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我院收治祖、孙三代患有家族性Q-T间期延长综合征共3例.治疗、随访5至19年.并对其家系成员24人作了直接病史询问、体格检查及心电图检查.报道如下.例1:先证者女.39岁.阵发性胸闷、心悸.发作性昏厥30年、本次昏厥再次发作而于1990年9月住院.作检;T37℃.BP18/12kPa.两肺听诊正常.心律极不规则,心界不扩大.昏厥发作时可闻及短阵心动过速.腹平软,肝脾未触及.神经系统检查正常.血、尿常规、血流、抗“O”、血脂测定及血钾、钠、钙、氯、镁、血清蛋白电泳均在正常范围,24小时尿钾4克.胸片示心肺正常.心脏二维超声检查正常.脑电图示正常.心电图示窦性心律82次/分+异位心律.Q-T间期为0.43秒.QTuc0.65秒.T、u波融合.u波高于T波.发作时出现阵发室速伴偶发短阵空颤.住院期间昏厥发作长达50分钟.经用利多卡因100mg静注未能控制.后用心得安5mg加10%葡萄糖液100ml静滴,阵发性室速明显减少,室颤消失,
The hospital received ancestral, Sun three generations with familial QT prolongation syndrome in a total of 3. Treatment, followed up for 5 to 19 years and its members of the family of 24 people made a direct history inquiry, physical examination and electrocardiogram.Report as follows Example 1: proband female .39 years old Paroxysmal chest tightness, palpitations Seizures of fainting 30 years, the fainting episodes again in September 1990 for examination; T37 ℃ .BP18 / 12kPa. Both lungs Auscultation normal heart rhythm is very irregular heart does not expand faint seizures can be heard and bradycardia tachycardia abdomen soft, liver and spleen not touched normal nervous system blood, urine, blood flow, anti-O Serum lipids and serum potassium, sodium, calcium, chloride, magnesium, serum protein electrophoresis are in the normal range, 24-hour urinary potassium 4 grams .Cardiac cardiomyopathy normal heart two-dimensional ultrasound was normal. ECG showed sinus rhythm 82 beats / min + ectopic rhythm .QT interval of 0.43 seconds .QTuc0.65 seconds .T, u wave fusion.u wave higher than the T wave.A paroxysmal onset of paroxysmal ventricular burst with occasional burst Vibrillation. During hospitalization, fainting episodes for up to 50 minutes. Lidocaine 100mg intravenous infusion failed to control .After the use of the safety of 5mg plus 10% glucose solution 100ml intravenous drip, burst VT significantly reduced ventricular fibrillation disappeared,