title: The ICD for primary prevention in patients with inherited cardiac diseases: indications, use, and outcome: a comparison with secondary prevention.
abstract: BACKGROUND
  Indications for prophylactic implantable cardioverter-defibrillator (ICD) therapy in patients with inherited cardiac diseases stem from observational studies and are uncertain. This study evaluates the efficacy and harm rate of ICD implantations for primary prevention compared with secondary prevention in inherited cardiac diseases.
  
  
  RESULTS
  Between January 1, 1993, and April 1, 2011, 354 patients with inherited cardiac diseases were treated with ICDs. Incidence rates of appropriate shocks in primary prevention patients with arrhythmogenic right ventricular cardiomyopathy and hypertrophic cardiomyopathy were 4.2 to 6.7/100 patient-years, whereas the risk for appropriate shocks in primary prevention patients with Brugada syndrome, long QT syndrome, or carrying the DPP6 haplotype approached zero. Conversely, in secondary prevention patients there was a considerably higher incidence rate of appropriate shocks. None of the indications for primary prevention were associated with appropriate shock therapy. One hundred twenty-three patients (35%) experienced ICD-related adverse events.
  
  
  CONCLUSIONS
  For Brugada syndrome, long QT syndrome, and DPP6 the efficacy of an ICD for primary prevention contrasts with the amount of harm, and factors that formed the indication for ICD implantation do not relate to the occurrence of appropriate shocks. The higher appropriate discharge rates in arrhythmogenic right ventricular cardiomyopathy and hypertrophic cardiomyopathy compared with primary electric diseases might result from a more advanced risk stratification scheme in these inherited cardiomyopathies.
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Louise R A
authors.initials: LR
authors.lastname: Olde Nordkamp
authors.forename: Arthur A M
authors.initials: AA
authors.lastname: Wilde
authors.forename: Jan G P
authors.initials: JG
authors.lastname: Tijssen
authors.forename: Reinoud E
authors.initials: RE
authors.lastname: Knops
authors.forename: Pascal F H M
authors.initials: PF
authors.lastname: van Dessel
authors.forename: Joris R
authors.initials: JR
authors.lastname: de Groot
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D053840:Brugada Syndrome / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D002135:Calcium-Binding Proteins / Q000235:genetics; D024741:Cardiomyopathy, Hypertrophic, Familial / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D004152:Dipeptidyl-Peptidases and Tripeptidyl-Peptidases / Q000235:genetics; D004554:Electric Countershock / Q000009:adverse effects / Q000295:instrumentation*; D004868:Equipment Failure; D005260:Female; D020022:Genetic Predisposition to Disease; D006239:Haplotypes; D006331:Heart Diseases / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D040941:Heredity; D006801:Humans; D007223:Infant; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics; D018579:Patient Selection; D010641:Phenotype; D015221:Potassium Channels / Q000235:genetics; D011322:Primary Prevention / Q000379:methods*; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D055502:Secondary Prevention; D013997:Time Factors; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D055815:Young Adult; D000099211:Phospholamban
chemical_list: D002135:Calcium-Binding Proteins; D004152:Dipeptidyl-Peptidases and Tripeptidyl-Peptidases; D009419:Nerve Tissue Proteins; D015221:Potassium Channels; D000099211:Phospholamban; C503535:DPP6 protein, human