Koga Y, Itaya M, Takahashi H, Koga M, Ikeda H, Itaya K, Toshima H
J Cardiogr Suppl. 1985(6):65-74.
Although apical hypertrophy is characterized by a spade-like configuration of the left ventricle and giant negative T waves on electrocardiograms, the identity of apical hypertrophy in the disease spectrum of hypertrophic cardiomyopathy (HCM) is not fully established. The present study compared the demography, familial occurrence, and acquired factors of 43 patients who had apical hypertrophy with those of 104 patients who had asymmetric septal hypertrophy (ASH). Demographically, apical hypertrophy occurred predominantly in middle-aged males (86%). Family surveys showed that 13% of siblings of apical hypertrophy were affected, significantly less than in obstructive (31%) or non-obstructive (29%) HCM with ASH. Thirty-eight percent of siblings of ASH patients less than 35 years of age were affected, with a male/female ratio of 4/5, suggesting an autosomal dominant inheritance. The acquired factors associated with apical hypertrophy were assessed in a case-control study. Relative risk (odds ratio) of the condition was 3.46 (p less than 0.05) in those with histories of hypertension, and increased further to 8.09 (p less than 0.001) in those who were often hypertensive according to their physician's evaluations. Thus a strong association of hypertension with apical hypertrophy was suggested. However, hypertension in this condition was usually mild and labile, the blood pressure reverted to the normal range within several days of hospital admission, implying that transient hypertension during daily activity is associated with apical hypertrophy. Therefore, blood pressure response during exercise stress tests of 25 patients with apical hypertrophy was compared with that of age- and sex-matched controls. Slopes of linear regression between systolic blood pressure and heart rate and oxygen consumption during exercise, were used as indices of blood pressure response. They were significantly greater in apical hypertrophy than in the controls (1.2 +/- 0.4 vs 0.9 +/- 0.3, p less than 0.01 and 4.3 +/- 1.7 vs 2.8 +/- 0.8, p less than 0.001). This trend was observed even in patients without histories of hypertension. These findings suggested that apical hypertrophy has an inheritance pattern different from that of ASH, and has a possible association with acquired factors such as hypertension. Therefore, apical hypertrophy seemed to be a disease entity distinct from HCM with ASH, though it might be included in the disease spectrum of HCM.
尽管心尖肥厚的特征是左心室呈铲状形态以及心电图上出现巨大倒置T波,但心尖肥厚在肥厚型心肌病(HCM)疾病谱中的归属尚未完全明确。本研究比较了43例心尖肥厚患者与104例不对称性室间隔肥厚(ASH)患者的人口统计学特征、家族发病情况及后天因素。在人口统计学方面,心尖肥厚主要发生于中年男性(86%)。家族调查显示,心尖肥厚患者的兄弟姐妹中有13%受影响,显著低于梗阻性(31%)或非梗阻性(29%)ASH型HCM。ASH患者中年龄小于35岁的兄弟姐妹有38%受影响,男女比例为4/5,提示为常染色体显性遗传。在一项病例对照研究中评估了与心尖肥厚相关的后天因素。有高血压病史者患该病的相对风险(比值比)为3.46(p<0.05),而根据医生评估经常患高血压者的相对风险进一步增至8.09(p<0.001)。因此提示高血压与心尖肥厚有很强的关联。然而,这种情况下的高血压通常较轻且不稳定,入院后数天内血压恢复至正常范围,这意味着日常活动期间的短暂高血压与心尖肥厚有关。因此,比较了25例心尖肥厚患者与年龄及性别匹配的对照组在运动应激试验中的血压反应。运动期间收缩压与心率及耗氧量之间的线性回归斜率用作血压反应指标。心尖肥厚患者的这些指标显著高于对照组(分别为1.2±0.4对0.9±0.3,p<0.01;4.3±1.7对2.8±0.8,p<0.001)。即使在无高血压病史的患者中也观察到了这种趋势。这些发现提示心尖肥厚具有与ASH不同的遗传模式,且可能与高血压等后天因素有关。因此,心尖肥厚似乎是一种有别于ASH型HCM的疾病实体,尽管它可能包含在HCM疾病谱中。