PROGRESSION OF LEFT VENTRICULAR HYPERTROPHY IN HYPERTROPHIC CARDIOMYOPATHY

确定左心室大小和分布是否 肥大在出生时就已经确定， 心肌病，或可能在第一年大幅增加 39名有家族史或形态学证据的儿童， 用超声心动图对肥厚型心肌病进行了连续研究。 患者最初接受调查的年龄为4-15岁（平均11岁）， 最近9-20岁（平均16岁）。 随访2.5-6.8年（平均4年），17例 患者表现出明显增加的幅度和程度的预先存在的 左心室肥厚，和其他5人证明从一个演变， 形态正常的心脏出现实质性肥大。 在 这22例患者，左室壁厚度增加， 引人注目（6- 23毫米; 101 ± 62%变化），大大超过了 预期会因正常生长而发生（13 ± 10%; p 小于0.001），与症状恶化无关，或 继发于主动脉下阻塞。 这些结果表明，左心室肥厚可能 在患有以下疾病的患者中自发发生或显著进展 儿童期肥厚性心肌病，当身体生长 可观 由于超声心动图检查可能在 儿童肥厚型心肌病发病前的形态学特征 发展，超声心动图筛查患者的年轻亲属 肥厚型心肌病不能总是明确排除这种疾病。

To determine whether magnitude and distribution of left ventricular hypertrophy is largely established at birth in patients with hypertrophic cardiomyopathy, or may substantially increase during the first years of life, 39 children with family history or morphologic evidence of hypertrophic cardiomyopathy were studied serially with echocardiography. Patients were initially investigated at ages 4-15 years (mean 11) and most recently at 9-20 years (mean 16). Over 2.5-6.8 year (mean 4) follow-up, 17 patients showed marked increase in magnitude and extent of pre-existent left ventricular hypertrophy, and 5 others demonstrated evolution from a morphologically normal appearing heart to substantial hypertrophy. In these 22 patients, increases in left ventricular wall thickness were striking (6-23mm; 101 plus/minus 62% change), greatly exceeded that expected to occur as a consequence of normal growth (13 plus/minus 10%; p less than 0.001) and were not associated with symptomatic deterioration or secondary to subaortic obstruction. These findings demonstrated that left ventricular hypertrophy may spontaneously develop or progress substantially in patients with hypertrophic cardiomyopathy during childhood when body growth is considerable. Since echocardiographic studies may be normal during childhood before the morphologic features of hypertrophic cardiomyopathy develop, echocardiographic screening of youthful relatives of patients with hypertrophic cardiomyopathy cannot always definitively exclude this disease.