Individuals with repaired Tetralogy of Fallot (rTOF) remain at risk for sudden cardiac death from ventricular tachycardia (VT). Transcatheter pulmonary valve replacement (TPVR) indications continue to broaden, yet its capability to reduce the risk of VT and sudden cardiac death remains unknown. Thus, in a cohort of participants with rTOF who are presenting for TPVR the investigators intend to: (1) quantify and localize right ventricular (RV) isthmuses with abnormal voltage and/or conduction velocity; (2) identify which RV isthmuses are at risk of being "jailed" by TPV prostheses; and (3) explore the feasibility of omnipolar technology to characterize wavefront directionality and differentiate slow conduction from conduction block.
Diagnosis of tetralogy of Fallot (TOF) or double outlet right ventricle (DORV)
Referred for transcatheter pulmonary valve replacement (TPVR) per routine clinical indications
Weight >=25 kg
None
Single group
Electroanatomic substrate mapping
Diagnostic testParticipants will undergo a sinus rhythm RV substrate map using the HD Grid catheter (Abbott) and the Ensite X electroanatomic mapping system (Abbott) prior to TPVR.
Jailed anatomical isthmus area
Percent jailing of each anatomical RV isthmus (jailed area / isthmus area x100)
Future arrhythmic event (composite)
Documented sustained VT, inducible sustained VT/VF at a future EP study, appropriate ICD therapy for VT/VF, sudden cardiac arrest
Presence of any jailed anatomical isthmus
\>50% of an anatomical isthmus covered by the TPV prosthesis
Number, location, and dimensions of electroanatomically normal isthmuses.
isthmuses with omnipolar voltage \>1.5 mV and conduction velocity \>0.5 m/sec
Number, location, and dimensions of electroanatomically abnormal isthmuses.
isthmuses with omnipolar voltage \<1.5 mV and conduction velocity \<0.5 m/sec
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Boston Children's Hospital
Lead sponsor
Abbott
Collaborator
