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为研究我国长QT综合征 (LQTS)患者的整体发病和治疗情况 ,选择按照 1993年Schwartz等提出的LQTS诊断标准确诊为本病的家系 76个。对先证者及其家族成员进行 6或 12导联ECG同步记录 ,对先证者的临床情况进行综合分析。结果 :先症者发病年龄 17.2± 14 .8岁 ,在 2 0岁以前发病的占 5 9.2 % ;以女性居多 ;发病症状有晕厥、黑、心悸、胸闷及其它如抽搐、胸背痛、头晕等 ;诱发因素有情绪紧张或激动、劳累、运动或体力劳动等 ;患者的QTc值为 0 .5 6± 0 .0 9s。LQTS患者的ECG上T波多变 ,QT间期可出现暂时正常化。在 76个LQTS先证者中 ,同时伴聋哑 1例 ,预激综合征 1例 ,心肌炎 2例 ,束支阻滞 2例 ,一过性房室阻滞 1例 ,高血压病 2例。根据ECG特点预测LQTS患者的基因型 :LQT1占 (31.6 % ) ,LQT2占 (5 3.9% ) ,LQT3占 (3.9% ) ,其余 (10 .5 % )心电图特征不明显 ,无法预测。多数患者服用 β 阻断剂类药物有效 ;在药物效果不好的患者中 ,有 4例安装起搏器 ,1例应用埋藏式心脏复律除颤器 (ICD) ,15例进行左心交感神经切除术 (LCSD) ,其中多数继续服用 β 阻断剂。结论 :我国的LQTS发病情况和临床表现与国外报道基本一致 ;根据ECG特点对LQTS患者进行的基因分型预测结果显示 ,我国的LQTS患者可能以LQT2为主。β 阻
To study the overall incidence and treatment of patients with long QT syndrome (LQTS) in our country, 76 pedigrees diagnosed as LQTS were selected according to the 1993 Schwartz et al. Criteria. The proband and its family members 6 or 12 lead ECG synchronization records, the proband’s clinical situation a comprehensive analysis. Results: The first symptom onset age of 17.2 ± 14.8 years old, before the age of 20 accounted for 52.2%; mostly female; incidence of symptoms of syncope, blackheads, palpitations, chest tightness and other such as convulsions, chest and back pain, Dizziness, etc .; inducing factors such as emotional stress or excitement, fatigue, exercise or manual labor; patients QTc value of 0.56 ± 0. 09s. T wave changes in patients with LQTS ECG, QT interval may be temporary normalization. Among the 76 LQTS probands, 1 was accompanied by deafness, 1 was pre-excitation syndrome, 2 was myocarditis, 2 was bundle branch block, 1 was transient ventricular block, and 2 was hypertension. The genotypes of LQTS patients were predicted according to the characteristics of ECG: LQT1 accounted for 31.6%, LQT2 accounted for 3.9% and LQT3 accounted for 3.9%, while the remaining ECGs were not obvious and could not be predicted. Most patients taking beta blockers are effective; in patients with poor drug efficacy, 4 patients with pacemaker, 1 case with implanted cardioverter-defibrillator (ICD), 15 cases of left ventricular sympathetic nerve Resection (LCSD), most of whom continue taking beta blockers. Conclusion: The incidence and clinical manifestations of LQTS in China are basically consistent with those in foreign countries. Genotyping of LQTS patients based on ECG characteristics shows that LQTS patients in our country may be mainly LQT2. β resistance