Huelmos A, Barba J, Martínez-Caro D
Departamento de Cardiología, Facultad de Medicina, Universidad de Navarra, Pamplona.
Rev Esp Cardiol. 1996 Jul;49(7):523-31.
Currently, cardiomyopathies are defined as "heart muscle diseases of unknown etiology". Restrictive cardiomyopathy is the rarest type of this disease, and the only one with no uniformly accepted diagnostic criteria. Its importance lies primarily in properly distinguishing it from constrictive pericarditis because of the direct consequences of mistakenly treating a case of constrictive pericarditis as if it were inoperable. Restrictive cardiomyopathy must be considered within the broader spectrum of diastolic dysfunction. Diagnostic criteria used show a general consensus for the diagnosis of restrictive cardiomyopathy in patients with clinical signs of heart failure in the presence of a nondilated, nonhypertrophic left ventricle with preserved contractility but abnormal diastolic function. Characteristically, right and left ventricular filling pressures are elevated and a dip and plateau diastolic pressure pattern appears. Restrictive cardiomyopathy may be either idiopathic and thus be a true cardiomyopathy in the strict sense of the term or secondary to an infiltrative disease of the myocardium. In this article we review the clinical and hemodynamic features of this disease and the different imaging techniques used in patients to help differentiate restrictive cardiomyopathy from constrictive pericarditis. Finally we study some types of restrictive cardiomyopathies.
目前，心肌病被定义为“病因不明的心肌疾病”。限制性心肌病是这种疾病中最罕见的类型，也是唯一没有统一公认诊断标准的类型。其重要性主要在于要正确地将其与缩窄性心包炎区分开来，因为将缩窄性心包炎误诊为无法手术治疗会带来直接后果。限制性心肌病必须在更广泛的舒张功能障碍范围内加以考虑。对于存在心力衰竭临床体征、左心室无扩张、无肥厚且收缩功能正常但舒张功能异常的患者，所采用的诊断标准在限制性心肌病的诊断方面已达成普遍共识。其特征是左右心室充盈压升高，并出现舒张期压力的下陷和平台模式。限制性心肌病可能是特发性的，因此从严格意义上讲是一种真正的心肌病，也可能继发于心肌的浸润性疾病。在本文中，我们回顾了这种疾病的临床和血流动力学特征，以及用于帮助区分限制性心肌病与缩窄性心包炎的不同影像学技术。最后，我们研究了一些类型的限制性心肌病。
