12例成人心肌致密化不全超声心动图及临床分析

目的探讨成人心肌致密化不全(NVM)患者的超声心动图(UCG)及临床特点。方法回顾性分析12例成人NVM患者的超声心动图及临床表现。UCG检查方法以四腔观、左心室长轴二腔观和左心室短轴观为主进行多切面扫查,重点观察近心尖1/3的左心室心肌与心内膜。结果12例患者UCG检查均可见受累心腔内不同程度异常隆突、粗大的肌小梁和其间深陷的隐窝影像,其中2例合并二尖瓣脱垂伴重度反流,1例主动脉瓣重度关闭不全。8例不同程序度血性心力衰竭,1例因心绞痛行冠状动脉造影见三支病变,1例脑梗塞,3例无异常。结论NVM临床病程迁延,临床表现各异,易造成误诊和漏诊。同时NVM具有特征性UCG改变,可区别于原发性扩张型心肌病。UCG是重要的诊断和筛查手段。     

心肌致密化不全与扩张型心肌病合并过度小梁化的对比分析

目的:探讨心肌致密化不全(NVM)与扩张型心肌病(DCM)合并过度小梁化的临床和超声心动图特点,明确对两者鉴别诊断价值。方法:对比分析31例NVM组及50例DCM合并过度小梁化组的性别、年龄、家族史、症状、心电图、脑钠肽(BNP)及超声心动图资料,着重观察两者超声心动图心腔大小、心肌壁、心内膜、彩色多普勒、血液动力学的特点,依据17节段分析法分析小梁化节段数目及程度。结果:(1)DCM合并过度小梁化组心功能分级更差,BNP明显较NVM组高(P0.05),心脏扩大程度也更明显,差异有统计学意义;(2)NVM组患者小梁化的节段数最多,节段数(9.82±2.02)个,心尖段(第17节段)均受累,非致密化心肌厚度(NC)和致密化心肌厚度(C)比值(NC/C)大(2.84±0.61),NC/C值2的节段数为(4.12±2.68)个;DCM合并过度小梁化组患者小梁化的节段数少,节段数(5.56±1.56)个,心尖段很少受累,NC/C值小(1.91±0.42),最多有1个节段NC/C值2。差别均具有统计学意义(P0.05)。结论:超声心动图是鉴别NVM与DCM的简便、实用、无创性检查手段。左心室心尖段明显呈致密化不全改变及至少2个游离壁节段收缩期的NC/C值2可诊断NVM,并可与DCM合并过度小梁化相鉴别。     

成人左室心肌致密化不全的超声心动图诊断

目的探讨成人左室心肌致密化不全的超声心动图特点。方法对45例成人左室心肌致密化不全患者进行超声心动图检查,多切面重点观察左室心肌与心内膜。结果超声心动图检查表明,左室腔内可见异常粗大呈蜂窝状的肌小梁和交错深陷的隐窝,其中34例有心力衰竭症状,3例进行心脏移植,11例患者受累心肌局限在左室心尖部,无任何症状。彩色多普勒可探及隐窝内有血流与心腔相通。结论超声心动图可快速、无创地诊断心肌致密化不全。     

实时三维超声心动图在心肌致密化不全诊断中的应用价值

目的探讨实时三维超声心动图(RT-3DE)对心肌致密化不全患者的应用价值。方法对家系中3例患者及其亲属先采用二维超声心动图检查,通过对左心室长轴、左心室短轴、心尖四腔等多切面扫查,观察心肌、心内膜的组织结构和血流特征以及测量心室功能,然后启动三维模式,获得清晰的三维图像。结果3例患者均取得了逼真的三维超声心动图,发现受累心腔内有异常隆突、粗大的肌小梁,互相交错,呈网状,其间存在深陷的隐窝,内见低速血流与心腔内血液相通。结论三维超声对细致结构的显示明显优于二维超声,且清晰敏感,在心肌致密化的诊断和鉴别诊断中有重要应用价值。     

成人左室心肌致密化不全的超声诊断分析

目的 探讨超声心动图对成人心肌致密化不全(NVM)的诊断价值,以提高临床及超声医师对该病的认识.方法 回顾性分析7例成人左室心肌致密化不全患者的心脏形态结构的超声心动图特点及血流改变特征.结果 7例患者超声心动图均可见左室腔内不同程度突起的肌小梁,形成网状结构,其间可见深陷的隐窝,病变均累积左室中下段,以心尖部为主,室间隔基底段基本正常,病变处心内膜节段性缺失.5例左房、左室增大,7例均有左室收缩及舒张功能减低.结论 NVM超声心动图具有特征性改变,是目前诊断该病的首选检查方法.     

