Hess O M, Goebel N H, Schneider J, Scholer Y, Krayenbühl H P
Schweiz Med Wochenschr. 1977 Jan 15;107(2):49-57.
In 6 women with the hemodynamic, angiographic and histological findings of endomyocardial fibrosis the clinical course was followed for 30 months. All six cases showed the typical partial obliteration of one (4 patients) or both (2 patients) heart chambers. Left ventricular pressure-volume relations elicited decreased distensibility of the left ventricle. The functional state in 2 patients with severe mitral insufficiency was improved following successful mitral valve replacement. One patient with left ventricular, and 2 patients with biventricular, obliteration were given drug therapy and in one case recompensation lasting 2 years was obtained. One patient with partial obliteration of the left ventricle and severe biventricular failure died after an observation period of 4 years. In summary it can be said that (1) endomyocardial fibrosis in Switzerland is observed mainly in females; (2) the clinical picture is dominated by severe congestive heart failure in which the heart size is only slightly enlarged; (3) systolic performance is normal or only moderately depressed despite severe restriction to filling and/or av-valve insufficiency; (4) partial obliteration of the right or left ventricle may be detected by echocardiography; (5) rheumatic fever may occasionally be the cause of endomyocardial fibrosis; (6) mitral valve replacement is accompanied by appreciable improvement in the patient's condition.
对6例具有心内膜心肌纤维化血液动力学、血管造影及组织学表现的女性患者进行了30个月的临床病程随访。所有6例患者均表现出典型的一个心腔(4例患者)或两个心腔(2例患者)部分闭塞。左心室压力-容积关系显示左心室扩张性降低。2例重度二尖瓣关闭不全患者在成功进行二尖瓣置换术后功能状态得到改善。1例左心室闭塞患者和2例双心室闭塞患者接受了药物治疗,其中1例获得了持续2年的病情代偿。1例左心室部分闭塞且严重双心室衰竭的患者在观察4年后死亡。总之,可以说:(1)瑞士的心内膜心肌纤维化主要见于女性;(2)临床表现以严重充血性心力衰竭为主,心脏大小仅轻度增大;(3)尽管充盈严重受限和/或房室瓣关闭不全,但收缩功能正常或仅中度降低;(4)超声心动图可检测到右心室或左心室部分闭塞;(5)风湿热偶尔可能是心内膜心肌纤维化的病因;(6)二尖瓣置换术可使患者病情明显改善。