左心室肌致密化不全8例临床分析

目的　探讨左心室肌致密化不全的临床及超声心动图特点。方法　分析左心室肌致密化不全临床表现、心电图、胸片、超声心动图特点。结果　左心功能不全 3例 ,肾栓塞 1例 ;室性期前收缩 2例 ;超声心动图均发现左心室腔内二尖瓣水平、腱索水平或乳头肌水平以下的心肌被网格状或蜂窝状的海绵样心肌组织所充填 ,可合并先天性心脏畸形。结论　左心室肌致密化不全临床上可表现为左心功能不全 ,室性心律失常及心内膜血栓伴系统栓塞 ,超声心动图是诊断本病的首选方法。     

孤立性心室肌致密化不全四例报告

目的 通过对四例孤立性心室肌致密化不全（INVM）临床资料的分析以提高对此病的认识。方法 对4例患儿的临床、心电图、胸部X线、超声心动图表现及1例心室造影结果进行分析和总结。结果 以阵发性胸闷、气短及头晕为主要症状；4例均出现心脏扩大，心音低钝，2例心律不齐，2例心尖部Ⅱ级收缩期杂音；心电图均表现为心肌缺血，2例室性早搏，1例阵发性室性心动过速合并完全性右束支传导阻滞；胸部X线检查提示心脏扩大；二维超声心动图在心室内探及大量突出的肌小梁及深陷的小梁隐窝；彩色多普勒提示小梁间有血流与心腔相通，2例出现左心收缩功能下降，1例左心舒张功能下降，1例心室造影提示心腔内肌小梁增多，排列紊乱，放射状充盈缺损。结论 INVM是一种病因不明的心室肌结构异常疾病。它对心脏带来三种危险：（1）心功能不全；（2）室性心律失常；（3）心内膜血栓伴各系统栓塞。超声心动图是诊断INVM的一线工具。     

Peabody运动发育评估与干预学习班将在京举办

答：由于心肌致密化不全（noncompaction of ventricular myocardium，NVM）临床表现无特异性，冠状动脉造影显示正常，X线和心电图检查很难将其与扩张型心肌病（DCM）鉴别，而超声心动图则可显示本病心室肌的异常结构特征与功能。超声心动图诊断标准包括：（1）心室壁异常增厚并呈现两层结构：薄且致密的心外膜层和厚而非致密的心内膜层，后者由粗大突起的肌小梁和小梁间的隐窝构成，且隐窝与室腔交通而具有连续性。     

心室肌致密化不全的超声心动图诊断

目的 采用超声心动图对心室肌致密化不全(NCVM)进行诊断,阐明其超声心动图特征。方法 将2006 年5 月至2015 年5 月接受超声心动图检查、且诊断为NCVM 的患儿53 例纳入研究,其中男28 例,女25 例,首次诊断年龄为15 d 至18 岁。采用经胸二维超声心动图和彩色多普勒进行定性诊断,于胸骨旁心室短轴切面观测量舒张末期疏松部/致密部(N/C)比值作为定量诊断标准。结果 在53 例患儿中,均观测到过度突出的肌小梁和深陷的小梁间隐窝,且心腔内的血流与肌小梁间的隐窝沟通。NCVM 累及的部位主要是心尖部(100%)和左室侧壁中间段(98%),其次为左室后壁中间段(49%)和左室下壁中间段(42%)。53 例NCVM 患儿中,N/C 比值为4.3±1.9(2.1~10.0)≥ 2。心功能不全占83%(44/53),这些患儿的左室射血分数为43%±9%。结论 超声心动图可以对NCVM 进行定性和定量诊断,并对其心功能进行评价。NCVM 以累及心尖部和左室侧壁中间段较常见,且常伴有左室射血分数减低。     

儿童心肌致密化不全

心肌致密化不全(NVM)是一种少见的先天性遗传性心肌病,以心腔内异常粗大的肌小梁和小梁间深陷的隐窝为特征。其临床表现多样且无特异性,以心功能不全、心律失常、血栓形成为主要特征。超声心动图和心脏磁共振成像在NVM诊断中具有重要意义。治疗上主要是对症治疗,内科治疗无效者可行心脏移植。本病预后差异大,病死率高,但无症状者预后相对较好。     

美国新生儿复苏教程高级师资培训班通知

答：近些年超声心动图检查发现，部分因心力衰竭就诊的患者心室腔内存在粗大突起的肌小梁和深陷隐窝，因而将其诊断为心肌致密化不全（noncompaction of ventricular myocardium，NVM）性心肌病。由于这些患者存在心脏扩大征象，临床症状与扩张型心肌病（DCM）相似，如何早期识别NVM，应当引起临床医生的关注。     

左心室心肌致密化不全超声心动图诊断进展

心肌致密化不全是一种发育不全的心肌表型, 病理特征是心室内异常增多的突出的肌小梁和深陷的小梁间隐窝。目前主要检查方法包括超声心动图、心脏磁共振成像以及心脏CT, 其中超声心动图是首选的影像学诊断技术, 但其各个标准并不统一, 尚无金标准。现就左心室心肌致密化不全超声心动图诊断技术的进展进行回顾和综述, 以期对该病的诊断有更清晰的认识和了解。     

儿童心肌致密化不全的临床表现及预后

心肌致密化不全是一种与基因相关的遗传性心肌病,以心室内异常粗大的肌小梁和交错的深隐窝为特征.心肌致密化不全病因及发病机制不清.心肌致密化不全临床少见,预后较差.该文就儿童心肌致密化不全的临床表现及预后进行阐述,加深对儿童心肌致密化不全的认识,提高临床诊治水平,提高患者生存质量,延长患儿生存期.     

