Doyama K, Hirose K, Fujiwara H
Cardiovascular Department, Ohtsu Red-Cross Hospital, Shiga, Japan.
Chest. 1990 Jun;97(6):1480-1. doi: 10.1378/chest.97.6.1480.
A 41-year-old woman had Noonan's syndrome. Her heart was complicated by asymmetric septal hypertrophy, hypertrophy of the left ventricular free wall, severe pulmonary stenosis, and right ventricular hypertension. On autopsy, a quantitative histologic analysis of the heart revealed that the area of disarray was limited both to the ventricular septum and the left ventricular free wall as in a normal heart. This is not typical of hypertrophic cardiomyopathy because the extent of disarray is high in most cases of hypertrophic cardiomyopathy. Some form of hypertrophic cardiomyopathy, however, seemed to be present in this patient because right ventricular pressure overload did not affect the left ventricular free wall. To clarify the relation between hypertrophic cardiomyopathy and Noonan's syndrome, quantitative histologic analysis is necessary.
一名41岁女性患有努南综合征。她的心脏合并有不对称性室间隔肥厚、左心室游离壁肥厚、严重肺动脉狭窄和右心室高压。尸检时，对心脏进行的定量组织学分析显示，心肌紊乱区域仅限于室间隔和左心室游离壁，如同正常心脏一样。这并非肥厚型心肌病的典型表现，因为在大多数肥厚型心肌病病例中，心肌紊乱程度较高。然而，该患者似乎存在某种形式的肥厚型心肌病，因为右心室压力超负荷并未影响左心室游离壁。为阐明肥厚型心肌病与努南综合征之间的关系，进行定量组织学分析是必要的。
