Loperfido F, Fiorilli R, Digaetano A, Di Gennaro M, Santarelli P, Bellocci F, Coppola E, Zecchi P
Br Heart J. 1982 Jun;47(6):588-95. doi: 10.1136/hrt.47.6.588.
The electrocardiographic and vectorcardiographic (Frank system) features of the first degree relatives of subjects with documented familial hypertrophic cardiomyopathy were analysed. A total of nine affected members and 29 relatives were examined in four families. THe subjects were considered to be affected when the septal to free posterior wall thickness ratio exceeded 1.3 at M-mode echocardiography. Four relatives had asymmetric septal hypertrophy. Among 25 relatives without evidence of asymmetric septal hypertrophy, two over 20 years and 10 under 20 years of age showed increased voltage of QRS anterior forces (Qz amplitude greater than 0.80 mV) on the orthogonal electrocardiogram. The vectorcardiographic data of the relatives under 20 years of age without evidence of asymmetric septal hypertrophy (18 subjects) were compared with those of 38 normal control subjects of comparable age range. The young relatives without disproportionate septal hypertrophy had significantly greater Qz amplitude and Q/Rz ratio than the normal control subjects. In contrast, the echocardiographic data were not significantly different. We suggest that the electrocardiographic finding of abnormal anterior forces in one or more first degree relatives of subjects with documented hypertrophic cardiomyopathy may constitute a valuable aid in ascertaining the genetic transmission of the disease and in recognising affected members without echocardiographic evidence of hypertrophic cardiomyopathy.
对有记录的家族性肥厚型心肌病患者的一级亲属的心电图和向量心电图(Frank 系统)特征进行了分析。在四个家族中,共检查了 9 名患病成员和 29 名亲属。当 M 型超声心动图显示室间隔与后壁游离厚度比值超过 1.3 时,这些受试者被认为患病。4 名亲属有不对称性室间隔肥厚。在 25 名无不对称性室间隔肥厚证据的亲属中,2 名 20 岁以上和 10 名 20 岁以下的亲属在正交心电图上显示 QRS 前向力电压升高(Qz 振幅大于 0.80 mV)。将 20 岁以下无不对称性室间隔肥厚证据的亲属(18 名受试者)的向量心电图数据与 38 名年龄范围相当的正常对照受试者的数据进行比较。无不成比例室间隔肥厚的年轻亲属的 Qz 振幅和 Q/Rz 比值明显高于正常对照受试者。相比之下,超声心动图数据无显著差异。我们认为,在有记录的肥厚型心肌病患者的一个或多个一级亲属中,心电图发现前向力异常可能有助于确定该疾病的遗传传递,并有助于识别无肥厚型心肌病超声心动图证据的患病成员。