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Implantable cardioverter defibrillator therapy in young patients with cardiomyopathies and channelopathies: a single Italian centre experience

Migliore, Federico; Silvano, Maria; Zorzi, Alessandro; Bertaglia, Emanuele; Siciliano, Mariachiara; Leoni, Loira; De Franceschi, Pietro; Iliceto, Sabino; Corrado, Domenico

Journal of Cardiovascular Medicine: July 2016 - Volume 17 - Issue 7 - p 485–493
doi: 10.2459/JCM.0000000000000395
Implantable defibrillators

Aims This study was designed to prospectively evaluate the risk–benefit ratio of implantable cardioverter defibrillator (ICD) therapy in young patients with cardiomyopathies and channelopathies.

Methods and 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).

Conclusion 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.

Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy

Correspondence to Federico Migliore, MD, PhD, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Via N. Giustiniani 2, 35121 Padova, Italy Tel: +39 049 8212332; fax: +39 049 8212309; e-mail:

Received 6 January, 2016

Revised 25 February, 2016

Accepted 20 March, 2016

© 2016 Italian Federation of Cardiology. All rights reserved.