7
Diagnostic testing and risk stratification in Duchenne, Becker,
and recessive limb-girdle muscular dystrophies
COR LOE Recommendations
1 B-NR 1. Coordinated care of patients with DMD, BMD, or
LGMD2 should be conducted in a medical setting where
there is access to expertise in the neurological, cardiac,
arrhythmic, pulmonary, and genetic manifestations of these
disorders.
1 B-NR 2. In patients with DMD, BMD, or LGMD2, guideline-
directed evaluation and therapy for heart failure is
recommended.
1 B-NR 3. In patients with DMD, BMD, or LGMD2,
cardiac evaluation including physical examination,
electrocardiogram (ECG), ambulatory ECG, and cardiac
imaging (echocardiography or cardiac magnetic resonance
imaging [CMR]) at diagnosis with periodic retesting is
recommended even in the absence of cardiac symptoms.
1 B-NR 4. In females who are carriers of a pathogenic or likely
pathogenic variant for DMD or BMD, screening cardiac
imaging (echocardiography or CMR) is recommended in
adulthood even in the absence of cardiac symptoms.
2a C-LD 5. In patients with DMD, BMD, or LGMD2 who have
symptoms of conduction disorder or arrhythmias without
an obvious cause, implantable cardiac monitoring is
reasonable.
