Divisions of Cardiovascular Diseases, General Internal Medicine, and Pediatric Cardiology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
J Am Coll Cardiol. 2010 Apr 27;55(17):1769-79. doi: 10.1016/j.jacc.2009.12.040.
Infiltrative cardiomyopathies are characterized by the deposition of abnormal substances that cause the ventricular walls to become progressively rigid, thereby impeding ventricular filling. Some infiltrative cardiac diseases increase ventricular wall thickness, while others cause chamber enlargement with secondary wall thinning. Increased wall thickness, small ventricular volume, and occasional dynamic left ventricular outflow obstruction (e.g., amyloidosis) can outwardly appear similar to conditions with true myocyte hypertrophy (e.g., hypertrophic cardiomyopathy, hypertensive heart disease). Likewise, infiltrative disease that presents with a dilated left ventricle with global or regional wall motion abnormalities and aneurysm formation (e.g., sarcoidosis) may mimic ischemic cardiomyopathy. Low-voltage QRS complex was the sine qua non of infiltrative cardiomyopathy (i.e., cardiac amyloid). However, low-voltage QRS complex is not a uniform finding with the infiltrative cardiomyopathies. The clinical presentation, along with functional and morphologic features, often provides enough insight to establish a working diagnosis. In most circumstances, however, tissue or serologic evaluation is needed to validate or clarify the cardiac diagnosis and institute appropriate therapy.
浸润性心肌病的特征是异常物质的沉积,导致心室壁逐渐变硬,从而阻碍心室充盈。一些浸润性心脏病会增加心室壁厚度,而另一些则会导致心室扩大,继而出现壁变薄。壁增厚、心室容积小和偶尔出现的左心室流出道动态梗阻(如淀粉样变性),在外在表现上可能与真正的心肌肥厚(如肥厚型心肌病、高血压性心脏病)的情况相似。同样,以左心室扩张伴整体或局部室壁运动异常和动脉瘤形成为特征的浸润性疾病(如结节病),可能类似于缺血性心肌病。低电压 QRS 波群是浸润性心肌病(即心脏淀粉样变性)的必备条件。然而,低电压 QRS 波群并不是浸润性心肌病的一致表现。临床表现,以及功能和形态学特征,通常可以提供足够的洞察力来确立一个初步诊断。然而,在大多数情况下,需要进行组织学或血清学评估来验证或澄清心脏诊断,并采取适当的治疗。