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

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\u200a=\u200a35), dilated cardiomyopathy (n\u200a=\u200a17), hypertrophic cardiomyopathy (n\u200a=\u200a15), Brugada syndrome (n\u200a=\u200a14), idiopathic ventricular fibrillation (n\u200a=\u200a5), left ventricular noncompaction (n\u200a=\u200a4), long-QT syndrome (n\u200a=\u200a4) and short-QT syndrome (n\u200a=\u200a2), who were 18-35 years old at the time of ICD implantation. During a mean follow-up of 72.6\u200a\ub1\u200a53.3 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\u200a=\u200a9). A threshold for ICD therapy less than 300\u200ams was associated with a borderline significant lower probability of inappropriate ICD interventions (hazard ratio\u200a=\u200a0.2; 95% confidence interval 0.02-1.2; P\u200a=\u200a0.07), whereas underweight status was an independent predictor of device-related complications (hazard ratio\u200a=\u200a5.4; 95% confidence interval 1.5-19.4; P\u200a=\u200a0.01). 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