Invasive electrophysiology study guided loop recorder implantation in myotonic dystrophy type 1: a prospective single-center study

FUNDING ACKNOWLEDGEMENTS: Type of funding sources: None. INTRODUCTION: In one third of the patients with myotonic dystrophy type 1 (DMT1) the cause of death is of cardiac origin. The most common cardiac manifestations are arrhythmias and conduction disturbances. PURPOSE: Due to its extreme long monitoring properties, implantable loop recorders (ILRs) are highly suitable to detect rare cardiac arrhythmias. To date, there are only limited data using ILRs in DMT1. We aimed to evaluate the role of the invasive electrophysiology study (EPS)-guided loop recorder implantation in patients with DMT. METHODS: In our prospective study 18 genetically confirmed DMT1 patients underwent non-invasive cardiac examination including transthoracic echocardiography, 24-hour Holter monitoring and surface ECG. In addition, EPS was performed in all cases to evaluate the risk of sudden cardiac death and infra-Hisian conduction properties. Based on the performed non-invasive diagnostic evaluation and EPS findings, patients were divided into low, intermediate and high-risk groups. For patients with normal investigation results and normal HV interval (<55 ms) only annual cardiac follow-up visits were planned (low-risk group). Patients with increased PR interval (>240 ms), QRS duration (>120 ms), or slightly prolonged HV interval (55-70 ms) underwent implantable loop recorder (ILR) implantation (intermediate-risk group). Permanent pacemaker (PM) or implantable cardiac defibrillator (ICD) was implanted in patients with 2nd/3rd degree atrioventricular block (AVB), significantly prolonged HV interval (>100 ms) or if sustained ventricular tachyarrhythmia could be induced during EPS. All patients were followed for at least 3 years. RESULTS: According to our study criteria, 8 patients could be categorized as "intermediate risk", 7 of them received implantable loop recorder (ILR). 1 ILR had to be removed 3 weeks after implantation due to skin infarction. During three-years of follow-up, 2 patients had many asymptomatic atrial high-rate episodes, but no 2nd or 3rd degree AVB, significant asystole or malignant ventricular arrhythmia were detected. CONCLUSIONS: Our single-center prospective study examined the EPS-guided ILR implantation for the risk stratification of DMT1 patients for the first time. A more extensive, multicenter study is warranted to define optimal management of DMT1 patients with slightly impaired infrahisian conduction.