Usefulness of an Implantable Loop Recorder to Detect Clinically Relevant Arrhythmias in Patients With Advanced Fabry Cardiomyopathy.
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 (ECGs) 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 men) 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 to 2.0 years). The primary end point 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 seconds) and 7 bradycardia events; 5 episodes of intermittent atrial fibrillation (>3 minutes) and 5 episodes of ventricular tachycardia (3 sustained and 2 nonsustained). 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.
Department of Cardiology, Innere Klinik II, Katharinen-Hospital, Unna, Germany; Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany. Electronic address: f.weidemann@katharinen-hospital.de.
Frank
F
Weidemann
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany; Department of Cardiology, II. Medizinische Klinik, Klinikum Straubing GmbH, Straubing, Germany.
Sebastian K G
SK
Maier
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Stefan
S
Störk
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Thomas
T
Brunner
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Dan
D
Liu
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Kai
K
Hu
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Nora
N
Seydelmann
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Andreas
A
Schneider
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany; Department of Cardiology, II. Medizinische Klinik, Klinikum Straubing GmbH, Straubing, Germany.
Jan
J
Becher
Medical Division, Nephrology and Cardiology, Charité, Campus Virchow Klinikum, Berlin, Germany.
Sima
S
Canan-Kühl
Medical Division, Nephrology and Cardiology, Charité, Campus Virchow Klinikum, Berlin, Germany.
Daniela
D
Blaschke
Catalan Institution for Research and Advanced Studies, Universitat Pompeu Fabra, Barcelona, Spain.
Bart
B
Bijnens
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Georg
G
Ertl
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Christoph
C
Wanner
Department of Cardiology, Comprehensive Heart Failure Center, Würzburg, Germany.
Peter
P
Nordbeck
D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D001281:Atrial Fibrillation / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D001919:Bradycardia / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D002304:Cardiac Pacing, Artificial; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000628:therapy; D016757:Death, Sudden, Cardiac / Q000517:prevention & control; D017147:Defibrillators, Implantable; D003951:Diagnostic Errors; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D000795:Fabry Disease / Q000150:complications / Q000628:therapy*; D005260:Female; D006323:Heart Arrest / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D010138:Pacemaker, Artificial; D011446:Prospective Studies; D019736:Prostheses and Implants; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D017216:Telemedicine
D000925:Anticoagulants