僧房弁弁輪部石灰化を合併した若年者Marfan症候群の治験例

A case report of surgical treatment of Marfan's syndrome with mitral annular calcification at young age

　Marfan症候群は心血管系に重篤な合併症を示すことが多く，成人では大動脈瘤や大動脈弁病変，小児では僧帽弁病変を示すことが多い。著者らはMarfan症候群と考えられる16歳の女性の僧帽弁閉鎖不全（以下MRと略す）を経験したが，この症例は16歳の若年でありながら僧帽弁弁輪部に石灰化を伴っていた。僧帽弁弁輪部の石灰化は加齢変化と考えられ，20歳未満の僧帽弁弁輪部の石灰化は極めて希有な現象であるので文献的考察も含めて報告する。

A 16 year old girl with Marfan's syndrome and mitral regurgitation is presented in this paper. At the time of preoperative cardiac catheterization, abnormal calcification of the mitral annulus was recognized. Operative findings showed floppy mitral valve, dilatation of the mitral valve annulus and the calcification of the posterior mitral annulus.

In this paper, the etiology of calcification of the mitral annulus, the relation between floppy valve and mitral annulus and clinical course of mitral annulus calcification are described. The calcification of the mitral annulus in the young patients with Marfan's syndrome were reported frequently. This case is the youngest case among reports published previously.