33
Management strategies Key points
• Management options discussed
included an aggressive evaluation
of syncope and consideration of
ICD implantation because of the
patient's cardiac involvement—both
conduction system disease and dilated
CM—in the setting of LGMD1B.
• Values elicited included patient
preference for pacemaker over ICD.
• She was treated with hydralazine,
beta-blockers, and diuretics. After
discussion with the patient and her
family, a CRT-P pacemaker was
placed.
• Syncope in this context is a serious
symptom and mandates aggressive
evaluation.
• Dilated CM in this context requires
treatment of left ventricular dysfunction
and heart failure.
• Biventricular pacing is used if the burden
of ventricular pacing is expected to be
significant.
• Shared decision making is important in
consideration of the type of device to be
implanted.
• Management options discussed
included periodic assessment for
brady- or tachyarrhythmias and
the high risk of conduction disease
and sudden death associated with
LGMD1B that could require device
implantation.
• The absence of documented
abnormalities on ECG and especially
symptom-rhythm correlation on
ambulatory monitoring supported
avoiding empiric cardiac rhythm
management (CRM) device
implantation in favor of long-term
cardiac rhythm monitoring.
• Patients with LGMD1B are at high risk
for both conduction disease and sudden
death.
• Symptoms are often the main driver of
long-term monitoring, in the absence of
arrhythmia or high-risk features on ECG
or ambulatory ECG monitoring.
• PR interval ≥240 ms and LBBB or
fascicular block are known to be risk
factors for future need for pacemaker
or ICD.
• EP testing can be employed for patients
where there is high suspicion of
conduction disease, with consideration of
programmed ventricular stimulation.
