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Management strategies Key points
• Management options discussed
centered on the risks and benefits of
the generator exchange procedure
and the alternatives to not having
generator change.
• EDMD2 puts him at high risk for
the development of potentially lethal
ventricular tachyarrhythmias.
• The patient had not had any shocks,
and his pacing requirements were
minimal.
• Discussion of his reluctance for ICD
generator exchange centered on the
risk and on the risks and benefits of
the procedure and the alternatives to
not having generator change.
• ICD generator change was ultimately
deferred based on shared decision
making.
• Patients with EDMD2 are at high risk
for the development of potentially lethal
ventricular tachyarrhythmias.
• Patients with NMDs who have
implantable devices need to be counseled
about the need for device and long-term
maintenance prior to implantation.
• Eliciting the patient's overall medical care
goals and preferences with consideration
of the individual's neuromuscular
prognosis is recommended when the
option of discontinuing device therapy
is present.
• Generator change presents an
opportunity to discuss goals of care and
patient preferences including their desire
to downgrade or avoid CIED therapy.
• Management options discussed
included anticoagulation and device
placement (ICD versus pacemaker) due
to AV block.
• Continued cardiovascular medical
therapy was recommended regardless of
arrhythmia management strategy due to
risk of CM.
• Values elicited in discussion included
options to preserve quality of life and
desire for protection against stroke and
sudden cardiac death.
• Therapy with warfarin was begun with
plans for ICD placement.
• Upon arrival for ICD placement, the
patient was in complete AV block.
ICD implantation was successfully
performed.
• Due to lack of representation of pediatric
patients with NMDs in previously
published trials of anticoagulation for
atrial arrhythmias and single-chamber
ICD placement, extrapolation of data
from adults was required in conjunction
with clinical decision making.
• Family history of progression of EDMD
to CM requiring transplant at 17 years of
age was factored into decision making.
• Anticoagulation is warranted due to
scarred atria with incessant arrhythmia
and poor atrial transport as a nidus for
thrombus.
• As direct oral anticoagulants are not
approved for use in children, warfarin was
prescribed.
