Comparison of Usefulness of Cardiac Resynchronization Therapy in Patients with Type 1 Myotonic Dystrophy with –vs- without Left Bundle Branch Block

Abstract Patients with type 1 myotonic dystrophy (MD1) show reduced LV systolic function in the presence of left bundle branch block (LBBB) due to electromechanical dyssynchrony. Our prospective study tracked a cohort of 64 MD1 patients that demonstrated a high burden of atrial and ventricular arrhythmias and conduction delays. Of these patients, 12 (19%) patients had LBBB, which was associated with reduced left ventricular systolic function. Eight of these patients received cardiac resynchronization therapy (CRT) devices resulting in reduction of median QRS complex duration from 173 ms to 166 ms (p = 0.04), and improvement in median left ventricular ejection fraction from 37% to 46% (p = 0.007). In conclusion, CRT device therapy is both feasible and effective in treating advanced cardiac disease in this vulnerable group of patients by improving left ventricular function.