Kawana A, Imaoka C, Kanemoto N
First Department of Internal Medicine, Tokai University School of Medicine, Isehara.
J Cardiol. 1987 Jun;17(2):389-98.
A 20-year-old woman whose echocardiograms showed a rare evolution from hypertrophic to dilated cardiomyopathy during a nine year observation period is described. This patient was initially diagnosed as having hypertrophic obstructive cardiomyopathy (HOCM) at the age of 12 years. Her echocardiogram showed marked thickening of the interventricular septum (IVS) and left ventricular posterior wall (LVPW), asymmetric septal hypertrophy (ASH) and systolic anterior motion of the mitral valve (SAM). Chest radiography revealed a prominent left ventricular border and a cardiothoracic ratio of 0.52. At 18 years of age she experienced onset of palpitation during the 16th week of pregnancy. Her echocardiogram recorded in June 1980 revealed a thickened IVS and LVPW with resolution of the SAM and of the narrow cavity. At 20 years of age she became pregnant again and was admitted to our hospital for the third time at 24 weeks gestation. On admission her blood pressure was 122/60 and her pulse was 56, and moist rales were audible over both lung fields. Peripheral edema was noted. Chest radiography revealed moderate cardiomegaly, a cardiothoracic ratio of 0.66, and congestion of the pulmonary vasculature. An echocardiogram showed thinning of the IVS and LVPW with hypokinesis and dilatation of the cavity compatible with dilated cardiomyopathy (DCM). She delivered a boy on July 15th 1982 at 32 weeks gestation following which she developed marked congestive heart failure. She expired one month later. A chest radiograph made one day before death revealed marked cardiomegaly, a cardiothoracic ratio of 0.76, prominent pulmonary vasculature and a pleural effusion. Autopsy was refused; therefore the terminal DCM-like features were not clarified pathologically. However, the slow progression from HOCM to HCM during eight years and then rapid progression from HCM to DCM during four months was most impressive.
本文描述了一名20岁女性，在9年的观察期内，其超声心动图显示出从肥厚型心肌病罕见地演变为扩张型心肌病的过程。该患者最初在12岁时被诊断为肥厚型梗阻性心肌病（HOCM）。她的超声心动图显示室间隔（IVS）和左心室后壁（LVPW）明显增厚，不对称性室间隔肥厚（ASH）以及二尖瓣收缩期前向运动（SAM）。胸部X线检查显示左心室边界突出，心胸比率为0.52。18岁时，她在怀孕第16周时出现心悸。1980年6月记录的超声心动图显示IVS和LVPW增厚，SAM和狭窄腔隙消失。20岁时她再次怀孕，并在妊娠24周时第三次入院。入院时她的血压为122/60，脉搏为56，双肺野可闻及湿啰音。发现有外周水肿。胸部X线检查显示中度心脏扩大，心胸比率为0.66，肺血管充血。超声心动图显示IVS和LVPW变薄，运动减弱，腔隙扩张，符合扩张型心肌病（DCM）。她于1982年7月15日妊娠32周时产下一名男婴，随后出现明显的充血性心力衰竭。一个月后她去世。死亡前一天拍摄的胸部X线片显示明显的心脏扩大，心胸比率为0.76，肺血管突出和胸腔积液。家属拒绝尸检；因此，终末期类似DCM的特征未得到病理证实。然而，从HOCM到HCM的八年缓慢进展以及从HCM到DCM的四个月快速进展最为引人注目。
