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心室致密化不全诊疗进展,杨萍内科,概述,心肌致密化不全(noncompactionventricularmyocardium,NVM)又称海绵状心肌(spongymyocardium)，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，本病是由于胚胎时期疏松的心肌组织致密化过程障碍导致小梁化的心肌持续存在所致的一种先天畸形解剖学上表现为无数突出的肌小梁和深陷的小梁间隐窝。临床表现缺乏特异性，常见的表现为心功能不全、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的诊断方法。,病理Originalmagnification,40)CLow-powerviewofhistologicsectionshowingmultipletrabeculationsandmyocardialprojections,withextensivefibrosis(Klatskintrichromestain;originalmagnification,10)DLow-powerviewofhistologicsectionshowingathickenedendocardiumoverlyingthemyocardialrecesses,withareasofpatchyfibrosis(Klatskinstain;originalmagnification,40),病理&病理生理,Microphotographofatransversesectionatthelevelofbothventriclesofaheartthatshowsextensivelydevelopedtrabeculaethatfilltheventricularlumen.Notetheformofthemorecompactedventricularseptum,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表现无特异性：1、心功能不全患者可表现为胸闷、心悸、气急、咳嗽、水肿等。,2、心律失常NVM病人绝大部分作心电图检查均显示有异常，表现为ST-T的改变、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，部分心律失常可以是致命性的3、栓塞包括脑血管意外、短暂脑缺血发作、肺栓塞、肠系膜梗死等,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,ApicalfourchamberB,parasternalshortaxisviewsdemonstratingtheprominenttrabeculationsanddeepintertrabecularrecesseslocalizedtotheregionsofhypertrophyinthemidandapicalanteriorandanterolateralsegmentsC,Shortaxisviewfurtherdepictingthetrabeculationsandintertrabecularrecessesintheanteriorandanterolateralsegments,超声诊断,超声诊断-三维超声,超声诊断,Two-dimensionalapical4chamberandparasternalshortaxisimagesattheleveloftheventriclesshowdilatationofbothventricles,multipletrabeculaeandintertrabecularrecessesininferior,lateral,anteriorwalls,middleandapicalportionsoftheseptumandapexoftheleftventricle.Amildpericardialeffusioncanbeobserved,超声诊断,Transthoracictwo-dimensionalstudywithcolorandcontinuouswaveDopplershowsleftventricularnoncompactionassociatedwithpatentductusarteriosus(PDA).Trabeculaeanddeeprecesseswithpenetrationofcolorcanbeobservedintheleftventricle.ContinuouswaveDopplerfromasuprasternalapproachatthelevelofthegreatvesselsregisterssystolic-diastolicflowthroughtheductusarteriosus.,超声诊断,Transthoracic2-Dechocardiograminapical4chamberandparasternalshortaxisatthelevelofbothventriclesdemonstratedilatation,deeptrabeculaeandintertrabecularrecessesintheinferior,lateral,anteriorwalls,middleandapicalportionsoftheseptumandapexoftheleftventricle.Therightventriclealsoshowsevidenceofnoncompaction,超声诊断,Two-dimensionalparasternalandcolorDopplerimagesatthelevelofbothventriclesthatshowthenoncompacted:compactedwallratioandhowthecolorenterstheintertrabecularrecesses,NildaEspinola-ZavaletaetalNon-compactedcardiomyopathy:clinical-echocardiographicstudy，CardiovascularUltrasound2006,4:35,超声发现-IN53CASES,LVEDD5811.38(normal:1000)DiastolicfunctionImpairedrelaxation14(26.4%)(E/A1.0)Restrictivepattern26(49.1%)(E/A1.5)Normal13(24.5%)(E/A=1.01.49)Thrombus3(5.7%)Leftventricle2Leftatrium1Pericardialeffusion3(5.7%)ValvularregurgitationMildmitral15(28%)Moderate-Severemitral23(43%)Moderateaortic1(1.9%)Mildtricuspid17(32%)Moderate-Severetricuspid17(32%)Isolatedventricularnoncompaction39(74%)Ventricularnoncompactionassociatedwithothercongenitalanomalies14(26%)LocalizationofventricularnoncompactionLeftventricle33(62%)Bothventricles20(38%)RatioofNoncompactedtoCompactedWall3.40.87,超声发现-IN53CASES,NildaEspinola-ZavaletaetalNon-compactedcardiomyopathy:clinical-echocardiographicstudy，CardiovascularUltrasound2006,4:35,鉴别诊断,（1）扩张性心肌病：可见心腔内突起的肌小梁，但突起程度轻；（2）缺血性心肌病：缺血区心肌运动异常，但没有异常突起的肌小梁，另外NVM由于没有心外膜冠脉的病变，冠脉造影多正常；（3）肥厚性心肌病：其心室肌的增厚可类似NVM，但没有深陷的小梁间隐窝。,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利尿、扩血管、强心有助于改善心功能双心室起搏心脏移植由于有频发室性心律失常和较高的心源性猝死的危险性，患者应每年行动态心电图检查,恶性心律失常的患者可植入除颤器。预防栓塞事件不管是否发现心脏内血栓，NVM患者宜行长期的抗凝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,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的小梁间隙或窦状隙供血。在正常随着心肌发育,疏松的小梁网逐渐致密化,小梁间隙变为毛细血管。小梁类型逐渐转变为致密化心肌,伴同它的供血的重构,
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