心肌致密化不全患者的彩色多普勒超声心动图特征

目的总结心肌致密化不全(NVM)患者的彩色多普勒超声心动图特征。方法选择2007年1月~2014年7月自海军总医院超声医学科应用彩色多普勒超声心动图诊断的NVM患者25例,其中男性18例,女性7例,年龄范围20~78岁,平均年龄(39.56±10.15)岁。15例有明显临床症状为有症状组,10例平时无症状为无症状组。彩色多普勒超声心动图检查确诊的NVM患者25例,分析其图像特征。结果NVM患者心脏构型特点均为心室腔内可见大量突起的肌小梁及深陷的小梁隐窝,病变以心尖部最为显著。有症状组的患者舒张期心肌非致密层与致密层比值(N/C),左室舒张末期内径均高于无症状组患者,左室射血分数较之明显减低,差异均有统计学意义(P均0.05),而收缩期N/C两组相比差异无统计学意义(P0.05)。结论 NVM具有特征性声像图表现,彩色多普勒超声心动图是诊断NVM可靠的检查方法。     

关注心肌致密化不全的临床诊断

近些年超声心动图检查发现,部分因心力衰竭而就诊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(noncom-paction 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     

孤立性左心室肌致密化不全10例分析

目的　评价超声心动图技术在诊断孤立性左室肌致密化不全中的作用。方法　 10例孤立性左室肌致密化不全患者 ,均为男性 ,年龄 2～ 6 0 (39± 17.7)岁。分别采用美国HP 5 5 0 0及日本Toshiba 140A型二维及多普勒超声心动图仪进行检查 ,探头频率 2 .5 3 .5MHz。部分患者接受超高速CT或磁共振成像检查。结果　 10例患者均有程度不同的左心功能不全、心律失常等临床表现 ,未见栓塞征象。孤立性左室肌致密化不全超声心动图特征如下 :(1)二维超声心动图可见多发性突入心室腔内的肌小梁 ,且呈节段性分布。好发于左室心尖部、前侧壁 ;(2 )多普勒超声心动图显示深陷于肌小梁隐窝间的血流与左室腔交通 ;(3)左心室壁呈非均匀性增厚或变薄。 10例患者均符合上述诊断标准。 2例行超高速CT检查 ,2例行磁共振成像检查 ,4例患者均可见左室心尖部、前侧壁肌小梁粗大 ,左心室腔明显扩大 ;小梁区心室壁明显增厚。 10例患者均因左心功能不全给予常规利尿剂、血管扩张剂及抗凝治疗。结论　孤立性左心室肌致密化不全是一种罕见的先天性心肌病 ,临床上可表现为渐进性左心功能不全、致命性心律失常及栓塞征象。超声心动图是无创诊断孤立性左室肌致密化不全的准确、可靠方法     

心肌致密化不全并发窦房传导阻滞1例报告并文献复习

患者男,20岁,平素身体健康,无心肌炎等心脏病史,亦无黑朦、晕厥、心悸、胸闷等不适,且完全能胜任日常体力活动。在我院行健康体检而发现心肌致密不全并发窦房传导阻滞。体格检查及胸部X片未见异常,心肌标志物等检测结果均正常。超声心动图检查：多切面显示左心室低位乳头肌至心尖部、左心室后壁和下壁可探及多发性突人心腔内的肌小梁,错综排列呈网状,彩色多普勒示小梁间隙内可见血流充盈并与心腔相通,心外膜呈薄层的致密心肌回声,     

扩张型心肌病中心室肌致密化不全的识别

目的:探讨扩张型心肌病(DCM)中心室肌致密化不全(NVM)的识别及临床诊断。方法:收集2006-09-2010-09期间在我院初步诊断为DCM的住院患者共551例,根据患者病史、临床表现、辅助检查(彩色多普勒超声心动图、心电图、心脏磁共振等)、诊治过程以及随访结果等资料进行回顾性分析。结果:551例患者中,经超声心动图及心脏磁共振发现并确诊为NVM的患者34例,占DCM总数的6.17%,其检出率从2006年起呈逐年上升趋势。34例确诊为NVM的患者均表现有不同程度的心力衰竭;32例(94.12%)出现心电图异常;彩色超声心动图检测34例均可见NVM的典型改变(100%),其病变部位均累及左室;20例NVM患者平均随访(23±11)个月,4例死亡,主要死因为心力衰竭加重。结论:NVM患者以心力衰竭和心律失常为主要表现就诊,在DCM患者超声心动图检测时要注意识别DCM的病因之一———NVM,其检出率将会逐年上升。由于NVM预后不良,药物治疗只能缓解症状,心脏移植为终末阶段的治疗选择。     