实时三维超声心动图对先天性心室肌致密化不全的诊断价值

目的 应用实时三维超声心动图（RT3DE）诊断儿童先天性心室肌致密化不全（NCVM），初步评价其应用价值。方法 2006年7至12月应用二维超声心动图诊断为NCVM的患儿4例，其中女性3例，男性1例，年龄2~11个月。采用RT3DE观察心脏肌小梁和隐窝的形态，计算左室射血分数，根据时间 容量曲线参数对左室同步性进行评价。结果 ①RT3DE对NCVM的形态观察：1例累及整个左室，3例累及左室中段和心尖部。②RT3DE对NCVM的量化诊断：收缩末期最大肌小梁顶部至心外膜表面距离与隐窝底部至心外膜表面距离的比值（X′s/Y′s）为3.3~5.0；舒张末期最大肌小梁顶部至心外膜表面距离与隐窝底部至心外膜表面距离的比值（X′d/Y′d）为2.6~4.4。③RT3DE对NCVM的心功能评价：左室射血分数为21.2%～56.4%。④RT3DE对NCVM的左室同步性评价：所有16个心肌节段到达每一个节段收缩期最小容量时间的标准差在心动周期中所占的比例为2.65%～19.30%，最大差值在心动周期中所占比例为8.75%～78.20%。结论 RT3DE不仅能够观察NCVM累及的范围，而且可以定量评价其严重程度，测定心功能以及评价同步化运动。     

儿童心肌致密化不全6例临床表现及基因变异分析

该文总结分析儿童心肌致密化不全（NVM）的临床和基因变异特点。6例NVM患儿起病年龄3个月至12岁,男4例,女2例,心律失常5例,心功能不全3例,精神差1例,胸闷、叹气1例。4例患儿检测到NVM相关基因变异,其中MYH7基因变异2例,PRDM16基因变异1例,ACTN2基因+TNNT2基因变异1例。心功能改善4例;2例患儿心功能改善不明显,其发病年龄小,超声心动图示收缩功能降低更明显,心肌酶、氨基末端脑钠肽前体升高更明显。NVM是引起儿童慢性心力衰竭的重要原因之一,对于首发有胸闷、叹气、心律失常、心影增大、心肌酶增高明显的患儿,进行超声心动图、心脏磁共振检查,可确诊NVM,NVM可有多种基因变异。     

心肌致密化不全25例

目的 总结小儿心肌致密化不全(NVM)的临床特征,以提高对该病的认识及诊治水平.方法 回顾性分析2005年1月- 2010年12月在本院住院的25例NVM患儿的临床表现、心脏彩超、ECG等辅助检查结果及治疗、随访情况.结果 25例患儿中有心功能不全表现者19例(76％);有心律失常者7例(28％),其中室性快速心律失常4例(16％);均未发现心内膜血栓形成.25例患儿彩色多普勒超声心动图均见典型的NVM改变,左心室受累23例(92％),右心室受累2例(8％);心脏扩大24例(96％),左心室射血分数＜50％ 20例(80％).住院前误诊为扩张型心肌病(DCM)3例,心内膜弹力纤维增生症(EFE)1例.随访0.4 ～5.6a,6例患儿死于顽固性心力衰竭,1例患儿猝死,4例失访.结论 小儿NVM的临床表现多以心功能不全及室性快速心律失常为主,极少形成心内膜血栓.小儿NVM易误诊为DCM、EFE等,彩色多普勒超声心动图在该病的诊断及鉴别诊断中具有重要价值.小儿NVM尚缺乏有效的治疗方法,预后差.     

