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Management strategies Key points
• The consensus from neurology, general
cardiology, and pulmonary medicine was
that the patient is currently stable with
reasonable prognosis at least for the next
several years.
• A discussion with the patient and his
accompanying father was held to review the
risks and benefits of a primary prevention
ICD in FA. The risk of sudden cardiac death
was discussed and shared decision making
was carried out to elicit the medical care
goals.
• Options discussed included ongoing
heart failure therapy with or without ICD
placement.
• Values elicited included the patient's desire
for protection against sudden death in light
of young age and satisfactory functional
capacity.
• The patient and family elected to proceed
with a single-chamber ICD.
• The procedure was performed without
complications. The postprocedural hospital
stay was prolonged due to slow recovery,
with eventual return to baseline functioning
after a 10-day acute rehabilitation stay.
• Two years following ICD placement, the
patient was doing well. The patient was
no longer employed due to difficulty with
transportation. No ICD therapies for
VAs occurred since implantation. ICD
interrogation revealed episodes of irregular
tachycardia in a monitoring zone consistent
with asymptomatic AF. A 14-day event
monitor showed AF episodes lasting up to
24 hours with rates of 80–160 bpm. The
patient was placed on anticoagulation, and
the beta-blocker dosage was increased.
• Progressive loss of muscle function
with wheelchair dependence
10–20 years after symptom onset is
common.
• Concentric hypertrophy is often
observed. It does not increase the
risk of sudden death as in other
genetic causes of HCM.
• About 10% of patients develop left
ventricular systolic dysfunction.
The role of GDMT in limiting
the progression of left ventricular
systolic dysfunction has not been
studied but is extrapolated from
other populations.
• Left ventricular systolic dysfunction
increases the risk of atrial and VAs
like in other disease states.
• Eliciting the overall goals of care
and preferences led to the patient's
decision to move ahead with
primary prevention ICD.
• Patients with NMDs can have
protracted admissions at CIED
placement due to underlying
skeletal muscle dysfunction
including respiratory involvement.
Therapy to return patients to
preimplant level of functioning is
necessary.
• Progressive noncardiac issues
typically limit the quality and
duration of life.
