64
Specific Conditions
4.3.5. Tetralogy of Fallot
COR LOE
Recommendations
erapeutic (cont'd)
2a C-LD
13. In adults with repaired TOF with moderate or greater
pulmonary valve dysfunction* and progressive ventricular
systolic dysfunction, pulmonary valve replacement (surgical
or transcatheter) is reasonable to preserve ventricular
function.
2b C-LD
14. In adults with repaired TOF with at least moderate
pulmonary valve dysfunction* and progressive functional
tricuspid regurgitation of a moderate or greater severity
associated with RV dilation, pulmonary valve intervention
may be reasonable to prevent worsening tricuspid valve
dysfunction and RV dilation.
2b C-LD
15. For carefully selected patients with repaired TOF and
hemodynamically tolerated monomorphic ventricular
tachycardia related to a well-defined anatomical isthmus,
ablation monotherapy may be considered in lieu of ICD
placement at centers with ACHD electrophysiolog y
expertise.
2b C-LD
16. In adults with repaired TOF and moderate or greater
pulmonary regurgitation and ventricular tachyarrhythmia
requiring treatment,
‡
pulmonary valve replacement (surgical
or percutaneous), in addition to electrophysiological
intervention, may be considered.
* Pulmonary valve dysfunction defined as moderate PR (CMR-derived RF ≥25%) or RVSP
>2/3 systemic pressure due to RVOT obstruction.
†
Refer to Table 32 and Table 33 for individual risk scores.
‡
Antiarrhythmic classes I/III or amiodarone, catheter ablation, electrical cardioversion,
defibrillation, pace-termination, or ICD shock for sustained ventricular arrhythmia.
CMR indicates cardiovascular magnetic resonance; ICD, internal cardioverter-defibrillator;
PR, pulmonary regurgitation; RF, regurgitant fraction; RV, right ventricular; RVOT, RV
outflow tract; RVSP, RV systolic pressure.
(cont'd)
