Danchin N, Voiriot P, Godenir J P, Neimann J L, Cherrier F, Faivre G
Presse Med. 1985 Sep 21;14(31):1645-8.
Fourteen cases of apical hypertrophic cardiomyopathy (i.e. hypertrophy localized to the distal half of the left ventricular wall) are reported. There were 12 men and 2 women aged from 21 to 84 years. Only one of the patients presented, at first consultation, with severe functional symptoms, namely stage IV dyspnoea. ECG tracings were always abnormal, but the classical giant T waves were found in only 7 patients. In the 9 patients who had cardiac catheterization the left ventricular end-diastolic pressure was raised, and angiography showed an "ace of spades" diastolic image of the left ventricle with systolic obliteration of its tip. The distribution of parietal hypertrophy was best studied by two-dimensional echocardiography: the left ventricular apex was affected alone in 7 patients and concomitantly with the adjacent segments of the left or right ventricle in the other 7 patients. A family study showed that 3 patients had a descendant with obstructive cardiomyopathy. At a 4.6 years' follow-up the course of the disease was usually favourable. Apical hypertrophic cardiomyopathy is not a particular entity but one of different possible forms of hypertrophic cardiomyopathy. It seems to be benign in most cases.
本文报告了14例心尖肥厚型心肌病（即肥厚局限于左心室壁远端一半）。患者年龄在21至84岁之间，其中男性12例，女性2例。初诊时仅有1例患者出现严重功能症状，即IV级呼吸困难。心电图始终异常，但仅7例患者出现典型的巨大T波。9例行心导管检查的患者左心室舒张末期压力升高，血管造影显示左心室舒张期呈“黑桃A”形影像，心尖部收缩期闭塞。通过二维超声心动图能更好地研究室壁肥厚的分布情况：7例患者仅左心室心尖部受累，另外7例患者左心室心尖部与左心室或右心室相邻节段同时受累。一项家族研究显示，3例患者有后代患梗阻性心肌病。在4.6年的随访中，疾病进程通常良好。心尖肥厚型心肌病并非一种特殊类型，而是肥厚型心肌病的不同可能形式之一。在大多数情况下，它似乎是良性的。
