Conclusion:
Malignant ventricular arrhythmias are a challenge in the management of
LMNA cardiomyopathy patients. The probability of midmyocardial substrate
is high in these patients and sometimes the use of an endocardial and
epicardial catheter approach is not effective in identifying
arrhythmogenic substrate. Careful planning in a LMNA-experienced center
with the availability of ablation tools to target the mid-myocardium may
optimize chances of successful catheter ablation.