title: Implantable cardioverter defibrillator therapy in young patients with cardiomyopathies and channelopathies: a single Italian centre experience.
abstract: OBJECTIVE
  This study was designed to prospectively evaluate the risk-benefit ratio of implantable cardioverter defibrillator (ICD) therapy in young patients with cardiomyopathies and channelopathies.
  
  
  RESULTS
  The study population included 96 consecutive patients [68 men, median age 27 (22-32) years] with cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (n = 35), dilated cardiomyopathy (n = 17), hypertrophic cardiomyopathy (n = 15), Brugada syndrome (n = 14), idiopathic ventricular fibrillation (n = 5), left ventricular noncompaction (n = 4), long-QT syndrome (n = 4) and short-QT syndrome (n = 2), who were 18-35 years old at the time of ICD implantation. During a mean follow-up of 72.6 ± 53.3 months, one patient with end-stage hypertrophic cardiomyopathy died because of acute heart failure, and 11 patients underwent orthotopic heart transplantation. Twenty patients (20.8%) had a total of 38 appropriate ICD interventions (4%/year), and 26 patients (27.1%) experienced a total of 49 adverse ICD-related events (5.4%/year), including 23 inappropriate ICD interventions occurring in nine patients (9.4%) and 26 device-related complications requiring surgical revision occurring in 20 patients (20.8%). Lead failure/fracture requiring lead extraction was the most common complication (n = 9). A threshold for ICD therapy less than 300 ms was associated with a borderline significant lower probability of inappropriate ICD interventions (hazard ratio = 0.2; 95% confidence interval 0.02-1.2; P = 0.07), whereas underweight status was an independent predictor of device-related complications (hazard ratio = 5.4; 95% confidence interval 1.5-19.4; P = 0.01).
  
  
  CONCLUSIONS
  In young patients with cardiomyopathies and channelopathies, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. However, because ICD-related adverse events are common, the risk/benefit ratio should be carefully assessed when considering ICD implantation in young people.
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Migliore
authors.forename: Maria
authors.initials: M
authors.lastname: Silvano
authors.forename: Alessandro
authors.initials: A
authors.lastname: Zorzi
authors.forename: Emanuele
authors.initials: E
authors.lastname: Bertaglia
authors.forename: Mariachiara
authors.initials: M
authors.lastname: Siciliano
authors.forename: Loira
authors.initials: L
authors.lastname: Leoni
authors.forename: Pietro
authors.initials: P
authors.lastname: De Franceschi
authors.forename: Sabino
authors.initials: S
authors.lastname: Iliceto
authors.forename: Domenico
authors.initials: D
authors.lastname: Corrado
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000145:classification / Q000401:mortality / Q000628:therapy*; D009202:Cardiomyopathies / Q000145:classification / Q000150:complications*; D053447:Channelopathies / Q000150:complications*; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D006329:Heart Conduction System / Q000503:physiopathology; D006333:Heart Failure / Q000401:mortality / Q000628:therapy*; D006801:Humans; D007558:Italy; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult