Successful Epicardial Mapping and Ablation of Ventricular Tachycardia after Failed Medical Therapy and Conventional Endocardial Ablation Therapy in a 10-Year-Old Girl with Severe Biventricular Arrhythmogenic Cardiomyopathy

Background: VTs in patients with ACM/ARVC are a severe and life-threatening condition and often caused by a critical epicardial substrate contributing to a scar-tissue-associated reentry mechanism. Sufficient ablation therapy of these substrates may require an epicardial approach accessed by subxyphoidal puncture which is only exceptionally performed in children.

Method: We report the case of a 10–year-old girl diagnosed with ARVC (pathologic variant in PKP2-Gene) with severely impaired biventricular function. She presented with recurrent nonsustained VT and syncope, an ICD was implanted and she was adequately shocked for VT after 4 months. During follow-up, atenolol, sotalol, amiodarone, and antiarrhythmic combination therapy failed to suppress VT. Two RV microreentry VT were conventionally mapped and one of the VTs, originating from the RVOT, could be successfully ablated. Later, three more VTs were identified from the RV anterior wall but a second endocardial ablation failed to suppress the VT. On high dose of amiodarone and milrinone for severe biventricular heart failure and in incessant intermittent slow VT, we performed a combined endocardial an epicardial mapping through a subxyphoidal transpericardial access using 3D high density mapping technique to identify epicardial scar tissue with late and fractionated potentials in the anterior RV wall as the electrophysiological substrate of the clinically relevant VTs. A pressure sensor enabled cooled tip ablation therapy was performed with a total of 83 ablations (cumulative length of 896 seconds). Procedure time was 235 minutes and radiation time was 14.1 minutes. After ablation, VT was no more inducible and no complications occurred.

Results: Here, in a patient with severe biventricuklar ACM/ARVC, only epicardial modification of VT resulted in a significant stabilization of cardiac rhythm and reduction of recurrent VT episodes. During 3-month follow–up, no further hospitalization had been necessary but she has been listed for heart transplant due to congestive heart failure.

Conclusion: An epicardial VT ablation approach can be a life-saving therapeutic option in children with incessant VT in ARVC leading to suppression or reduction of VT episodes and improvement in quality of life. Nevertheless, the specific limitations and risks associated with this complex procedure have to be taken into account.

Publication History

Article published online:
12 February 2022