Hashimoto K, Yata Y, Miyamoto H, Morita K, Horikoshi S, Arai T
Department of Cardiac Surgery, Tokyo Jikeikai Medical College.
Kyobu Geka. 1992 Oct;45(11):1027-30.
A 12-year-old girl with Marfan syndrome was referred to our department because of severe mitral regurgitation. The patient was pale and in a pre-shock condition. The echocardiogram revealed a chordae rupture of the posteromedial papillary muscle at the posterior leaflet. A moderate dilatation of the aortic annulus (30 mm) and the ascending aorta (45 mm) without aortic regurgitation was also observed. Emergent mitral valve replacement was performed without replacement of the aortic valve or the ascending aorta. After two months, the patient developed bilateral pneumothorax, which was resistant to continuous suction therapy and finally required surgical treatments. Despite the relatively uneventful recovery, a lethal rupture of dissecting aortic aneurysm into the pericardial cavity, developed four months after the initial operation. We discussed the desired surgical approaches and respiratory problems in patients with connective tissue disorder.
一名患有马凡综合征的12岁女孩因严重二尖瓣反流转诊至我科。患者面色苍白，处于休克前期状态。超声心动图显示后叶后内侧乳头肌腱索断裂。还观察到主动脉瓣环（30毫米）和升主动脉（45毫米）中度扩张，无主动脉反流。紧急进行了二尖瓣置换术，未置换主动脉瓣或升主动脉。两个月后，患者出现双侧气胸，持续吸引治疗无效，最终需要手术治疗。尽管恢复过程相对平稳，但在初次手术后四个月，发生了致命的主动脉夹层动脉瘤破裂进入心包腔。我们讨论了结缔组织疾病患者理想的手术方法和呼吸问题。
