Usefulness of an Implantable Loop Recorder to Detect Clinically Relevant Arrhythmias in Patients with Advanced Fabry Cardiomyopathy

• Published in: The American journal of cardiology Vol. 118; no. 2; pp. 264 - 274
• Main Authors: Weidemann, Frank, MD, Maier, Sebastian K.G., MD, Störk, Stefan, MD, Brunner, Thomas, Liu, Dan, MD, Hu, Kai, MD, Seydelmann, Nora, MD, Schneider, Andreas, Becher, Jan, MD, Canan-Kühl, Sima, MD, Blaschke, Daniela, MD, Bijnens, Bart, PhD, Ertl, Georg, MD, Wanner, Christoph, MD, Nordbeck, Peter, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 15.07.2016; Elsevier Limited
• Subjects: Adult; Aged; Anticoagulants - therapeutic use; Arrhythmias, Cardiac - diagnosis; Arrhythmias, Cardiac - etiology; Arrhythmias, Cardiac - therapy; Atrial Fibrillation - diagnosis; Atrial Fibrillation - drug therapy; Atrial Fibrillation - etiology; Bradycardia - diagnosis; Bradycardia - etiology; Bradycardia - therapy; Cardiac arrhythmia; Cardiac Pacing, Artificial; Cardiomyopathy; Cardiomyopathy, Hypertrophic - complications; Cardiomyopathy, Hypertrophic - therapy; Cardiovascular; Cardiovascular disease; Death, Sudden, Cardiac - prevention & control; Defibrillators, Implantable; Diagnostic Errors; Electrocardiography; Electrocardiography, Ambulatory; Fabry Disease - complications; Fabry Disease - therapy; Female; Flow velocity; Heart Arrest - diagnosis; Heart Arrest - etiology; Heart Arrest - therapy; Heart rate; Humans; Intubation; Male; Middle Aged; Pacemaker, Artificial; Patients; Prospective Studies; Prostheses and Implants; Surveillance; Tachycardia, Ventricular - diagnosis; Tachycardia, Ventricular - etiology; Tachycardia, Ventricular - therapy; Telemedicine; Ultrasonic imaging; Fabry disease; Holter electrocardiogram; implantable loop recorder; arrhythmia
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2016.04.033

Abstract Patients with genetic cardiomyopathy that involves myocardial hypertrophy often develop clinically relevant arrhythmias that increase the risk of sudden death. Consequently, guidelines for medical device therapy were established for hypertrophic cardiomyopathy, but not for conditions with only anecdotal evidence of arrhythmias, like Fabry cardiomyopathy. Patients with Fabry cardiomyopathy progressively develop myocardial fibrosis, and sudden cardiac death occurs regularly. Because 24-hour Holter electrocardiograms (ECG) might not detect clinically important arrhythmias, we tested an implanted loop recorder for continuous heart rhythm surveillance and determined its impact on therapy. This prospective study included 16 patients (12 male) with advanced Fabry cardiomyopathy, relevant hypertrophy, and replacement fibrosis in loco typico . No patients previously exhibited clinically-relevant arrhythmias on Holter-ECGs. Patients received an implantable loop recorder, and were prospectively followed with telemedicine for a median of 1.2 years (range 0.3–2.0 years). The primary endpoint was a clinically meaningful event, which required a therapy change, captured with the loop recorder. Patients submitted data regularly (14±11 times per month). During follow-up, 21 events were detected (including 4 asystole, i.e. ECG pauses ≥3 s) and 7 bradycardia events; 5 episodes of intermittent atrial fibrillation (>3 min) and 5 episodes of ventricular tachycardia (3 sustained, 2 non-sustained). Subsequently, as defined in the primary end-point 15 events leaded to a change of therapy. These patients required therapy with a pacemaker or cardioverter-defibrillator implantation and/or anticoagulation therapy for atrial fibrillation. In conclusion, clinically relevant arrhythmias that require further device and/or medical therapy are often missed with Holter-ECGs in patients with advanced stage Fabry cardiomyopathy, but they can be detected by telemonitoring with an implantable loop recorder.