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Atrial arrhythmias in EDMD and LGMD1B
COR LOE Recommendations
1 B-NR 1. In patients with EDMD or LGMD1B, anticoagulation is
recommended for AF or AFL, taking into consideration the
risk of bleeding on oral anticoagulation.
1 B-NR 2. In patients with EDMD, anticoagulation is recommended
for atrial standstill, taking into consideration the risk of
bleeding on oral anticoagulation.
VAs, sudden cardiac death, and use of ICDs in EDMD and
LGMD1B
COR LOE Recommendations
1 B-NR 1. In patients with EDMD or LGMD1B in whom ICD
therapy is planned, an ICD system with permanent pacing
capability is recommended.
1 B-NR 2. In patients with EDMD or LGMD1B who are survivors
of spontaneously occurring hemodynamically significant
sustained VT or VF, ICD therapy is indicated if concordant
with the patient's goals of care and clinical status.
1 B-NR 3. In patients with EDMD or LGMD1B with at least one of
the following : second-degree or third-degree AV block,
PR interval ≥230 ms, or spontaneous HV ≥70 ms, ICD
therapy is recommended if concordant with the patient's
goals of care and clinical status.
1 B-NR 4. In patients with EDMD or LGMD1B with an LVEF ≤35%
despite GDMT, ICD therapy is indicated if concordant
with the patient's goals of care and clinical status.
1 B-NR 5. In patients with EDMD or LGMD1B in whom clinically
relevant VAs are induced during EP study, ICD therapy is
recommended if concordant with the patient's goals of care
and clinical status.
2a B-NR 6. In patients with EDMD or LGMD1B with LVEF <45%
and nonsustained VT, an ICD is reasonable if concordant
with the patient's goals of care and clinical status.
2a C-LD 7. In patients with EDMD or LGMD1B with at least one
of the following : LBBB, RBBB, or AF/AFL with slow
ventricular response (ventricular rate <50 bpm), ICD
therapy is reasonable if concordant with the patient's goals
of care and clinical status.
2b C-LD 8. In patients with EDMD or LGMD1B with symptomatic
sinus node dysfunction or sinus bradycardia with heart rate
<40 bpm, ICD therapy may be considered if concordant
with the patient's goals of care and clinical status.
