Kuhn H, Mercier J, Köhler E, Frenzel H, Hort W, Loogen F
Eur Heart J. 1983 Nov;4 Suppl F:93-104. doi: 10.1093/eurheartj/4.suppl_f.93.
Previous clinical studies in patients with hypertrophic cardiomyopathies predominantly comprised patients with typical subaortic HOCM and in many other studies patients with 'hypertrophic' cardiomyopathies were investigated; i.e. no distinction was made between patients with the typical obstructive, the atypical obstructive and the non-obstructive forms of hypertrophic cardiomyopathy. In addition, in the more differentiated studies, the differential diagnosis between HOCM and HNCM was based mainly on the presence and absence of the subaortic pressure gradient. This is also true for the clinical characterization of both types of hypertrophic cardiomyopathy. Finally, diagnostic descriptions of atypical (mid-ventricular) HOCM exist only in the form of case reports, i.e. they are not based on observations in a large number of patients. Therefore a comparative study in 353 patients with hypertrophic cardiomyopathies was designed to characterize the clinical picture of patients with HNCM and with atypical HOCM in relation to typical HOCM with special reference to the differential diagnosis. The study revealed a profoundly different diagnostic profile of the three subsets of hypertrophic cardiomyopathies. The distinction between the three subsets may serve as a more differentiated basis for clinical studies related to diagnosis, therapeutic approaches, long-term follow-up observations and prognostic evaluation of patients with hypertrophic cardiomyopathies.
先前针对肥厚型心肌病患者的临床研究主要纳入典型的主动脉瓣下肥厚型梗阻性心肌病患者,在许多其他研究中,研究对象为“肥厚型”心肌病患者;也就是说,未对典型梗阻性、非典型梗阻性和非梗阻性肥厚型心肌病患者进行区分。此外,在更具区分性的研究中,肥厚型梗阻性心肌病与非肥厚型梗阻性心肌病之间的鉴别诊断主要基于主动脉瓣下压力阶差的有无。这对于两种类型肥厚型心肌病的临床特征描述同样适用。最后,非典型(心室中部)肥厚型梗阻性心肌病的诊断描述仅以病例报告的形式存在,即并非基于对大量患者的观察。因此,设计了一项针对353例肥厚型心肌病患者的对比研究,以描述非肥厚型梗阻性心肌病和非典型肥厚型梗阻性心肌病患者相对于典型肥厚型梗阻性心肌病患者的临床情况,并特别提及鉴别诊断。该研究揭示了肥厚型心肌病三个亚组截然不同的诊断特征。这三个亚组之间的区分可为有关肥厚型心肌病患者的诊断、治疗方法、长期随访观察和预后评估的临床研究提供更具区分性的基础。