Clinical features and management of congenital long QT syndrome: a report on 54 patients from a national registry
Clinical features and management of congenital long QT syndrome: a report on 54 patients from a national registry
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DOI：
10.1007/s00380-003-0733-9
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发表时间：
2003
影响因子：
1.5
通讯作者：
Cui-lan Li;D. Hu;X. Qin;Yun-tian Li;Pingjing Li;Wen-ling Liu;Zhi-ming Li;Lei Li;Le-Xin Wang
中科院分区：
文献类型：
--
作者：
Cui-lan Li;D. Hu;X. Qin;Yun-tian Li;Pingjing Li;Wen-ling Liu;Zhi-ming Li;Lei Li;Le-Xin Wang
为了评估中国先天性长QT综合征（LQTS）的临床特征和治疗，我们从我们新建立的国家登记处收集了54例LQTS患者（14男40女）的临床资料。所有患者均有症状，晕厥是最常见的症状。首次出现症状的平均年龄为17.9±15.6岁（0.5 ~ 62岁）；55.6%的人在20岁前出现首发症状。这些症状最常见的诱因是体育锻炼或情绪压力。校正后QT间期平均为0.55±0.08 s。根据心电图标准，LQT1患者14例（25.9%），LQT2患者28例（51.9%），LQT3患者2例（3.7%）。30例（55.6%）患者在入组时接受Β-blockers治疗，其中心得安是最常用的药物，平均日剂量为57.5±39.1 mg。4例患者行左心交感神经切除术。平均随访24.9±13.2个月后，接受抗肾上腺素能治疗的患者中有3.1%（1/32）和未接受抗肾上腺素能治疗的患者中有9.1%（2/22）死于心源性猝死（P≪0.05）。我们得出结论，LQT2可能是这些患者中最常见的亚型。抗肾上腺素能治疗未得到充分利用，迫切需要对医生和患者进行有关疾病性质及其最佳抗肾上腺素能治疗的教育。
To assess the clinical features and the management of congenital long QT syndrome (LQTS) in China, we collected the clinical data of 54 LQTS patients (14 males and 40 females) from our newly established national registry. All patients were symptomatic, with syncope being the most common symptom. The average age when the first symptoms occurred was 17.9 ± 15.6 (range, 0.5–62) years; 55.6% of them had the first symptoms before the age of 20. The most common triggers of the symptoms were physical exercise or emotional stress. The average corrected QT interval was 0.55 ± 0.08 s. Using ECG criteria, there were 14 (25.9%) LQT1 patients, 28 (51.9%) LQT2, and 2 (3.7%) LQT3. Thirty (55.6%) patients were treated with Β-blockers at the time of enrollment, with propranolol being the most commonly used drug, with an average daily dose of 57.5 ± 39.1 mg. Four patients underwent left cardiac sympathectomy. After an average follow-up of 24.9 ± 13.2 months, 3.1% (1/32) of patients with antiadrenergic therapy and 9.1% (2/22) without antiadrengergic therapy died of sudden cardiac death (P≪ 0.05). We concluded that LQT2 might be the most common subtype in these patients. Antiadrenergic treatment was underused, raising the urgent need for educating both physicians and patients on the nature of the disease and its optimal antiadrenergic therapy.