Implantable loop recorder monitoring in inherited cardiomyopathies: yield of clinically relevant arrhythmias

Abstract Background Implantable loop recorders (ILRs) are a minimally invasive tool for the diagnosis of arrhythmias in patients with unexplained syncope or asymptomatic arrhythmias. Their use in patients with inherited arrhythmia syndromes has been recommended in the recent 2022 ESC Guidelines (1). However, the clinical utility of ILRs in patients with inherited cardiomyopathies remains unexplored. Purpose The purpose of this study was to evaluate the yield of ILR monitoring in detecting clinically relevant events in a single cohort of patients with inherited cardiomyopathies. Methods We prospectively collected demographic, clinical, and follow-up data from patients with inherited cardiomyopathies (Arrhythmogenic Cardiomyopathy, ACM; Hypertrophic Cardiomyopathy, HCM; Dilated Cardiomyopathy, DCM; Left Ventricular Non-Compaction, LVNC) who were implanted with an ILR and were followed with telemedicine. The primary endpoint was a clinically significant event recorded with the ILR that required an update to an implantable cardioverter defibrillator (ICD). Results The study cohort included 53 consecutive patients (55% males; mean age at ILR implantation 35±14 years) with inherited cardiomyopathies: 33/53 (62%) with ACM, 9/53 (17%) with HCM, 7/53 (13%) LVNC, 4/33 (8%) DCM. None of the patients implanted had previously experienced life-threatening arrhythmias. During a median follow-up of 2.6 years (IQR 1.4-3.7 years) after ILR implantation, clinically relevant arrhythmic events were documented in 9/53 (17%) of patients. Specifically, sustained or non-sustained ventricular tachycardia episodes were detected in 8 patients (5 with ACM, 2 with LVNC, 1 with LMNA-related DCM), whereas one patient with HCM showed symptomatic pauses > 3 seconds due to sick sinus syndrome. In all cases, the detection of such episodes led to the upgrade to ICD implantation. Conclusions ILR monitoring of patients with inherited cardiomyopathies identified clinically relevant events in almost one-fifth of patients, suggesting that the use of ILR may substantially improve the management of these patients. Figure 1. Representative example of clinically actionable event detected by implantable loop recorder (ILR) in a patient with inherited cardiomyopathy. The patient, a 19-year-old male with a definitive diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) and a pathogenic PKP2 truncating variant, presented with palpitations and vertigo during physical exertion one year after the implantation of an ILR. ILR monitoring documented a rapid tachycardia with a mean frequency of 250 bpm lasting 3 minutes, prompting an indication for an implantable cardioverter defibrillator (ICD).Figure 1.