Lassabe G, Kieny J R, Dumeny P, Grison D, Sacrez A
Sem Hop. 1983 Dec 1;59(44):3047-51.
33 patients with hypertrophic cardiomyopathy were followed for a mean duration of 48 months. 29 of them were reviewed; 6 had died, including 4 with a familial form belonging to two different families. No cases of sudden death were observed. The mortality rate was 4 p. cent at 1 year, 11 p. cent at two years and 21 p. cent at 5 years. The clinical course was marked by a functional deterioration in one-third of cases and, on echocardiography, by an increase in the diastolic diameter of the left ventricle and in the thickness of the septum, independent of the clinical course. Subjects from "high risk" families have a very poor prognosis (4 deaths out of 7 patients at an average age of 25). These families present major conductive disturbances on the electrocardiogram and a very marked parietal hypertrophy on the echocardiogram. No other prognosis factor independent of the familial aspect was revealed.
对33例肥厚型心肌病患者进行了平均48个月的随访。其中29例接受了复查;6例死亡,包括4例家族性形式的患者,分属于两个不同家族。未观察到猝死病例。1年时死亡率为4%,2年时为11%,5年时为21%。临床病程的特点是三分之一的病例出现功能恶化,超声心动图显示左心室舒张直径和室间隔厚度增加,与临床病程无关。“高危”家族的患者预后很差(7例患者中有4例死亡,平均年龄25岁)。这些家族在心电图上表现出严重的传导障碍,在超声心动图上表现出非常明显的心肌肥厚。未发现与家族因素无关的其他预后因素。