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β-Blocker Therapy Failures in Symptomatic Probands with Genotyped Long-QT Syndrome

机译：基因型长QT综合征的先证者的β受体阻滞剂治疗失败

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摘要

β-Blocker therapy is one of the principal therapies for congenital long-QT syndrome (LQTS). However, breakthrough cardiac events occur while being treated with β-blockers. We sought to determine the frequency of and clinical correlates underlying β-blocker therapy failures in genotyped, symptomatic LQTS probands. The medical records were analyzed only for genotyped LQTS probands who presented with a LQTS-attributable clinical event and were receiving β-blocker therapy. The study cohort comprised 28 such patients: 18 KCNQ1/KVLQT1(LQT1), 7 KCNH2/HERG (LQT2), and 3 SCN5A (LQT3). The prescribed β-blocker was atenolol (12), propranolol (10), metoprolol (4), and nadolol (2). β-Blocker therapy failure was defined as breakthrough cardiac events including syncope, aborted cardiac arrest (ACA), appropriate implantable cardioverter-defibrillator (ICD) therapy, or sudden death occurring while on β-blocker therapy. During a median follow-up of 46 months, 7/28 (25%) LQTS probands experienced a total of 15 breakthrough cardiac events. Their initial presentation was ACA (3), bradycardia during infancy (2), and syncope (2). The underlying genotype was KVLQT1 (6) and HERG (1). Two breakthroughs were attributed to noncompliance. Of the 13 breakthroughs occurring while compliant, 10 occurred with atenolol and 3 with propranolol (p = 0.03). In this study cohort, one-fourth of genotyped LQTS probands failed β-blocker therapy. Treatment with atenolol, young age at diagnosis, initial presentation with ACA, KVLQT1 genotype, and noncompliance may be important factors underlying β-blocker therapy failures.
机译：β-Blocker疗法是先天性长QT综合征（LQTS）的主要疗法之一。但是，用β受体阻滞剂治疗时会发生突破性心脏事件。我们试图确定基因型，有症状的LQTS先证者中β受体阻滞剂治疗失败的频率及其临床相关性。仅对出现LQTS归因的临床事件并接受β受体阻滞剂治疗的基因型LQTS先证者分析病历。该研究队列包括28位此类患者：18位KCNQ1 / KVLQT1（LQT1），7位KCNH2 / HERG（LQT2）和3位SCN5A（LQT3）。规定的β受体阻滞剂为阿替洛尔（12），普萘洛尔（10），美托洛尔（4）和纳多洛尔（2）。 β-阻滞剂治疗失败的定义为突破性心脏事件，包括晕厥，心脏骤停（ACA）流产，适当的植入式心脏复律除颤器（ICD）治疗或在进行β阻滞剂治疗时突然死亡。在46个月的中位随访期间，7/28（25％）LQTS先证者经历了15次突破性心脏事件。他们最初的表现是ACA（3），婴儿期心动过缓（2）和晕厥（2）。潜在的基因型是KVLQT1（6）和HERG（1）。违规有两个突破。在顺应性发生的13次突破中，阿替洛尔发生10次，普萘洛尔发生3次（p = 0.03）。在本研究队列中，基因型LQTS先证者的四分之一未能通过β受体阻滞剂治疗。使用阿替洛尔治疗，诊断时年龄较小，初次出现ACA，KVLQT1基因型和不依从性可能是导致β受体阻滞剂治疗失败的重要因素。

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  • 来源
    《Pediatric Cardiology》 |2004年第5期|459-465|共7页
  • 作者

    R. Chatrath; C. M. Bell; M. J. Ackerman;

  • 作者单位

    Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology Mayo Clinic College of Medicine;

    Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology Mayo Clinic College of Medicine;

    Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology Mayo Clinic College of MedicineDepartment of Internal Medicine/Division of Cardiovascular Diseases Mayo Clinic College of MedicineDepartment of Molecular Pharmacology and Experimental Therapeutics Mayo Clinic College of MedicineLong QT Syndrome Clinic Guggenheim 501 Mayo Clinic;

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  • 正文语种 eng
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  • 关键词

    Long QT syndrome; Drugs; Risk factors; Beta-blockers;

    机译：长QT综合征;药物;风险因素;β受体阻滞剂;

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