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Sterile Pericarditis in Aachener Minipigs As a Model for Atrial Myopathy and Atrial Fibrillation
Published on: September 24, 2021
[When do you implant a pacemaker in myotonic dystrophy?]
Dominique Babuty1, Bénédicte Lallemand, Valérie Laurent
1Université François-Rabelais, CHU de Tours, hôpital Trousseau, 37044 Tours, France. d.babuty@chu-tours.fr
Abstract:
Myotonic dystrophy is the most frequent adult form of hereditary muscular dystrophy caused by a mutation on the DMPK gene. Myotonic dystrophy leads to multiple systemic complications related to weakness, respiratory failure, cardiac arrhythmias and cardiac conduction disturbances. Age of death is earlier in myotonic dystrophy patients than in general population with a high frequency of sudden death. Several mechanisms are involved in sudden death: atrio-ventricular block, severe ventricular arrhythmias or non-cardiac mechanism. The high degree of atrio-ventricular block is a well-recognized indication of pacemaker implantation but the prophylactic implantation of pacemaker should be considered to prevent sudden death in asymptomatic myotonic dystrophy patients. A careful clinical evaluation needs to be done for the identification of patients at high risk of sudden death. The resting ECG and SA ECG are non-invasive tools useful to select the patients who need an electrophysiologic study. In presence of prolonged HV interval more than or equal to 70 ms one can discuss the implantation of a prophylactic pacemaker. The choice of an implantable cardiac defibrillator is preferred in presence of spontaneous ventricular tachycardia or an alteration of the left ventricular ejection fraction.
Insights
Myotonic dystrophy patients face increased sudden death risk from cardiac issues. Prophylactic pacemakers are recommended for high-risk individuals, guided by ECG and electrophysiologic studies.
Area of Science:
- Cardiology
- Genetics
- Neurology
Context:
- Myotonic dystrophy is the most common adult hereditary muscular dystrophy, linked to DMPK gene mutations.
- It causes systemic complications including cardiac arrhythmias and conduction disturbances.
- Patients experience reduced lifespan and high sudden death rates.
Purpose:
- To identify risk factors for sudden death in myotonic dystrophy patients.
- To evaluate the utility of non-invasive tests for risk stratification.
- To guide prophylactic pacemaker and defibrillator implantation decisions.
Summary:
- Sudden death in myotonic dystrophy arises from atrio-ventricular block, ventricular arrhythmias, or non-cardiac factors.
- High-grade atrio-ventricular block warrants pacemaker implantation.
- Prophylactic pacemaker implantation should be considered in asymptomatic patients with a prolonged HV interval (≥70 ms) on electrophysiologic study.
- Implantable cardiac defibrillators are indicated for spontaneous ventricular tachycardia or reduced left ventricular ejection fraction.
Impact:
- This research aids in early identification of myotonic dystrophy patients at high risk of sudden cardiac death.
- It supports the prophylactic implantation of pacemakers and defibrillators, potentially improving survival rates.
- Clinical evaluation, resting ECG, and SA ECG are crucial non-invasive tools for patient selection.
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