Comparison of Usefulness of Cardiac Resynchronization Therapy in Patients With Type 1 Myotonic Dystrophy With Versus
Anish Nikhanj1, Soori Sivakumaran1, Haran Yogasundaram1
1Division of Cardiology, Department of Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Canada; Mazankowski Alberta Heart Institute, University of Alberta, Edmonton, Canada.
Insights
Cardiac resynchronization therapy improves heart function in type 1 myotonic dystrophy patients with left bundle branch block. This treatment effectively reduces QRS duration and enhances left ventricular ejection fraction in this patient group.
Area of Science:
- Cardiology
- Neuromuscular Disorders
- Medical Devices
Background:
- Type 1 myotonic dystrophy (DM1) patients often exhibit cardiac abnormalities, including arrhythmias and conduction delays.
- Left bundle branch block (LBBB) in DM1 is associated with impaired left ventricular (LV) systolic function due to electromechanical dyssynchrony.
Purpose of the Study:
- To evaluate the feasibility and effectiveness of cardiac resynchronization therapy (CRT) in DM1 patients with LBBB and reduced LV systolic function.
Main Methods:
- Prospective study of 64 DM1 patients.
- Analysis of 12 patients (19%) with LBBB.
- Eight patients received CRT devices; outcomes including QRS duration and LV ejection fraction (LVEF) were assessed.
Main Results:
- CRT implantation in DM1 patients with LBBB was feasible.
- Median QRS duration decreased from 173 ms to 166 ms (p=0.04).
- Median LVEF improved significantly from 37% to 46% (p=0.007).
Conclusions:
- Cardiac resynchronization therapy is a viable and effective treatment for advanced cardiac disease in DM1 patients with LBBB.
- CRT improves LV systolic function and reduces electromechanical dyssynchrony in this vulnerable population.
Abstract:
Patients with type 1 myotonic dystrophy show reduced left ventricular systolic function in the presence of left bundle branch block due to electromechanical dys-synchrony. Our prospective study tracked a cohort of 64 type 1 myotonic dystrophy patients that demonstrated a high burden of atrial and ventricular arrhythmias and conduction delays. Of these patients, 12 (19%) patients had left bundle branch block, which was associated with reduced left ventricular systolic function. Eight of these patients received cardiac resynchronization therapy devices resulting in reduction of median QRS complex duration from 173 to 166 ms (p = 0.04), and improvement in median left ventricular ejection fraction from 37% to 46% (p = 0.007). In conclusion, cardiac resynchronization therapy device therapy is both feasible and effective in treating advanced cardiac disease in this vulnerable group of patients by improving left ventricular function.
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