title: ICD therapy for primary prevention of sudden cardiac death after Mustard repair for d-transposition of the great arteries.
abstract: BACKGROUND
  Years and decades after the Mustard atrial switch repair for d-Transposition of the great arteries, there is a subset of patients at increased risk for sudden cardiac death due to ventricular tachyarrhythmias. To date, little is known about indications, efficacy and benefit of internal cardioverter defibrillator (ICD) therapy indication in these patients.
  
  
  METHODS
  To characterize the Mustard patients already treated with an ICD, we conducted a single center case control study (n = 41). The charts of all patients after Mustard procedure at our institution were systematically reviewed for history, echocardiographic findings, arrhythmias, hemodynamics and medication as well as ICD discharges and complications.
  
  
  RESULTS
  Significant differences between ICD (n = 12) and non-ICD patients (n = 29) were found regarding the stage of heart failure, need for heart failure medication, QRS duration and left ventricular diameter and performance. Inappropriate ICD discharges due to rapidly conducted atrial reentrant tachycardia and sensing failure were frequently observed. In 17 % of our Mustard patients with an ICD, infection of the implantation site required surgical revision. All ICD patients were of male gender.
  
  
  CONCLUSIONS
  Patients after ICD implantation represent a subgroup among our Mustard patients with a more advanced state of cardiac disease. Not only systemic right ventricular diameter and performance but also subpulmonary left ventricular performance was an important factor predicting the long-term course of these patients. Prevention of inappropriate discharges requires decent device programming, close monitoring of lead integrity and treatment of atrial tachycardias.
authors.affiliation: Department of Pediatric Cardiology and Intensive Care Medicine, University Hospital, Georg-August-University, Robert-Koch-Str 40, 37075, Göttingen, Germany.
authors.forename: David
authors.initials: D
authors.lastname: Backhoff
authors.forename: Matthias
authors.initials: M
authors.lastname: Müller
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Ruschewski
authors.forename: Thomas
authors.initials: T
authors.lastname: Paul
authors.forename: Ulrich
authors.initials: U
authors.lastname: Krause
mesh_terms: D000328:Adult; D006328:Cardiac Catheterization / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D016022:Case-Control Studies; D016009:Chi-Square Distribution; D016757:Death, Sudden, Cardiac / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011322:Primary Prevention / Q000379:methods*; D012189:Retrospective Studies; D018570:Risk Assessment; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D017180:Tachycardia, Ventricular / Q000150:complications / Q000175:diagnosis; D013997:Time Factors; D014188:Transposition of Great Vessels / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome