title: Indications and outcome of implantable cardioverter-defibrillators for primary and secondary prophylaxis in patients with noncompaction cardiomyopathy.
abstract: BACKGROUND
  Noncompaction cardiomyopathy (NCCM) is a rare, primary cardiomyopathy, with initial presentation of heart failure, emboli, or arrhythmias, including sudden cardiac death. Implantable cardioverter-defibrillators (ICDs) are frequently used for primary and secondary prevention in different cardiomyopathy patients, but data about ICD in NCCM are scarce. The aim of this study was, therefore, to investigate ICD indications and outcomes in NCCM patients.
  
  
  RESULTS
  We collected prospective data from our NCCM cohort (n = 77 pts, mean age: 40 ± 14 years). ICD was implanted in 44 (57%) patients with NCCM according to the current ICD guidelines for nonischemic cardiomyopathies: in 12 for secondary prevention (7 × ventricular fibrillation, 5 × sustained ventricular tachycardia [VT]) and in 32 patients for primary prevention (heart failure/severe LV dysfunction). During a mean follow-up of 33 ± 24 months, 8 patients presented with appropriate ICD shocks due to sustained VT after median 6.1 [1-16] months. This included 4 of 32 (13%) patients in the primary prevention group and 4 of 12 (33%) in the secondary prevention group (P = 0.04). 9 patients presented with inappropriate ICD therapy: 6 (19%) in the primary and 3 (25%) in the secondary prevention group, at a median follow-up of 4 (2-23) months.
  
  
  CONCLUSIONS
  In our cohort of NCCM patients, an ICD was frequently implanted for primary or secondary prevention of sudden cardiac death. At follow-up, frequent appropriate ICD therapy was observed in both groups, supporting the application of current ICD guidelines for primary and secondary prevention of sudden cardiac death in NCCM.
authors.affiliation: Thoraxcenter, Erasmus MC, Rotterdam, The Netherlands. k.caliskan@erasmusmc.nl
authors.forename: Kadir
authors.initials: K
authors.lastname: Caliskan
authors.forename: Tamas
authors.initials: T
authors.lastname: Szili-Torok
authors.forename: Dominic A M J
authors.initials: DA
authors.lastname: Theuns
authors.forename: Attila
authors.initials: A
authors.lastname: Kardos
authors.forename: Marcel L
authors.initials: ML
authors.lastname: Geleijnse
authors.forename: Aggie H M M
authors.initials: AH
authors.lastname: Balk
authors.forename: Ron T
authors.initials: RT
authors.lastname: van Domburg
authors.forename: Luc
authors.initials: L
authors.lastname: Jordaens
authors.forename: Maarten L
authors.initials: ML
authors.lastname: Simoons
mesh_terms: D000328:Adult; D009202:Cardiomyopathies / Q000503:physiopathology / Q000628:therapy; D015331:Cohort Studies; D017147:Defibrillators, Implantable / Q000639:trends; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011322:Primary Prevention / Q000379:methods*; D011446:Prospective Studies; D055502:Secondary Prevention / Q000379:methods*; D016896:Treatment Outcome; D055815:Young Adult