47
Management strategies Key points
• Referral for evaluation by pulmonary medicine
showed restrictive pulmonary function testing
consistent with severe respiratory muscle
involvement.
• Coordinated care of patient was conducted
with discussion with neurolog y, pulmonary
medicine, and cardiac EP regarding neuromuscular
prognosis. Consensus that the likelihood of poor
cardiac or respiratory outcome over the next 2–4
years was high.
• Conference with the patient and husband held
to elicit the medical care goals. The discussion
included the high risk for both cardiac and
pulmonary complications of DM1. The risk of
sudden cardiac death was discussed based on
the severe cardiac conduction disease on the
ECG. Included in the discussion was a review
of the potential benefit of a primary prevention
pacemaker or ICD. An option discussed included
ongoing follow-up without device implantation.
• The patient and family elected to proceed with a
dual-chamber pacemaker implantation.
• The procedure was done with anesthesia support
and required intubation. Pacemaker was implanted
without complications. However, there was failure
to wean off the ventilator post-procedure. The
patient remained in the intensive care unit for 2
weeks due to neuromuscular-related respiratory
failure.
• Despite marginal respiratory status, the patient
was able to wean off the ventilator with nocturnal
bi-level positive airway pressure support, and the
patient was discharged 3 weeks post-implantation.
• At 6-month follow-up after pacemaker
implantation, complete heart block was observed
with pacing suppression. No escape rhythm was
observed with pacing at 30 bpm.
• The patient had progressive respiratory
insufficiency and succumbed to pneumonia 2 years
after pacemaker implantation.
• The husband sent a thank you note to the care team
for providing his wife with additional time to spend
with him and their son who had congenital DM1.
• Poor functional status
portends the high risk for
poor mid- to long-term
outcomes.
• Empiric pacemaker
implantation without further
testing is reasonable given the
ECG with severe conduction
disease.
• Clinical benefit of empiric
ICD placement remains
uncertain and would be
potentially less advantageous
as long-term outcome in the
patient is poor.
• Eliciting the overall goals
of care and preferences led
to the patient's decision to
go ahead with pacemaker
implantation.
• Coordinated care between
neurolog y, pulmonary
medicine, cardiac EP,
and anesthesiolog y led
to a procedure with the
anticipated issues but overall
favorable outcome.
