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Kokyu to Junkan Volume 32, Issue 3 （March 1984）

呼吸と循環 32巻3号（1984年3月発行）

Japanese English

研究

進行性筋ジストロフィー症における僧帽弁逸脱症の成因についての検討—胸椎，胸郭の変形および左心機能との関係について The causative mechanisms of mitral valve prolapse in progressive muscular dystrophy:Thorax and thoracic spine deformity and left ventricular dysfunction 矢澤良光 ¹ , 宮谷信行 ¹ , 高澤直之 ¹ , 柴田昭 ² Yoshimitsu Yazawa ¹ , Nobuyuki Miyatani ¹ , Naoyuki Takasawa ¹ , Akira Shibata ² ¹国立新潟療養所 ²新潟大学医学部第一内科 ¹Niigata National Sanatorium ²First Department of Internal Medicine, Niigata University School of Medicine pp.275-279

発行日 1984年3月15日 Published Date 1984/3/15

DOI https://doi.org/10.11477/mf.1404204407

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Abstract 文献概要
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　進行性筋ジストロフィー症（progressive muscular dys—trophy,PMD）では僧帽弁逸脱症（mitral valve pro—lapse,MVP）が高頻度に合併することが報告されている^1〜6）。MVPの成因に対しても検討されているが，その主要な要因としては，左室心筋や乳頭筋の変性および線維化による左室のasynergyや乳頭筋の機能不全が報告されている^2〜4）。

　一方，PMDにおいては胸椎や胸郭の変形が次第に進むことが知られており，この胸郭変形の心肺機能への影響は充分に考えられるところである。全身の結合組織の疾患やその疑いの疾患とMVPとの関係については多くの報告がみられ^7〜14），僧帽弁やその支持組織にも何らかの組織変化が同時に存在することがMVPの原因として考えられている。

　The causative mechanisms of mitral valve prolapse (MVP) were evaluated in 58 patients (pts) with progressive muscular dystrophy (PMD). Two possible causes, 1) left ventricular (LV) dysfunction and 2) thorax and thoracic spine deformities were assessed. Pts were classified into three groups by echocardiographic findings. Group 1 (GI) : 31 pts MVP, G2 : 11 pts c MVP by M-mode echocardiogram only, G3 : 16 pts c MVP by both twodimensional and M-mode echocardiograms.

　LV functions evaluated by systolic time intervals and fractional shortening showed no significant differences among three groups.

　The degree of scoliosis of the thoracic spine defined by chest plain film was not related to the incidence of MVP. On the other hand, the pts with anteriorly deformed thoracic spines were found to have a very high incidence of MVP as follows. Lordotic or straight spines were found in 32.3%, 100%, 93.8% in Gl, G2 and G3 respectively. And the incidence of MVP in cases with kyphosis, straight spine and lordosis was 4.8%, 66.7% and 77.8% respectively.

　The shape of the thorax defined as the ratio of anteroposterior (AP) internal diameter and transverse internal diameter was more flattened in G2 and G3 than in G1.

　From these results, we concluded as follows :

　1) Depressions of LV function were not related to the incidence of MVP.

　2) The lordotic or straight spines and the thoraces with short AP internal thoracic diameters were thought to be major factors for the occurrence of MVP in PMD.