Rippe J, Fishbein M C, Carabello B, Angoff G, Sloss L, Collins J J, Alpert J S
Br Heart J. 1980 Dec;44(6):621-9. doi: 10.1136/hrt.44.6.621.
Four hundred and ninety-nine surgically excised valves were examined for pathological evidence of myxomatous degeneration. Thirty-six valves (7%) had myxomatous degeneration as a major pathological finding. Fourteen valves (3%) had significant myxomatous degeneration of the pars fibrosa, a finding which we define as "primary myxomatous degeneration". Echocardiographic findings and catheterisation results were correlated with the clinical course, surgical results, and follow-up in these 14 patients. Echocardiograms in 10 of the 11 patients who had them (91%) showed abnormalities suggesting the presence of primary myxomatous degeneration. Echocardiography was more helpful than angiography in diagnosis. The histological pattern of primary mitral myxomatous degeneration appears to be identical to that seen in patients with mitral valve prolapse and five of six patients with mitral lesions had echocardiographic evidence of prolapse. None of the patients with primary myxomatous degeneration of the aortic valve had syphilis of Marfan's syndrome. While the aetiology of primary myxomatous degeneration of cardiac valves is not known, a link to a more generalised disorder is suggested.
对499个手术切除的瓣膜进行了检查，以寻找黏液瘤样变性的病理证据。36个瓣膜（7%）有黏液瘤样变性作为主要病理发现。14个瓣膜（3%）有纤维部分的显著黏液瘤样变性，我们将这一发现定义为“原发性黏液瘤样变性”。对这14例患者的超声心动图检查结果和心导管检查结果与临床病程、手术结果及随访情况进行了相关性分析。11例进行了超声心动图检查的患者中有10例（91%）显示有提示原发性黏液瘤样变性存在的异常。超声心动图在诊断方面比血管造影更有帮助。原发性二尖瓣黏液瘤样变性的组织学模式似乎与二尖瓣脱垂患者所见相同，6例二尖瓣病变患者中有5例有超声心动图显示的脱垂证据。主动脉瓣原发性黏液瘤样变性的患者中无一例患有梅毒或马方综合征。虽然心脏瓣膜原发性黏液瘤样变性的病因尚不清楚，但提示与一种更广泛的疾病有关。
