14
Duchenne, Becker, and Recessive Limb-girdle
Muscular Dystrophies
Table 3. Clinical scenarios for the management of arrhythmias
in DMD, BMD, and recessive LGMD2
Clinical scenario
LGMD2
3. A 38-year-old woman with LGMD2 reports several episodes of recurrent, unprovoked
syncope, some resulting in minor injury. ECG shows sinus rhythm and right bundle
branch block (RBBB). Echocardiogram shows normal LVEF with biatrial dilation.
The 30-day event recorder shows sinus pauses with ventricular asystole (2.5–4.0
seconds) with overall heart rate range 60–110 beats per minute (bpm). She ambulates
with assistance from her husband and the use of a rolling walker.
(cont'd)
