普萘洛尔对21例长QT综合征的临床疗效观察

观察β 阻断剂普萘洛尔对长QT综合征 (LQTS)患者的疗效。选择适于服用β 阻断剂又能规律服药的 2 1例患者进行用药前后的对比研究。结果 :患者年龄 2 4 .4± 12 .4岁 ,首次发病年龄 11.2± 11.1岁。跟踪观察的时间为 15 .9± 32 .8个月。服用普萘洛尔的剂量为 6 3± 4 4mg/d ,按体重计算为 1.3± 0 .7mg/kg ,到达 2± 4mg/kg的患者有 3例 (14 .3% )。部分首次发病时间较早的高危患者 ,由于本身的基础心率较慢 ,普萘洛尔很难加到所需剂量 ,故服用普萘洛尔的同时 ,有 8例行左心交感神经切除术、1例安装起搏器、1例埋置埋藏式心脏复律除颤器 ,2例服用美西律。普萘洛尔使QT间期 (QTc)从用药前的 5 70± 70ms降低到 5 0 0± 6 0ms(P <0 .0 0 0 1) ;同时心率并未降低 (分别为 6 5± 11次 /分和 6 5± 10次 /分 ,P =NS)。用药前有晕厥发作的 19例用药后发作次数显著减少 ,平均晕厥发作次数由 1.90± 1.72次 /年降至 0 .17± 0 .4 0次 /年 ,P =0 .0 2。单纯普萘洛尔与联合治疗亚组之间无显著性差别。结论 :普萘洛尔能有效降低大多数LQTS患者的晕厥次数 ,缩短复极时程 ,但对那些首次发病时间较早的高危患者 ,可能需联合应用其它治疗方式。

The Clinical Effect of Propranolol on Long QT Syndrome in 21 Patients

To observe the effects of propranolol,a β-blocker, on the long QT syndrome (LQTS), 21 long QT syndrome(LQTS) patients who were taken propranolol regularly were selected to study the effects of propranolol on the LQTS patients. Results: The mean age for patients were 24.4±12.4 years and age distribution for the first attack is 11.2±11.1. The dosage of propranolol was 63±44 mg/day or 1.3±0.7 mg/kg. During administration of propranolol , there were 8cases underwent left cardiac sympathetic denervation (LCSD), 1 case implanted with pacemaker, 1 case implanted with implantable cardioverter defibrillator(ICD), and 2 cases taken mexiletine. The QTc decreased from 570±70 to 500±60 ms ( P <0.0001) after 15.9±32.8 months follow-up. At the same time, the rest heart rate was not changed significantly. The syncope episodes in 19 of 21 patients who had syncope attack before treatment decreased from 1.90±1.72 to 0.17±0.40 times per year after long-term administration of propranolol, P =0.02. Conclusion:Propranolol could decrease the syncope episodes in most LQTS patients and shorten repolarization. However, for some patients, combined LCSD,pacemaker, or ICD may be needed.