Semsarian C, French J, Trent R J, Richmond D R, Jeremy R W
Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, NSW.
Aust N Z J Med. 1997 Feb;27(1):51-8. doi: 10.1111/j.1445-5994.1997.tb00914.x.
Hypertrophic cardiomyopathy (HCM) is associated with mutations of genes coding for major sarcomeric proteins, but the mechanism of hypertrophy is unknown. As hypertrophy may not develop until adolescence, an altered response to physiological growth stimuli may regulate the hypertrophy process.
This study examined the relationship between age and changes in left ventricular (LV) wall thickness in patients with HCM.
Forty-three patients who had definite electrocardiographic and echocardiographic evidence of HCM were studied with serial 2D and M-mode echocardiograms at least two years apart (mean interval 5.5 +/- 3.0 years). LV cavity dimensions, septal and posterior wall thicknesses, and LV mass indices were compared with data from an age- and gender-matched control group.
In patients with HCM aged ten to 20 years (n = 9), there was an increase in septal wall thickness during the study period from 15.9 +/- 6.2 mm to 19.3 +/- 2.1 mm (p < 0.01). This increase (3.4 +/- 2.5 mm) greatly exceeded the change in septal thickness observed in the control group between the ages of ten and 20 years (0.8 +/- 0.3 mm, p < 0.01). There was a lesser increase in posterior wall thickness from 9.8 +/- 2.1 mm to 11.5 +/- 3.5 mm (p = 0.07). In patients with HCM aged 21-40 years (n = 11), there was also an increase in septal wall thickness during the study period from 16.0 +/- 2.2 mm to 17.8 +/- 3.0 mm (p < 0.05), but no change in septal thickness in the control group. In contrast, the patients aged > 40 years (n = 23) showed no significant change in either septal or posterior wall thickness during the study period. LV mass index increased in the ten to 20 years age group from 128 +/- 24 g/m2 to 164 +/- 20 g/m2 (p = 0.01), but this increase was not observed in the older age groups.
LV hypertrophy is progressive, particularly in the septum, during adolescence and early adult life in patients with HCM. As progressive hypertrophy may continue after somatic growth has ceased, an abnormal myocardial response to physiological growth regulators is less likely to be the principal stimulus to hypertrophy. Gene-gene interactions, changes in haemodynamic load or environmental factors may modulate the development of hypertrophy. Serial measurements of ventricular wall thickness in the first two decades of life, and probably until the fourth decade of life are advisable in patients suspected of having HCM.
肥厚型心肌病（HCM）与编码主要肌节蛋白的基因突变有关，但肥大的机制尚不清楚。由于肥大可能直到青春期才会出现，对生理生长刺激的反应改变可能调节肥大过程。
本研究探讨HCM患者年龄与左心室（LV）壁厚度变化之间的关系。
对43例有明确心电图和超声心动图证据的HCM患者进行研究，至少相隔两年（平均间隔5.5±3.0年）进行系列二维和M型超声心动图检查。将左心室腔尺寸、室间隔和后壁厚度以及左心室质量指数与年龄和性别匹配的对照组数据进行比较。
在10至20岁的HCM患者（n = 9）中，研究期间室间隔厚度从15.9±6.2mm增加到19.3±2.1mm（p < 0.01）。这种增加（3.4±2.5mm）大大超过了对照组在10至20岁之间观察到的室间隔厚度变化（0.8±0.3mm，p < 0.01）。后壁厚度从9.8±2.1mm增加到11.5±3.5mm，增加幅度较小（p = 0.07）。在21 - 40岁的HCM患者（n = 11）中，研究期间室间隔厚度也从16.0±2.2mm增加到17.8±3.0mm（p < 0.05），而对照组室间隔厚度无变化。相比之下，年龄大于40岁的患者（n = 23）在研究期间室间隔和后壁厚度均无显著变化。10至20岁年龄组的左心室质量指数从128±24g/m²增加到164±20g/m²（p = 0.01），但在老年组未观察到这种增加。
在HCM患者的青春期和成年早期，左心室肥大是进行性的，尤其是在室间隔。由于在身体生长停止后进行性肥大可能继续，心肌对生理生长调节因子的异常反应不太可能是肥大的主要刺激因素。基因 - 基因相互作用、血流动力学负荷变化或环境因素可能调节肥大的发展。对于疑似患有HCM的患者，建议在生命的前二十年，可能直到第四十年进行心室壁厚度的系列测量。
