Curative ablation in a 12-month-old baby with severe ventricular systolic dysfunction and incessant ventricular tachycardia (original) (raw)
In patients with tetralogy of Fallot (ToF), inducible sustained VT is associated with approximately five-fold risk of sudden cardiac death or clinical sustained VT. 2 Patients with Rastelli repairs have potential substrates for the development of macro-reentrant VT as per repaired ToF, including VSD patches and RV suture lines. The approach to VT ablation in patients with congenital heart disease is founded on review of all imaging studies, original surgical/ interventional reports, and documented tachyarrhythmias. In the current case, identified anatomical and electrical barriers to conduction around which circuits may propagate included the Rastelli conduit, patch-repaired VSD, TA, and RVOT. When activation and entrainment mapping cannot be performed due to non-inducibility or haemodynamic instability, pace-mapping and/or non-contact mapping can be utilized. In our patient due to the presence of a scar around and in proximity to the outflow tract extensive pace mapping of potential macro re-entry isthmi was also undertaken to exclude an exit site from a potential macro re-entry circuit. Interestingly, the suspected site was located within the native remnant RVOT, where ablation was performed in sinus rhythm. High rates of appropriate ICD shocks occur in primary and secondary prevention patients with repaired ToF. 3 The only predictor of appropriate ICD shocks was a prior ventriculotomy incision. For such patients, VT ablation is not a stand-alone therapy for the prevention of sudden cardiac death. Conclusion Ventricular tachycardia appears to be infrequent following Rastelli repair, but have been poorly characterized. This case demonstrates the feasibility, safety, and potential efficacy of RVOT VT ablation in the setting of Rastelli repair.
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