63
4.3.5. Tetralogy of Fallot
COR LOE
Recommendations
Diagnostic (cont'd)
2a B-NR
5. In adults with repaired TOF and moderate risk for future
sustained ventricular tachycardia and/or SCD, invasive
electrophysiolog y evaluation is reasonable to inform clinical
management.
2a C-EO
6. In adults with repaired TOF in whom adequate data cannot
be obtained noninvasively in the setting of an arrhythmia,
heart failure, unexplained ventricular dysfunction, suspected
pulmonary hypertension, residual hemodynamic lesions, or
cyanosis, cardiac catheterization with angiography is reasonable
for assessing hemodynamics to direct management and therapies.
2b C-LD
7. In adults with repaired TOF undergoing pulmonary valve
placement (surgical or transcatheter), preprocedural invasive
electrophysiolog y evaluation with programmed ventricular
stimulation and 3-dimensional mapping may be considered
to identify and treat latent monomorphic ventricular
tachycardia substrates.
2b C-LD
8. In adults with repaired TOF, noninvasive evaluation (12-lead
electrocardiography, contrast-enhanced MR imaging, and/
or CT angiography) may be considered to characterize
ventricular tachycardia substrates.
erapeutic
1 B-NR
9. In symptomatic adults with repaired TOF and moderate
or greater pulmonary valve dysfunction,* pulmonary valve
replacement (surgical or transcatheter) is recommended for
relief of symptoms.
2a B-NR
10. In asymptomatic adults with repaired TOF, moderate or greater
pulmonary valve dysfunction,* and at least 2 of the following—
RV end-systolic volume index >80 mL/m
2
, RV end-diastolic
volume ≥2x LV end-diastolic volume, RV ejection fraction
≤46%, LV ejection fraction ≤50%, or progressive decline in
exercise capacity—pulmonary valve replacement (surgical
or transcatheter) is reasonable for improving outcomes,
including lowering risks for ventricular arrhythmias and death.
2a B-NR
11. In adults with repaired TOF and high risk (by clinical
markers and/or multivariable score
†
) for SCD, placement of
an ICD is reasonable to prevent SCD.
2a B-NR
12. In adults with repaired TOF and appropriate ICD therapies
for monomorphic ventricular tachycardia, adjunctive catheter
ablation is reasonable to reduce ventricular tachyarrhythmia
burden.
(cont'd)