心肌致密化不全合并弥漫性冠状动脉右室瘘1例

目的：探讨心肌致密化不全合并弥漫性冠状动脉瘘的临床特点及诊断方法。方法：报告1例心肌致密化不全合并弥漫性冠状动脉右室瘘患儿,回顾其临床症状、辅助检查结果和治疗方案。在国外数据库（Pubmed、OVID和Elsevier）和国内数据库（万方和维普）中检索心肌致密化不全合并冠状动脉瘘和心肌病合并冠状动脉瘘病例的文献,检索时间为建库至2017年3月30日。总结此类病例的临床表现、诊断和治疗。结果：本文患儿为6月龄女婴,因“胃纳差伴盗汗2周”于2013年10月12日在上海交通大学医学院附属上海儿童医学中心心内科就诊。二维超声心动图显示,左心室增大伴左室致密化不全、左右冠状动脉扩张、右心室内心肌窦状间隙持续状态、二尖瓣中度返流、心室收缩功能低下等。心导管造影及冠状动脉造影检查显示,右冠状动脉及左前降支弥漫性微小右心室瘘、二尖瓣返流、左心室增大。予地高辛、速尿、安体舒通后患儿症状稍好转。国外数据库中检索到2例心肌致密化不全合并冠状动脉左室瘘的成年病例,以进行性呼吸困难和心前区疼痛为主要临床表现,心肌缺血表现更明显,诊断主要依靠二维超声心动图及心导管造影和冠状动脉造影检查,缺乏有效治疗手段;有部分心肌病合并心肌致密化不全患者接受选择性冠状动脉瘘介入封堵术的尝试。结论：心肌致密化不全合并冠状动脉瘘是一类罕见的先天性心脏发育异常疾病,婴儿临床症状主要为纳差、盗汗等心功能不全的表现,确诊主要依靠二维超声心动图以及心导管造影、冠脉造影检查,目前尚缺乏有效治疗手段,以强心、利尿、扩血管改善心功能治疗为主。     

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??Abstract?? Objective??To explore the clinical features of noncompaction of the ventricular myocardium??NVM?? in 123 children. Methods??Clinical manifestation?? electrocardiogram?? chest X-ray and echocardiogram of 123 patients as well as 31 cases of cardiac catheterization and 24 of cardiac CT of NVM were studied. Results??Among 123 patients?? 50 suffered from chest discomfort?? short breath and much sweat?? 110 were cardiac dilatation?? and a decrease of the intensity of heart sounds was found in 30 children?? 65 coexisted with cardiac abnormalities??104 patients had ST-T change on electrocardiogram. Cardiac arrhythmia was found in 29 cases?? 98 cases of cardiothoracic ratio > 0.5 were noticed in chest X-ray. Echocardiograph examination showed that numerous prominent ventricular trabeculations and deep intertrabecular recesses were presented in ventricle. Characteristic changes of image in cardiac catheterization and cardiac CT were found as well. Conclusion??Clinical features of NVM in children are a depressed cardiac function and arrhythmia?? an infrequency of systemic embolization with a higher rate of coexistence of cardiac abnormalities. Echocardiography is a reliable promising diagnostic tool for NVM.     

Myocardial noncompaction: report of three cases

Myocardial noncompaction is a rare type of cardiomyopathy which can be an isolated entity or in association with other congenital heart diseases. We present three children with myocardial noncompaction: one male with isolated left ventricular noncompaction, another with right ventricular noncompaction and dysplastic tricuspid valve, and the last with left ventricular noncompaction, ventricular septal defect and coarctation of aorta, to stress especially the different clinical forms of the disorder and the importance of early diagnosis, as it may result in a fatal outcome.     

Transient Postnatal Heart Failure Caused by Noncompaction of the Right Ventricular Myocardium

Isolated noncompaction of the ventricular myocardium is a cardiomyopathy influencing almost exclusively the left ventricle in children and adults. We report a case of a neonate presenting with right ventricular failure caused by a noncompaction of the right ventricle. However, later course of the disease demonstrated biventricular involvement. Right ventricular noncompaction can be a cause of early postnatal right ventricular failure.     

Isolated Right Ventricular Noncompaction in a Newborn

Noncompaction of the ventricular myocardium is a rare cardiomyopathy characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses. The prevalence of left ventricular noncompaction is 0.01% in adults and 0.14% in pediatric patients. Although the usual site of involvement is the left ventricle, the right ventricle and septum can be affected as well. Previously, right ventricular noncompaction has been described only in a few cases of newborns with congenital heart defects and in adult patients. This report presents a newborn with isolated right ventricular noncompaction. To the authors’ knowledge, this is the first newborn patient with isolated right ventricular noncompaction but no congenital heart defect involving only the right ventricle.     

Extent of myocardial noncompaction: comparison between MRI and echocardiographic evaluation

Background: Noncompaction of the left ventricular myocardium is an important cause of cardiomyopathy. There is no clear consensus about its diagnostic criteria or the diagnostic test of choice. MRI is increasingly used in the pediatric cardiac field because of its superior and objective image quality. Objective: To compare the echocardiographic and MRI findings in four patients with recently diagnosed ventricular noncompaction. Materials and methods: We compared the extent of myocardial involvement shown at MRI and echocardiography in four individuals, two patients with echocardiographic diagnosis of left ventricular noncompaction, and two family members of one of the patients. Results: In all patients, MRI showed wider area of involvement than echocardiography. A definite diagnosis was entertained in only two patients by echocardiography but in all by MRI. Cine imaging was diagnostic of the disease in all patients. Black-blood pool imaging with double-inversion recovery sequence also helped to visualize the abnormal areas by showing slow flow artifacts in the four- and two-chamber images. Conclusion: MRI provided better delineation of the extent of the abnormal trabeculation in patients with noncompaction of the left ventricular myocardium. It was particularly useful when the myocardial involvement was subtle, as in the asymptomatic family members.