Kuhn H, Gietzen F, Mercier J, Lösse B, Köhler E, Schulte H D, Bircks W, Loogen F
Z Kardiol. 1983 Feb;72(2):83-98.
To characterize the different types of hypertrophic cardiomyopathy [typical (subaortic) hypertrophic obstructive cardiomyopathy (HOCM) (n = 235), atypical (midventricular or apical) HOCM (n = 33), and hypertrophic nonobstructive cardiomyopathy (HNCM) (n = 85)], studies of the clinical picture, course, and prognosis were performed in 353 patients. Clinical picture. There were found to be distinct differences between the diseases in terms of incidence, symptoms, findings at auscultation, carotid pulse tracings, ECG (incidence of abnormal negative T-waves), and echocardiography. Echochardiography proved to be diagnostically less specific than invasive methods, however, and in particular often failed to distinguish between atypical HOCM and HNCM. Clinical course. There was no evidence of a change from one form of hypertrophic cardiomyopathy to the other. The Sokolow-Lyon index in the ECG did not increase in any group. The rate of complications (endocarditis, systemic emboli) varied between 0.61 and 1.28 events per 100 patient years. Only 7.4%-23.5% of patients with HOCM improved as a result of conservative treatment, compared to 83%-87.5% of surgical patients. In addition, the rate of postoperative syncope was reduced by 90%. Prognosis. 90 patients were operated on. Operative mortality in typical HOCM was 4.5% over the last 5 years. If this is disregarded, the cumulative survival rates are significantly higher in surgical patients with typical HOCM than in those treated conservatively. The data confirm surgical treatment to be the therapy of choice in patients with HOCM refractory to conservative treatment. The prognosis seems to be improved by operation.
为了对不同类型的肥厚型心肌病进行特征描述[典型(主动脉瓣下)肥厚型梗阻性心肌病(HOCM)(n = 235)、非典型(心室中部或心尖部)HOCM(n = 33)以及肥厚型非梗阻性心肌病(HNCM)(n = 85)],对353例患者进行了临床表现、病程及预后的研究。临床表现。发现这些疾病在发病率、症状、听诊结果、颈动脉脉搏描记图、心电图(异常负向T波的发生率)以及超声心动图方面存在明显差异。然而,超声心动图在诊断上的特异性低于侵入性方法,尤其常常无法区分非典型HOCM和HNCM。临床病程。没有证据表明一种肥厚型心肌病会转变为另一种形式。心电图中的索科洛夫 - 里昂指数在任何组中均未升高。并发症(心内膜炎、全身性栓塞)的发生率为每100患者年0.61至1.28次事件。HOCM患者中只有7.4% - 23.5%经保守治疗后病情改善,相比之下手术患者的改善率为83% - 87.5%。此外,术后晕厥发生率降低了90%。预后。90例患者接受了手术。在过去5年中,典型HOCM的手术死亡率为4.5%。若不考虑这一点,典型HOCM手术患者的累积生存率显著高于保守治疗患者。数据证实手术治疗是对保守治疗无效的HOCM患者的首选治疗方法。手术似乎改善了预后。