12
Table 3. Clinical scenarios for the management of arrhythmias
in DMD, BMD, and recessive LGMD2
Clinical scenario
DMD
1. A 27-year-old man with DMD is found to have progressive left ventricular
dysfunction (most recent LVEF 29%) despite maximally tolerated GDMT for >1
year. ECG shows sinus rhythm, PR interval 140 ms, QRS duration 100 ms, and a
prominent R wave in lead V
1
. He requires a power wheelchair and full assistance
for daily needs. Mechanical ventilation via tracheostomy is required, and nutrition
is provided through enterostomy due to recurrent aspiration pneumonitis.
Hospitalization for heart failure has not been observed.
BMD
2. A 31-year-old man with BMD is found to have stable left ventricular dysfunction
(most recent LVEF 32%) despite maximally tolerated GDMT for >1 year. He has
exertional dyspnea and fatigue corresponding to NYHA function class III. He
ambulates most of the day, uses a wheelchair for long distance mobility, and has a
service animal to assist with activities of daily living. ECG shows sinus rhythm with
LBBB with PR interval 160 ms, and QRS duration 150 ms.
Duchenne, Becker, and Recessive Limb-girdle
Muscular Dystrophies
