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Bradycardias, conduction disorders, and use of pacing or
CRT in mitochondrial myopathies including FA
COR LOE Recommendations
1 B-NR 1. In patients with mitochondrial myopathies including
FA and documented symptomatic bradycardia due to
sinus node dysfunction or any degree of AV block, PPM
implantation is indicated if concordant with the patient's
goals of care and clinical status.
1 B-NR 2. In patients with mitochondrial myopathies including FA
and third-degree or advanced second-degree AV block at
any anatomical level, with or without symptoms, PPM
implantation is indicated if concordant with the patient's
goals of care and clinical status.
Diagnostic testing and risk stratification in mitochondrial
myopathies including FA
COR LOE Recommendations
1 B-NR 1. Coordinated care of patients with mitochondrial
myopathies including FA 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 mitochondrial myopathies including FA,
cardiac evaluation including examination, ECG, ambulatory
ECG, and cardiac imaging (echocardiography or CMR) at
diagnosis with periodic retesting is recommended even in the
absence of cardiac symptoms.
Diagnostic testing and risk stratification in FSHD
COR LOE Recommendation
2a B-NR 1. In patients with FSHD, cardiac evaluation including
examination, ECG, ambulatory ECG, and cardiac imaging
(echocardiography or CMR) at diagnosis with periodic
retesting are reasonable even in the absence of cardiac
symptoms.
Mitochondrial Myopathies Including Friedreich Ataxia
Facioscapulohumeral Muscular Dystrophy
