40
Table 7. NMDs, use of pacemakers and ICDs, shared
decision-making principles, and end-of-life
care decisions
NMD
Frequency of
pacemaker implant Typical pacemaker indications
DMD Infrequent Symptomatic bradycardia, heart block
BMD,
LGMD2
Infrequent Symptomatic bradycardia, heart block
DM1 Frequent Primary prevention due to heart block risk
Symptomatic bradycardia
DM2 Moderate Primary prevention due to heart block risk
Symptomatic bradycardia
EDMD,
LGMD1B
Moderate Primary prevention due to heart block risk
FSHD Rare Normal general population indications
Mitochondrial
myopathies
Moderate Primary prevention due to heart block risk
Symptomatic bradycardia
FA Rare Symptomatic bradycardia
Shared Decision-making and End-of-life Care
Shared decision-making and end-of-life decisions
COR LOE Recommendations
1 C-EO 1. In patients with NMD who are considering or have a
pacemaker or ICD, education on function including
deactivation should be periodically discussed with the patient,
their family members, and/or healthcare decision makers.
1 C-EO 2. In patients with NMD in whom the presence of
conduction disorder portends a risk of VAs, the decision
of whether to implant a pacemaker or ICD should be
concordant with the patient's overall medical care goals and
clinical status.
1 C-EO 3. In patients with NMD who are considering ICD
replacement and are undertaking advanced care planning,
discussing the options of deferring ICD replacement is
recommended.
