自己心膜により僧帽弁後尖パッチ拡大を行った神経線維腫症Ⅰ型の1例

Posterior Mitral Leaflet Patch Augmentation with Autologous Pericardium in a Neurofibromatosis TypeⅠPatient：Report of a Case

はじめに　神経線維腫症Ⅰ型（NFⅠ）はカフェ・オ・レ斑や神経線維腫を主徴とし，皮膚病変のほか眼病変，骨病変，脊髄腫瘍などの多彩な症候を呈する．3.6％に血管障害を合併するとされ1），腎動脈や椎骨動脈，鎖骨下動脈などの狭窄や閉塞，瘤化が報告されている2～4）．心臓手術においては，冠状動脈の瘤化や閉塞により冠状動脈血行再建術の報告も散見されるが5～7），弁膜症手術はまれである．われわれは，僧帽弁閉鎖不全症（MR），三尖弁閉鎖不全症（TR），心房細動を合併したNFⅠ例に対し，僧帽弁形成術，三尖弁輪形成術，maze手術を施行したので報告する．

The patient was a 61-year-old man with neurofibromatosis typeⅠ, who had several papules in a whole body. Medical history included atrial fibrillation and cerebral embolism. Transthoracic and transesophageal echocardiogram revealed severe mitral valve regurgitation and tricuspid valve regurgitation due to annular dilation with atrial enlargement, tethering of the posterior mitral leaflet, the anterior mitral leaflet prolapse with chordal rupture. Mitral valve repair and tricuspid annuloplasty, maze procedure were performed via median sternotomy. Mitral valve repair was performed by chorda reconstruction with artificial chordae to A2, patch-augmentation of the posterior leaflet with 0.6％ glutaraldehyde-treated autologous pericardial patch and ring annuloplasty. There was no abnormal bleeding during surgery, and surgical site infection was not observed. Postoperative echocardiogram showed the augmented posterior leaflet created a deep and tightly uniform coaptation to the anterior leaflet and mitral regurgitation was well controlled.