Frischknecht Barbara S, Attenhofer Jost Christine H, Oechslin Erwin N, Seifert Burkhardt, Hoigné Philipp, Roos Malgorzata, Jenni Rolf
Division of Cardiology, University Hospital Zurich, Zurich, Switzerland.
J Am Soc Echocardiogr. 2005 Aug;18(8):865-72. doi: 10.1016/j.echo.2005.03.011.
Echocardiographic characteristics typical of isolated left ventricular noncompaction (IVNC) have been well defined. The aim of this study was to validate diagnostic criteria of IVNC in valvular or hypertensive heart disease (HHD) or dilated cardiomyopathy.
We conducted a retrospective analysis of records and blind review of videotapes of all 19 patients with IVNC seen within 7 years in comparison with randomly selected patients from the same study period with dilated cardiomyopathy (31 patients), HHD (22 patients), and chronic severe valvular heart disease: mitral regurgitation (22 patients); aortic regurgitation (20); and aortic stenosis with bicuspid (22) or tricuspid (22) valves.
Clinical characteristics and electrocardiographic findings did not differ between IVNC and other diseases. In IVNC, all patients had noncompacted (NC) segments with a 2-layered structure and wall thickening, and in most patients perfused recesses (95%) or hypokinetic segments (89%) were present. Both hypertrabeculation or presence of a meshwork were specific for IVNC, but the sensitivity for IVNC was only 11% for hypertrabeculation, respectively, 68% for meshwork. In dilated cardiomyopathy, perfused recesses (48%) and a 2-layered structure (26%) were seen but without wall thickening of these segments; all NC criteria including wall thickening were fulfilled in one patient (3%) only. In valvular heart disease or HHD, perfused recesses and a 2-layered myocardium were rare: two patients (5%) with aortic stenosis and one patient with HHD (5%) had NC. Although in IVNC wall thickening was confined to the 2-layered myocardial segments, it was diffuse in other diseases.
Although some NC criteria are occasionally found in other heart disease, the combination of all criteria is very specific. All criteria of NC are rarely met in other disease than IVNC (< or = 5%).
孤立性左心室心肌致密化不全（IVNC）的典型超声心动图特征已得到明确界定。本研究的目的是验证IVNC在瓣膜性或高血压性心脏病（HHD）或扩张型心肌病中的诊断标准。
我们对7年内所见的19例IVNC患者的记录进行了回顾性分析，并对录像带进行了盲法审查，同时与同一研究期间随机选择的扩张型心肌病患者（31例）、HHD患者（22例）以及慢性重度瓣膜性心脏病患者：二尖瓣反流（22例）；主动脉瓣反流（20例）；以及二叶式（22例）或三叶式（22例）瓣膜的主动脉瓣狭窄患者进行比较。
IVNC与其他疾病之间的临床特征和心电图表现无差异。在IVNC中，所有患者均有具有两层结构和壁增厚的心肌致密化不全（NC）节段，且大多数患者存在灌注隐窝（95%）或运动减弱节段（89%）。粗大的肌小梁或网状结构的存在对IVNC具有特异性，但粗大肌小梁对IVNC的敏感性仅为11%，网状结构的敏感性分别为68%。在扩张型心肌病中，可见灌注隐窝（48%）和两层结构（26%），但这些节段无壁增厚；所有包括壁增厚的NC标准仅在1例患者（3%）中得到满足。在瓣膜性心脏病或HHD中，灌注隐窝和两层心肌很少见：2例主动脉瓣狭窄患者（5%）和1例HHD患者（5%）有NC。尽管在IVNC中壁增厚局限于两层心肌节段，但在其他疾病中是弥漫性的。
尽管在其他心脏病中偶尔会发现一些NC标准，但所有标准的组合具有很高的特异性。除IVNC外，其他疾病很少同时满足所有NC标准（≤5%）。
