先天性僧帽弁狭窄症（7ヵ月児）の1手術例

Surgical repair of congenital mitral stenosis

　先天性僧帽弁狭窄症は，内科的治療に抵抗し増悪するため，しばしば乳児早期に手術が必要とされる。しかし，その発生頻度のまれなこと，他の心奇形を合併していることが多いことから，その診断は難しく，正しく診断されて乳児期に手術を行った症例は数少ない。また，その手術成績も乳児例では惨胆たるものである。今回我我は，まれな先天性僧帽弁狭窄症（CMS）の乳児例を経験し，手術を行った。残念ながら患児は3週後に肺合併症にて死亡したが，剖検所見により得られたCMSの病態生理に文献的検討を加え，診断，管理，手術方針について言及する。

This report presented 7 month-old-infunt suffering from congenital mitral stenosis, which was a rare cardiac anomaly. The patient showed the respiratory and heart failure, which were observed early in life. These symptons were recurrent and intractable aga-inst the medical treatment.

At operation, the margins of mitral leaflet were thicked and rolled, the choldae tendineae were shortened, the inter-chordal spaces were completely obliterated by fibrous tissue. The successful openmitral commissurotomy produced the larger mitral orifice (8 mm to 14mm in diameter) and the reduce of elevated left atrial pressure.

In spite of the good course of postoperative hemo-dynamic condition, the respiratory complications (aterectasis and emphysema) were aggravated day after day. The patient died in 22nd postoperative day. At autopsy, mitral valve complex (mitral valve-choldae-papillary muscle) was conformed as "typical type" of congenital mitral stenosis described byRuckman and Van Praagh. Microscopicaly the myo-cardium showed the marked vacuolar degeneration. This change was worse especially in the site of attachment of papillary muscles, comapred to LV free wall, in which the angiographical depression of LV wall motion was recognized preoperatively.

In addition to the investigation of references reported before, we discussed the clinical course, management, diagnosis, and operative procedures in congenital mitral stenosis.