26
Figure 3. Rhythm management and CIED implantation in
patients with EDMD or LGMD1B
Patient with EDMD/LGMD1B
a
YES
YES
YES
YES
YES
LVEF <45% and
nonsustained VT
Mild to
moderate
conduction
disorder
d
and/or
VA suspected
Symptomatic
sinus node
dysfunction or
bradycardia
<40 bpm
NO
NO
NO
NO
NO
NO
NO
NO
NO
NO
Cardiac evaluation
b
(1)
Implantable
cardiac monitor
(2a)
ICD concordant with
patient goals of care
and clinical status
ICD concordant with
patient goals of care
and clinical status
Symptoms of
conduction
disorder
d
/bradycardia
or arrhythmias
and nondiagnostic
noninvasive
testing
e
ICD concordant
with patient
goals of care and
clinical status
ICD concordant
with patient
goals of care and
clinical status
Emery-Dreifuss and Limb-girdle Type 1B
Muscular Dystrophy
LBBB, RBBB,
or AF/AFL with
ventricular rate
<50 bpm
PR ≥230 ms, 2nd or
3rd degree AV block,
or HV ≥70 ms
