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Item does not contain fulltext, BACKGROUND: Since their implementation in clinical practice, remote home monitoring systems (HM) have undoubtedly become an added value in patients with implantable devices for cardiac rhythm management. The aim of this study was to investigate the impact of HM on clinical management and outcome in patients with channelopathies and other arrhythmogenic diseases who received an implantable cardioverter defibrillator (ICD). METHODS: Fifty-four patients (age 6 months--74 years) were followed by means of HM in our ICD clinic. Alerts and/or device-related clinical events were analysed in all patients and subsequent clinical decisions were made if indicated. RESULTS: During an average observation time of 27 months, 46 alerts were received from 32 different patients. Five patients (9%) received appropriate therapies for life-threatening arrhythmias and four patients (8%) inappropriate therapies because ofT wave oversensing or supraventricular tachycardias. Three patients had alerts due to electrical noise (two on the atrial, one on the ventricular channel). Overall, 18 alerts (39%) required a modification of the pharmacological therapy or the programming of the device. Mean anticipation of clinical visits based on the alerts was 92.6 +/- 56 days (median 97, interquartile range 50-150). CONCLUSION: HM substantially improves the clinical management of patients with cardiac arrhythmogenic disease by early recognition of device-related inappropriate therapies and subsequent anticipation of treatment adaptation.