title: Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life-Threatening Arrhythmias.
abstract: BACKGROUND
  Patients with dilated cardiomyopathy (DCM) may present with ventricular arrhythmias early in the disease course, unrelated to the severity of left ventricular dysfunction. These patients may be classified as having an arrhythmogenic DCM (AR-DCM). We investigated the phenotype and natural history of patients with AR-DCM.
  
  
  RESULTS
  Two hundred eighty-five patients with a recent diagnosis of DCM (median duration of the disease 1 month, range 0 to 7 months) and who had Holter monitoring at baseline were comprehensively evaluated and followed for 107 months (range 29 to 170 months). AR-DCM was defined by the presence of ≥1 of the following: unexplained syncope, rapid nonsustained ventricular tachycardia (≥5 beats, ≥150 bpm), ≥1000 premature ventricular contractions/24 hours, and ≥50 ventricular couplets/24 hours, in the absence of overt heart failure. The primary end points were sudden cardiac death (SCD), sustained ventricular tachycardia (SVT), or ventricular fibrillation (VF). The secondary end points were death from congestive heart failure or heart transplantation. Of the 285 patients, 109 (38.2%) met criteria for AR-DCM phenotype. AR-DCM subjects had a higher incidence of SCD/SVT/VF compared with non-AR-DCM patients (30.3% vs 17.6%, P=0.022), with no difference in the secondary end points. A family history of SCD/SVT/VF and the AR-DCM phenotype were statistically significant and cumulative predictors of SCD/SVT/VF.
  
  
  CONCLUSIONS
  One-third of DCM patients may have an arrhythmogenic phenotype associated with increased risk of arrhythmias during follow-up. A family history of ventricular arrhythmias in DCM predicts a poor prognosis and increased risk of SCD.
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics, University of Colorado, Aurora, CO (A.S., S.L.G., D.S., E.E.S., M.G.T., L.M.) Cardiovascular Department "Ospedali Riuniti", Hospital and University of Trieste, Italy (A.S., G.S., M.M., G.B., F.B.).
authors.forename: Anita
authors.initials: A
authors.lastname: Spezzacatene
authors.affiliation: Cardiovascular Department "Ospedali Riuniti", Hospital and University of Trieste, Italy (A.S., G.S., M.M., G.B., F.B.).
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Sinagra
authors.affiliation: Cardiovascular Department "Ospedali Riuniti", Hospital and University of Trieste, Italy (A.S., G.S., M.M., G.B., F.B.).
authors.forename: Marco
authors.initials: M
authors.lastname: Merlo
authors.affiliation: Cardiovascular Department "Ospedali Riuniti", Hospital and University of Trieste, Italy (A.S., G.S., M.M., G.B., F.B.) Cardiovascular Center, Trieste, Italy (G.B., A.D.L.).
authors.forename: Giulia
authors.initials: G
authors.lastname: Barbati
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics, University of Colorado, Aurora, CO (A.S., S.L.G., D.S., E.E.S., M.G.T., L.M.).
authors.forename: Sharon L
authors.initials: SL
authors.lastname: Graw
authors.affiliation: Cardiovascular Department "Ospedali Riuniti", Hospital and University of Trieste, Italy (A.S., G.S., M.M., G.B., F.B.).
authors.forename: Francesca
authors.initials: F
authors.lastname: Brun
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics, University of Colorado, Aurora, CO (A.S., S.L.G., D.S., E.E.S., M.G.T., L.M.).
authors.forename: Dobromir
authors.initials: D
authors.lastname: Slavov
authors.affiliation: Cardiovascular Center, Trieste, Italy (G.B., A.D.L.).
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Lenarda
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics, University of Colorado, Aurora, CO (A.S., S.L.G., D.S., E.E.S., M.G.T., L.M.).
authors.forename: Ernesto E
authors.initials: EE
authors.lastname: Salcedo
authors.affiliation: Cincinnati Children's Hospital, Cincinnati, OH (J.A.T.).
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
authors.affiliation: Beth Israel Deaconess Medical Center, Boston, MA (J.E.S.).
authors.forename: Jeffrey E
authors.initials: JE
authors.lastname: Saffitz
authors.affiliation: University of Arizona Medical Center, Tucson, AZ (F.I.M.).
authors.forename: Frank I
authors.initials: FI
authors.lastname: Marcus
authors.affiliation: University of Rochester Medical Center, Rochester, NY (W.Z.).
authors.forename: Wojciech
authors.initials: W
authors.lastname: Zareba
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics, University of Colorado, Aurora, CO (A.S., S.L.G., D.S., E.E.S., M.G.T., L.M.).
authors.forename: Matthew R G
authors.initials: MR
authors.lastname: Taylor
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics, University of Colorado, Aurora, CO (A.S., S.L.G., D.S., E.E.S., M.G.T., L.M.).
authors.forename: Luisa
authors.initials: L
authors.lastname: Mestroni
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D002423:Cause of Death; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000517:prevention & control; D018450:Disease Progression; D018572:Disease-Free Survival; D015716:Electrocardiography, Ambulatory; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D015995:Prevalence; D012042:Registries; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors
keywords: arrhythmia; cardiomyopathy; prognosis; sudden death