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1、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渐认识，2006年心肌病分类为遗传性心肌病 心肌致密化不全是一种罕见的先天性心肌病，其病因及发病率不明，可能是由于在胚胎早期网状肌小梁致密化过程失败导致小梁化的心肌持续存在所致。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。,病理&病理生理,心脏扩大，心肌重量增加，冠状动脉通畅。主要病变为受累的心室腔内多发异常粗大的肌小梁和交错深陷的隐窝，可不同程度地累及心室壁的内2/3 非致密心肌肌束明
2、显肿大，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,病理&病理生理,进行性心力衰竭系收缩功能及舒张功能受损引起 慢性缺血可能是收缩功能受损的主要原因，多个异常隆起的肌小梁对血液的需求和心脏血供不匹配造成了慢性缺血 致密化不全心肌的范围和慢性缺血程度以及伴发的疾病决定了病程的进展 舒张功能减低是由于粗大的肌小梁引起的室壁主动弛张障碍和室壁僵硬度增加，顺应性下降,病理&病理生理,心肌致密化不全大部分孤立存在，有时也合并有其他的先天性心脏病，如房间隔缺损、室间隔缺损，其他复杂的紫绀性先天性心脏病，或冠状动脉畸形、心脏瓣膜
3、畸形等 心肌致密化不全可以合并各种心律失常，包括预激综合征、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 在某些儿童患者中，可以观察到一些面部畸形，如前额突出、斜视、小面脸、低耳垂等,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 (he
4、matoxylin 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 endocard
5、ium 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 of t
6、he more compacted ventricular septum,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 临床表现无特异性，心肌致密化不全患者出现症状的年龄和临床表现程度个体差异很大，从无症状到严重心功能不全，或发生猝死 虽然心肌致密化不全是先天性心肌病，但心脏病症状的出现常在成年,临床特点,Demographic characteristics of 53 patients Male gender 25 (47.2%) Age at Diagnosis, All 43.2 14.76 (range 1674) Men 40.9 13.65( range 1660)
7、Women 45.2 15.65(range 2174) Age, years Number Percent 1020 2 (3.8%) 2130 13 (24.5%) 3140 7 (13.2%) 4150 11 (20.8%) 50 20 (37.7%) Duration 7 5 months( range 124) Prevalence in the INCICh 4/10,000/5 years,临床特点,Clinical and electrocardiographic characteristics IN 53 CASES OF NVM Precordial pain 11 （21
8、%） Syncope 5 （9.4%） NYHA Functional class I/II 40 （75%） NYHA Functional class III/IV 13 （25%） Familiar occurrence 16 （30.2%） Cardiac Rhythm Sinus 48 （90.5%） Atrial fibrillation 3 （5.7%） Pacemaker 2 （3.8%） Bundle branch block 25 （47.2%） Left 18（72%） Right 7（ 28%） With premature ventricular contractio
9、ns 21 （40%） With supraventricular escape beats 14 （26.4%） 24 hour Holter Runs of premature ventricular contractions 17 （32%） Sustained ventricular tachycardia 4 （7.5%）,临床特点,Congenital heart disease associated with NVM(n=14) Ebsteins Anomaly + ASD 2 Uhls Anomaly + ASD 1 Atrial septal aneurysm + PFO 2
10、 Double outlet right ventricle + Pulmonary stenosis 1 Asymmetric septal hypertrophic cardiomyopathy 1 Unicuspid mitral valve 2 Atrial septal defect + MVP 1 Double mitral orifice + Moderate mitral regurgitation 1 Bicuspid aortic valve + Moderate aortic regurgitation 1 Persistent ductus arteriosus 2,临
11、床特点-临床表现,心力衰竭，主要是左心功能不全，也可合并右心功能不全 快速性室性心律失常多见，包括易发生心脏猝死的室性心动过速，左束支传导阻滞也比较多见 心脏栓塞事件，尽管病变心腔小梁间隙内容易形成血栓，但所引起的体循环栓塞事件并不多见,临床特点,诊断,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的诊断有一定帮助 超声心动图目前仍然是诊断心肌致密化不全的可靠方法,诊断-CT&MRI,诊断-超声诊断标准,左室或右室腔内可探及无数突出增大的肌小梁，错综排列，小梁间见大小不等的深陷间隙 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节段最为明显，可波及
12、室壁中段，一般不累及基底段室壁，从室间隔中部到心尖部肌小梁逐渐增多，占据大部分心尖区心腔，小梁外侧近心外膜有薄层接近于正常心肌厚度的致密心肌回声，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，运动明显减弱，收缩和舒张功能均减低，并可合并多种其他畸形,超声诊断,A, Apical four chamber B, parasternal short axis views demonstrating the prominent trabeculations and deep intertrabecular recesses localized to the regions of hyper
13、trophy 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,超声诊断,超声诊断-三维超声,超声诊断,Two-dimensional apical 4 chamber and parasternal short axis images at the level of the
14、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,超声诊断,Transthoracic two-dimensional study with col
15、or 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 reg
16、isters systolic-diastolic flow through the ductus arteriosus.,超声诊断,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
17、apical portions of the septum and apex of the left ventricle. The right ventricle also shows evidence of noncompaction,超声诊断,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 intertrabecula
18、r recesses,Nilda Espinola-Zavaleta etal Non-compacted cardiomyopathy: clinical-echocardiographic study，Cardiovascular Ultrasound 2006, 4:35,超声发现-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 (2
19、4.5%) (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 (
20、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,超声发现-IN 53 CASES,Nilda Espinola-Zavaleta etal Non-compacted cardiomyopathy: c
21、linical-echocardiographic study，Cardiovascular Ultrasound 2006, 4:35,治 疗,心肌致密化不全的治疗与扩张型心肌病的治疗相似，主要是针对并发症加以治疗 患者的预后取决于病变分布范围，范围较小者可以终生无症状，范围较大的患者可以较早就发生进行性心力衰竭，甚至死亡,随 访,patients with a noncompacted:compacted ratio greater than 3 and ventricular noncompaction in three or more segments are in functiona
22、l class III/IV rather than functional class I/II For patients in functional class III/IV with ventricular arrhythmia and noncompaction of three or more segments, probability of survival to 9 months was 75%. Probability of survival to 15 months in these patients was 48%,Nilda Espinola-Zavaleta etal N
23、on-compacted cardiomyopathy: clinical-echocardiographic study，Cardiovascular Ultrasound 2006, 4:35,随 访 7（1-24）M in 53 cases,Heart failure requiring hospitalization 7 (13%) Deaths 3 (5.7%) Heart failure 3 Implantation of intracardiac defibrillator 3 (5.7%) Thromboembolic events 3 (5.7%) Cerebrovascul
24、ar accident 2 Transient ischemic attack 1,Nilda Espinola-Zavaleta etal Non-compacted cardiomyopathy: clinical-echocardiographic study，Cardiovascular Ultrasound 2006, 4:35,随 访,Nilda Espinola-Zavaleta etal Non-compacted cardiomyopathy: clinical-echocardiographic study，Cardiovascular Ultrasound 2006, 4:35,