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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Article info
Publication history
Published online: September 09, 2019
Received in revised form:
August 21,
2019
Received:
June 15,
2019
Footnotes
Funding: University Hospital Foundation, University of Alberta (Edmonton, Canada) Hospital (Grant number: 41769).
Identification
Copyright
© 2019 Elsevier Inc. All rights reserved.
