8
Bradycardias, conduction disorders, and use of pacing
or CRT in Duchenne, Becker, and recessive limb-girdle
muscular dystrophies
COR LOE Recommendations
1 B-NR 1. In patients with DMD, BMD, or LGMD2, with
documented symptomatic bradycardia due to any degree of
sinus node dysfunction or AV block, permanent pacemaker
(PPM) implantation is indicated if concordant with the
patient's goals of care and clinical status.
1 B-NR 2. In patients with DMD, BMD, or LGMD2 and third-degree
or advanced second-degree AV block at any anatomical level,
with or without symptoms, PPM implantation is indicated if
concordant with the patient's goals of care and clinical status.
2a B-NR 3. In patients with DMD, BMD, or LGMD2 with an LVEF
≤35% despite guideline-directed medical therapy (GDMT)
with a combination of sinus rhythm, le bundle branch
block (LBBB), QRS duration ≥150 ms, and New York Heart
Association (NYHA) class II to class IV symptoms, or in
those with suspected RV pacing-induced CM or anticipated
RV pacing ≥40%, CRT is reasonable if concordant with the
patient's goals of care and clinical status.
Duchenne, Becker, and Recessive Limb-girdle
Muscular Dystrophies