助 超聲心動(dòng)圖目前仍然是診斷心肌致密化不全的可靠方法,診斷-CT&MRI,診斷-超聲診斷標(biāo)準(zhǔn),左室或右室腔內(nèi)可探及無數(shù)突出增大的肌小梁，錯(cuò)綜排列，小梁間見大小不等的深陷間隙 彩色多普勒可探及間隔內(nèi)有血流與心腔相通 病變以近心尖部1/3節(jié)段最為明顯，可波及室壁中段，一般不累及基底段室壁，從室間隔中部到心尖部肌小梁逐漸增多，占據(jù)大部分心尖區(qū)心腔，小梁外側(cè)近心外膜有薄層接近于正常心肌厚度的致密心肌回聲，而室間隔及左室后壁基底部心肌結(jié)構(gòu)基本正常 受累心腔增大，運(yùn)動(dòng)明顯減弱，收縮和舒張功能均減低，并可合并多種其他畸形,超聲診斷,A, Apic

6、al 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 r

7、ecesses in the anterior and anterolateral segments,超聲診斷,超聲診斷-三維超聲,超聲診斷,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 a

8、nd apical portions of the septum and apex of the left ventricle. A mild pericardial effusion can be observed,超聲診斷,Transthoracic two-dimensional study with color and continuous wave Doppler shows left ventricular noncompaction associated with patent ductus arteriosus (PDA). Trabeculae and deep recess

9、es 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.,超聲診斷,Transthoracic 2-D echocardiogram in apical4 chamber and parasternal short a

10、xis 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,超聲診斷,Two-dimensional pa

11、rasternal 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 2006, 4:35,超聲

12、發(fā)現(xiàn)-IN 53 CASES,LVEDD 58 11.38 (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

13、%) 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

14、 (62%) Both ventricles 20 (38%) Ratio of Noncompacted to Compacted Wall 3.4 0.87,超聲發(fā)現(xiàn)-IN 53 CASES,Nilda Espinola-Zavaleta etal Non-compacted cardiomyopathy: clinical-echocardiographic study，Cardiovascular Ultrasound 2006, 4:35,鑒別診斷,（1）擴(kuò)張性心肌?。嚎梢娦那粌?nèi)突起的肌小梁，但突起程度輕； （2）缺血性心肌病：缺血區(qū)心肌運(yùn)動(dòng)異常，但沒有異常突起的肌小梁，另外 NVM由于沒有心外膜冠脈的病變，冠脈造影多正常； （3）肥厚性心肌?。浩湫氖壹〉脑龊窨深愃?NVM，但沒有深陷的小梁間隱窩。,治 療,心肌致密化不全的治療與擴(kuò)張型心肌病的治療相似，主要是針對(duì)并發(fā)癥加以治療利尿、擴(kuò)血管、強(qiáng)心有助于改善心功能 雙心室起搏 心臟移植 由于有頻發(fā)室性心律失常和較高的心源性猝死的危險(xiǎn)性，患者應(yīng)每年行動(dòng)態(tài)心電圖檢查,惡