Résumé :
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Communication n° 566 Introduction : Patients with lamin A/C gene (LMNA) mutations have frequent cardiac involvement characterized by conduction defects and arrhythmias, heart failure and high frequency of sudden death (SD). While SD is not adequately prevented by pacemaker implantation, we hypothesized that SD may be consecutive to ventricular arrhythmia (VA) and therefore could be adequately prevented by internal cardioverter defibrillator (ICD) implantation. Objectives : to evaluate the benefit of the preventive ICD implantation in this specific genetically proven cardiac disease. Methods: Twenty one consecutive patients carrying LMNA mutations, showing various phenotypes, referred for pacemaker implantation were included in this prospective observational study and ICD was implanted. No other criteria were mandatory. Data have been carefully reviewed by two cardiologists blinded to any information. Results: ICD implantation was uncomplicated in all patients (15 M / 7 F, 41.1Æ12.8 years). After a mean 31.8Æ19.4 months follow-up, 12 patients had documented VA (ventricular tachycardia or fibrillation), including 6 with ventricular fibrillation. VA was treated by pacing overdrive in 1 patient, and 8 patients had ICD delivered shock; VA was not sustained in the 3 remaining. Interestingly, VA occurred in 5 patients previously asymptomatic. Nine patients had preserved LVEF; it was not predictable by any factor, including the presence of spontaneous or induced VA, supra-ventricular arrhythmia, or medical treatment. Conclusion: Ventricular arrhythmias are common and may be responsible of sudden death in LMNA mutated patients, even at early stage of the disease where LVEF is preserved. Prophylactic ICD implantation is effective in primary prevention of sudden death in these patients and should be considered in this setting.
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