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本文报道同一家族6例小心脏综合征患者,其中姐妹二人先后猝死,死亡年龄均在30岁左右。小心脏综合征的主要诊断际准是:(1)X 线胸片示心影狭小,心胸比率成人在0.40以下,儿童在0.42以下;(2)心电图Ⅰ导联R 波电压较低,Ⅱ、Ⅲ导联有相对高大的R 波;(3)超声心动图在Ⅱ区测量,心脏直径≤60mm。此综合征常伴有神经循环衰弱症及直立性调节障碍。本家族患者遗传学检查符合常染色体显性遗传特征。
This article reports the same family of 6 patients with cardiac syndrome, in which the sisters two have sudden death, the death age is about 30 years old. Cardiac syndrome is the main diagnostic criteria for small: (1) X-ray showed narrow heart, cardiomyopsis rate of adults below 0.40, children below 0.42; (2) lead R wave ECG lower voltage, Ⅱ, Ⅲ lead has a relatively high R wave; (3) echocardiography measured in Ⅱ area, the heart diameter ≤ 60mm. This syndrome is often accompanied by neurocytic failure and erectile dysfunction. This family of patients with genetic tests consistent with autosomal dominant genetic features.