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.