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本文观察37例小儿肥厚型心肌病(HCM)的自然病程。所有病儿确诊为HCM时年龄均≤14岁,平均7±4岁。其中男24例,女13例。经过心导管检查,发现其中13例(35%)有跨越左室流出道的压力阶差,平均为5.6±3.6kPa(42±27mmHg)。确诊后给予心得安(26例)或/和异搏定(11例)治疗,随访2-18(平均9.2±5.1年)。作者发现:左室流出道有压力阶差的病例,其临床、心电图、血流动力学特点均和无压力阶差者相似。而患儿心功能中～重度减退者(NYHA分级Ⅲ～Ⅳ级),其昏厥发生率较高(p<0.001);血流动力学指标中射血分数较低,肺动脉压和右室舒张末压较高.
This article observed 37 cases of hypertrophic cardiomyopathy (HCM) of the natural course. All children diagnosed with HCM age ≤ 14 years, an average of 7 ± 4 years old. There were 24 males and 13 females. After cardiac catheterization, 13 (35%) of them showed pressure gradient across the left ventricular outflow tract with an average of 5.6 ± 3.6 kPa (42 ± 27 mmHg). After the diagnosis of propranolol (26 cases) or / and verapamil (11 cases) were treated, followed up 2-18 (average 9.2 ± 5.1 years). The authors found that patients with pressure-gradient left ventricular outflow tract had similar clinical, electrocardiographic and hemodynamic characteristics to those without pressure gradient. The cardiac function in children with ~ severe subsidence (NYHA grade Ⅲ ~ Ⅳ grade), the higher the incidence of syncope (p <0.001); hemodynamic indicators of low ejection fraction, pulmonary artery pressure and right ventricular end-diastolic High pressure.