Left ventricular noncompaction with a single coronary artery of anomalous origin

Noncompaction of ventricular myocardium (NVM) is a morphogenetic anomaly of ventricular myocardium that leads to the development of cardiomyopathy. It is frequently associated with other congenital cardiac malformations. A 75-year-old woman was admitted with resting dyspnea lasting for several days. Two-dimensional echocardiography demonstrated an enlarged left ventricle with global impairment of systolic function. The myocardial trabeculations, with multiple recesses, were observed at the apex and mid-ventricular segment of the left ventricle. Contrast echocardiography showed filling of the recesses with the contrast agent. Dynamic contrast magnetic resonance imaging also showed distinctive features of NVM corresponding to the echocardiographic findings. In addition, a coronary artery originating from the proximal ascending aorta was observed. Aortogram and coronary angiography confirmed that the coronary artery had an aberrant origin from the ascending aorta and right coronary artery was an anomalous origin from left anterior descending coronary artery. This case suggests that NVM can present with other life threatening cardiovascular anomalies and different imaging studies are helpful for a comprehensive diagnosis.     

Isolated form of spongy myocardiopathy]

Noncompaction of the ventricular myocardium sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The characteristic echocardiographic findings of this disease consist of multiple myocardial trabeculations and deep intertrabecular recesses communicating with the left ventricular cavity. Familial occurrence has been observed. We present an illustrative case of isolated noncompaction of the ventricular myocardium in a 16-year-old patient, with the typical clinical and echocardiographic features of the disease. The literature on the topic is reviewed.     

Endocardial and epicardial radiofrequency ablation of ventricular tachycardia associated with dilated cardiomyopathy: the importance of low-voltage scars

OBJECTIVES: The purpose of this study was to evaluate the occurrence, locations, and relationship of ventricular tachycardia (VT) to low-voltage areas in dilated cardiomyopathy (DCM). BACKGROUND: The substrate causing monomorphic VT after infarction is characterized by regions of low-voltage (<1.5 mV) scar on electroanatomic maps. The substrate causing VT associated with DCM is less well defined. METHODS: A total of 28 patients were studied with endocardial (26 patients) and epicardial (8 patients) electroanatomic mapping. The VT circuits were defined by entrainment or pace mapping. RESULTS: Ventricular tachycardia was due to focal VT in 5, bundle-branch re-entry in 2, and myocardial re-entry in 22 patients (both focal and re-entry VTs in 1 patient). All patients with myocardial re-entry had endocardial (20 of 20 patients) and/or epicardial (7 of 7 patients mapped) scar. Most (63%) endocardial scars were adjacent to a valve annulus. Of the 19 VT circuit isthmuses identified, 12 were associated with an endocardial scar and 7 with an epicardial scar. All myocardial re-entrant VTs were abolished in 12 of 22 patients, and inducible VT was modified in 4 patients. During follow-up of 334 +/- 280 days, 54% of patients with myocardial re-entry were free of VT despite frequent episodes before ablation. CONCLUSIONS: The VTs in DCM are most commonly the result of myocardial re-entry associated with scar. Scars are often adjacent to a valve annulus, deep in the endocardium, and can be greater in extent on the epicardium than on the endocardium. The use of epicardial mapping and radiofrequency is likely to improve success.     

Pathogenesis of dilated cardiomyopathy: a study based on comparison of the clinical features with other related conditions

To investigate the pathogenesis and pathophysiology of dilated cardiomyopathy (DCM), we studied 28 patients with DCM by echocardiography and endomyocardial biopsy, and compared their findings with those of 34 patients including eight with myocarditis, seven with alcoholics, 12 with hypertensives and seven patients with hypertrophic cardiomyopathy. All 12 patients in the hypertensive group had congestive heart failure without accompanying high blood pressure, and prominent dilatation and uniform wall motion abnormality of the left ventricle observed echocardiographically on admission. After medical management, both heart failure and the echocardiographic abnormalities gradually resolved. Those in the alcoholic group had larger left ventricles and uniform wall motion abnormality compared to those in the other groups. The myocarditis and hypertrophic cardiomyopathy groups had smaller left ventricles, non-uniform wall motion and larger % myocardial fibrosis. Both ventricles in the hypertrophic cardiomyopathy group were thicker than those of the other three groups. Each patient with DCM had individual echocardiographic abnormalities, which could be categorized as two subsets depending on the degree of left ventricular dilatation and uniformity of the wall motion. The one was characterized by a prominently dilated left ventricle and uniform wall motion abnormality similar to the alcoholic group, and the other had less marked left ventricular dilatation and heterogeneous wall motion abnormality similar to the myocarditis group. From these findings, it was suggested that there are common factors to specific myocardial disease in the pathogenesis and pathophysiology of DCM, and thus, DCM might include many subsets of different etiologies.     

