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先天性耳聋、Q-T间期延长、发作性扭转型室性心动过速、心室颤动所致的昏厥称为Jervell-Lange-Nielsen综合征,无先天性耳聋者称Romano-Ward综合征。常规治疗是应用β-阻滞剂心得安等药物控制症状。本文报道我院自1984年10月以来收治的3例Romano-Ward综合征患者,因用心得安等治疗不能控制昏厥发作,经安置VVI永久心脏起搏器联合心得安治疗后能满意控制发作。病例介绍 [例1]赵××,女,44岁,棉纺厂工人,1984年10月18日入院。患者有发作性昏厥20年,近2年发作频繁加剧,常于行走、高声谈笑、体位改变、劳累、情绪激动后发生昏厥。昏厥发作前原有频繁室性早搏,Q-T间期延长,发作时心电图呈现扭转型室性心动过速。长期服用心得安,每日30mg,仍然不能控制发作。患者的母亲、姨母有类似病史,已病故,其2个女儿也有类似症状。体检:发育正常,血压130/80,发作时神志不清,心率55～90次,心律不齐,无病理性杂音。
Congenital deafness, Q-T prolongation, episodic torsades-type ventricular tachycardia, ventricular fibrillation caused by syncope is called Jervell-Lange-Nielsen syndrome, congenital deafness were called Romano-Ward syndrome. Routine treatment is the application of β-blockers and other drug control symptoms. This article reports from our hospital since October 1984 admitted to 3 cases of Romano-Ward syndrome patients, due to anxiety and other treatment can not control the fainting episodes, after the placement of VVI permanent pacemaker combined with propranolol treatment can be satisfactorily controlled seizures. Case description [Example 1] Zhao × ×, female, 44 years old, cotton mill workers, admitted to hospital on October 18, 1984. Patients have episodes of syncope 20 years, the past two years increased seizures, often walking, talking loudly, changing posture, exertion, emotional excitement after syncope. Fetal seizures before frequent premature ventricular contractions, Q-T interval prolongation, electrocardiogram showed torsades de pointes ventricular tachycardia. Long-term use of safe, daily 30mg, still can not control the attack. The patient’s mother, aunt has a similar history, has died, the two daughters have similar symptoms. Physical examination: normal development, blood pressure 130/80, episodes of confusion, heart rate 55 to 90 times, arrhythmia, no pathological noise.