El-Assaad Iqbal, Al-Kindi Sadeer G, Abraham JoEllyn, Sanatani Shubhayan, Bradley David J, Halsey Colby, Law Ian H, Balaji Seshadri, Shetty Ira, Aziz Peter F
Department of Pediatrics, Cleveland Clinic Children's, Cleveland, Ohio.
Harrington Heart & Vascular Institute, University Hospitals Case Medical Center & Case Western Reserve University, Cleveland, Ohio.
Heart Rhythm. 2016 Oct;13(10):2034-9. doi: 10.1016/j.hrthm.2016.07.016. Epub 2016 Jul 17.
Arrhythmia management has become the major treatment challenge in adult patients with congenital heart disease (ACHD).
We sought to investigate the utility and safety profile of dofetilide for atrial arrhythmias in ACHD.
A retrospective chart review was performed. We included patients (age ≥18 years) with congenital heart disease who had atrial fibrillation (AF) or intra-atrial reentrant tachycardia treated with dofetilide.
We identified 64 patients with a mean age at initiation of 42 ± 14 years. ACHD type included single ventricle (n = 19, 30%), transposition of the great arteries (n = 14, 22%), atrial septal defect (n = 9, 14%), tetralogy of Fallot (n = 8, 12%), atrioventricular canal defect (n = 5, 8%), mitral/aortic stenosis (n = 7, 11%), and other (n = 2, 3%). Thirty-five (55%) had atrial fibrillation, and 29 (45%) had intra-atrial reentrant tachycardia. A total of 3 (4.7%) patients had major inpatient adverse events: torsades de pointes (n = 1, 1.5%), ventricular tachycardia (n = 1, 1.5%), and corrected QT prolongation requiring discontinuation (n = 1, 1.5%). Dofetilide was discontinued in 1 patient because of sinus node dysfunction, and another patient discontinued therapy before discharge because of persistent arrhythmia. Of the patients who were discharged on dofetilide (n = 59, 92%), 40 (68%) had adequate rhythm control and 19 (32%) had partial rhythm control. After a median follow-up of 3 years, 29 (49%) patients remained on dofetilide and 2 (3%) patients died. Reasons for discontinuation included waning effect (n = 16, 57%), side effects (n = 5, 18%), noncompliance (n = 2, 7%), successful ablation (n = 3, 11%), high cost (n = 1, 3.5%), and unknown (n = 1, 3.5%).
Dofetilide remains a viable antiarrhythmic drug option in this challenging population. At 3 years, 49% remained on dofetilide. Close monitoring of renal function, concomitant medications, and corrected QT interval is required.
心律失常的管理已成为成年先天性心脏病（ACHD）患者的主要治疗挑战。
我们旨在研究多非利特用于ACHD患者房性心律失常的有效性和安全性。
进行了一项回顾性病历审查。我们纳入了年龄≥18岁、患有先天性心脏病且接受多非利特治疗心房颤动（AF）或房内折返性心动过速的患者。
我们确定了64例患者，开始治疗时的平均年龄为42±14岁。ACHD类型包括单心室（n = 19，30%）、大动脉转位（n = 14，22%）、房间隔缺损（n = 9，14%）、法洛四联症（n = 8，12%）、房室管缺损（n = 5，8%）、二尖瓣/主动脉瓣狭窄（n = 7，11%）以及其他（n = 2，3%）。35例（55%）患有心房颤动，29例（45%）患有房内折返性心动过速。共有3例（4.7%）患者发生主要住院不良事件：尖端扭转型室速（n = 1，1.5%）、室性心动过速（n = 1，1.5%）以及需要停药的QT间期延长（n = 1，1.5%）。1例患者因窦房结功能障碍停用多非利特，另1例患者在出院前因持续性心律失常停药。在出院时服用多非利特的患者中（n = 59，92%），40例（68%）实现了充分的心律控制，19例（32%）实现了部分心律控制。中位随访3年后，29例（49%）患者仍在服用多非利特，2例（3%）患者死亡。停药原因包括疗效减弱（n = 16，57%）、副作用（n = 5，18%）、不依从（n = 2，7%）、成功消融（n = 3，11%）、费用高昂（n = 1，3.5%）以及不明原因（n = 1，3.5%）。
在这一具有挑战性的人群中，多非利特仍然是一种可行的抗心律失常药物选择。3年后，49%的患者仍在服用多非利特。需要密切监测肾功能、合并用药情况以及QT间期。