Isolated non-compaction of left ventricular myocardium]

Noncompaction of the ventricular myocardium, also known as "spongy myocardium", is a rare congenital abnormality resulting from an arrest in the normal endomyocardial embryogenesis. The echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intratrabecular recesses in communication with the left ventricular cavity. This entity is a not well known cause of dilated cardiomyopathy. Some cases were described as X-linked familial forms. We report the clinical case of a 13-year-old female patient with severe left ventricular disfunction, a very trabeculated left ventricle on echocardiography and two admissions in class IV heart failure.     

Noncompaction of the ventricular myocardium combined with polycystic kidney disease

Noncompaction of the ventricular myocardium (NVM) is a rare cardiac abnormality of unknown etiology. The condition is characterized by prominent and excessive trabeculations in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Polycystic kidney diseases are characterized by the formation of multiple cysts in the kidneys and liver and, less frequently, in the pancreas. Cardiovascular abnormalities including hypertension, mitral valve prolapse, and intracranial aneurysms are also frequently recognized. However, polycystic kidney disease and isolated ventricular noncompaction have not previously been correlated. Here, we describe one case of isolated noncompaction of ventricular myocardium with polycystic kidney disease, coupled with a progressive worsening of heart failure. We confirmed these abnormalities using contrast echocardiography, abdominopelvic computed tomography, and cardiac magnetic resonance imaging.     

Isolated noncompaction of the left ventricular myocardium in an elderly patient.

Noncompaction of the ventricular myocardium (NVM) is a rare disorder of endomyocardial morphogenesis characterized by numerous, prominent trabeculations and deep intertrabecular recesses. It is commonly associated with congenital heart disease, but the isolated form (INVM) is not associated with other structural heart diseases. Clinical reports of INVM have been limited to a few case reports and small series of pediatric patients. INVM is considered to be a form of congenital abnormal endomyocardial morphogenesis caused by abnormal cessation of the embryonic development of the ventricular myocardium; most reported cases have been pediatric patients, and autopsy cases of elderly patients have been quite rare. In the present case, an elderly female had INVM associated with severely disturbed left ventricular (LV) function and an enlarged left ventricle similar to dilated cardiomyopathy. The echocardiogram showed prominent trabeculations and deep intertrabecular recesses of the LV walls, especially in the posterior and apical areas. LV contrast echocardiography revealed markedly protruberant trabeculations, which were also observed with computed tomography. Five years later, the patient died of refractory heart failure and ventricular fibrillation. The autopsy revealed numerous excessively prominent trabeculations in the LV myocardium, with deep intertrabecular recesses containing thrombi.     

Left ventricular noncompaction associated with a sinoatrial node artery originating from the posterolateral branch of the right coronary artery

Noncompaction of the ventricular myocardium (NVM) is a rare unclassified cardiomyopathy which is characterized by multiple prominent trabeculations and deep intertrabecular recesses. This cardiomyopathy can be isolated or in combination with other congenital cardiac disorders, including coronary artery abnormalities. A 56-year-old female patient presented to the cardiology department with complaints of exertional dyspnoea and chest pain. Transthoracic echocardiography revealed left ventricular dilatation with diffuse hypokinesis. Multiple prominent trabeculations with deep inter-trabecular recesses were observed at the left ventricular apex. Also, coronary angiography demonstrated a sinoatrial node artery originating from the posterolateral branch of the right coronary artery.     

Noncompaction of the ventricular myocardium: report of two cases with bicuspid aortic valve demonstrating poor prognosis and with prominent right ventricular involvement

Noncompaction of the ventricular myocardium is a rare, unclassified cardiomyopathy due to an arrest of myocardial morphogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular spaces communicating with the left ventricular (LV) cavity. The disease typically involves the LV myocardium, but right ventricular (RV) involvement is not uncommon. The clinical manifestations include heart failure (HF) signs, ventricular arrhythmias and cardioembolic events. Noncompacted myocardium may occur as an isolated cardiac lesion, as well as it can be in association with congenital anomalies. We describe two illustrative cases of noncompaction of the ventricular myocardium, a 19-year-old male with bicuspid aortic valve and progressive worsening of HF, and a 61-year-old male with marked RV involvement in addition to LV apical involvement, both with the typical clinical and echocardiographic features of the disease.