title: Use of dofetilide in adult patients with atrial arrhythmias and congenital heart disease: A PACES collaborative study.
abstract: 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.
authors.affiliation: Department of Pediatrics, Cleveland Clinic Children's, Cleveland, Ohio.
authors.forename: Iqbal
authors.initials: I
authors.lastname: El-Assaad
authors.affiliation: Harrington Heart & Vascular Institute, University Hospitals Case Medical Center & Case Western Reserve University, Cleveland, Ohio.
authors.forename: Sadeer G
authors.initials: SG
authors.lastname: Al-Kindi
authors.affiliation: Minneapolis Heart Institute, Abbott Northwestern Hospital,, Minneapolis, Minnesota.
authors.forename: JoEllyn
authors.initials: J
authors.lastname: Abraham
authors.affiliation: Children's Heart Centre, BC Children's Hospital, University of British Columbia, Vancouver, Canada.
authors.forename: Shubhayan
authors.initials: S
authors.lastname: Sanatani
authors.affiliation: Michigan Congenital Heart Center, University of Michigan, Ann Arbor, Michigan.
authors.forename: David J
authors.initials: DJ
authors.lastname: Bradley
authors.affiliation: Cardiovascular Center, University of Michigan, Ann Arbor, Michigan.
authors.forename: Colby
authors.initials: C
authors.lastname: Halsey
authors.affiliation: Division of Pediatric Cardiology, University of Iowa Children's Hospital, Iowa City, Iowa.
authors.forename: Ian H
authors.initials: IH
authors.lastname: Law
authors.affiliation: Division of Pediatric Cardiology, Oregon Health and Science University, Portland, Oregon.
authors.forename: Seshadri
authors.initials: S
authors.lastname: Balaji
authors.affiliation: Division of Pediatric Cardiology, Advocate Children's Hospital, Oak Lawn, Illinois.
authors.forename: Ira
authors.initials: I
authors.lastname: Shetty
authors.affiliation: Division of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio. Electronic address: azizp@ccf.org.
authors.forename: Peter F
authors.initials: PF
authors.lastname: Aziz
mesh_terms: D000328:Adult; D000889:Anti-Arrhythmia Agents / Q000008:administration & dosage / Q000009:adverse effects; D001281:Atrial Fibrillation / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D016903:Drug Monitoring / Q000379:methods / Q000706:statistics & numerical data; D004562:Electrocardiography / Q000379:methods; D005260:Female; D006330:Heart Defects, Congenital / Q000145:classification / Q000150:complications / Q000453:epidemiology; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D008875:Middle Aged; D010043:Outcome and Process Assessment, Health Care; D010627:Phenethylamines / Q000008:administration & dosage / Q000009:adverse effects; D012189:Retrospective Studies; D013449:Sulfonamides / Q000008:administration & dosage / Q000009:adverse effects; D013611:Tachycardia, Atrioventricular Nodal Reentry / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D014481:United States / Q000453:epidemiology; D028761:Withholding Treatment / Q000706:statistics & numerical data
chemical_list: D000889:Anti-Arrhythmia Agents; D010627:Phenethylamines; D013449:Sulfonamides; C063533:dofetilide
keywords: Atrial fibrillation; Congenital heart disease; Dofetilide; Intra-atrial reentrant tachycardia; Torsades de pointes; Ventricular tachycardia