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Top 10 Take-Home Messages
1. Shared decision-making among patients, their families, and
clinicians is essential whenever diagnostic studies or therapies,
particularly those that are invasive, are being utilized or
contemplated. Counseling and education may result in patients'
refusal or withdrawal of such measures if inconsistent with their
goals of care, and this should be respected.
2. Cardiac testing is appropriate in most patients with neuromuscular
disorders (NMDs) to evaluate for cardiac involvement. The type
of cardiac test and the need for and frequency of repeat testing
is governed by the underlying disorder, results of previous or
new studies, and the patient's symptomatic status. It should be
noted that skeletal muscle impairment may mask or confound
cardiovascular symptoms, requiring heightened vigilance to cardiac
involvement and modification of testing.
3. Previously published guideline-based indications for cardiovascular
implantable electronic device (CIED) use, including cardiac
resynchronization therapy (CRT), and for management of
cardiomyopathy (CM) and heart failure may be applied in patients
with NMDs. For some indications, the level of evidence (LOE)
and/or class of recommendation (COR) in the current document
have been modified from prior guidelines to reflect the under-
representation of patients with NMDs in past studies.
4. A patient's overall prognosis may be affected by the impact of their
underlying neuromuscular condition. Condition-specific technical
challenges including body habitus (such as kyphoscoliosis),
respiratory muscle weakness and sedation-related risks may
influence clinical management. These effects may dominate a
patient's clinical picture and prognosis, possibly attenuating the
benefit from arrhythmia therapy, particularly CIED implantation,
when compared with other patient populations.
5. Patients with Duchenne muscular dystrophy (DMD), Becker
muscular dystrophy (BMD), and recessive forms of limb-girdle
muscular dystrophy (LGMD) rarely develop bradyarrhythmias,
but CM, heart failure, and ventricular arrhythmias (VAs) may
occur with increased frequency. When indicated, CIED therapy in
these patients may pose technical challenges and limited benefit,
particularly in those with advanced neuromuscular impairment.
Overview
