Outcomes of cardiac pacing in patients with myotonic dystrophy type 1: A single specialist centre study

Abstract

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Cardiac conduction disease and sudden cardiac death is known to be associated with Myotonic Dystrophy type 1 (MD). Patients often require device implantation to prevent sudden cardiac death. It is not known how frequently device implantation occurs and how quickly conduction disease progresses. Objective To define the rate and type of device implantation, rate of pacing and outcome in MD patients managed in a single specialist centre. Method Retrospective analysis of pacing and outcome data on consecutive MD patients managed at Specialist Centre in the UK between 2011 and 2021. Results 24/119 MD patients were implanted with a cardiac device. Male:Female 13:11. At implant, patients had a mean age of 56 (95% CI 52 to 59), mean PR interval 217 ms (95% CI 203 to 230), mean QRSd 123ms (95% CI 109 to 137). Devices implanted included 16 DDDR, 4 CRT-D, 1 CRT-P, 1- ICD and 2 VVI pacemakers. 10 (42%) died during follow up of 10 years. 12 out of 19 (63%) with an atrial pacing lead required a mean increase in atrial pacing of 15% (95% CI 5 to 25) during follow up. 12 out of 24 (50%) of patients required mean increase of 13% (95% CI 5 to 22) in Ventricular pacing. 7 (88%) patients who died had evidence of progressive conduction disease prior to death. Conclusion In our cohort of myotonic dystrophy patients 20% required device implantation. There was a higher incidence of progressive atrial rather than ventricular pacing, despite the known concern about progressive AV block. Despite pacing, mortality remained high and was more frequent in patients with progressive conduction disease. Increases in pacing demand could be early indicator of disease progression.