35
VAs, sudden cardiac death, and use of ICDs in mitochondrial
myopathies including FA
COR LOE Recommendations
1 B-NR 1. In patients with mitochondrial myopathies including
FA with spontaneously occurring VF or sustained
hemodynamically significant VT, ICD therapy is indicated if
concordant with the patient's goals of care and clinical status.
2a B-NR 2. In patients with mitochondrial myopathies including
FA with an LVEF ≤35% despite GDMT, ICD therapy is
reasonable if concordant with the patient's goals of care and
clinical status.
Atrial arrhythmias in mitochondrial myopathies including FA
COR LOE Recommendation
1 B-NR 1. In patients with mitochondrial myopathies including FA,
anticoagulation according to established guidelines and
clinical context is recommended for AF or AFL taking into
consideration the risks of thromboembolism and the risks
of bleeding on oral anticoagulation.
Bradycardias, conduction disorders, and use of pacing or
CRT in mitochondrial myopathies including FA
COR LOE Recommendations
2a B-NR 3. In patients with FA with an LVEF ≤35% despite GDMT,
with a combination of sinus rhythm, LBBB, QRS duration
≥150 ms, and NYHA class II to class IV symptoms, or in
those with suspected RV pacing-induced CM or anticipated
RV pacing ≥40%, CRT is reasonable if concordant with the
patient's goals of care and clinical status.
2a B-NR 4. In patients with mitochondrial myopathies including FA
with progressive ECG conduction disorder including any
degree of AV or fascicular block, PPM implantation is
reasonable if concordant with the patient's goals of care and
clinical status.
(cont'd)