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procedures. There is little experience with mechanical valves in this setting and there is concern about adequate anticoagulation. Indications for TV annuloplasty, closure of residual VSDs, and/or aortic root dilation/AR surgery should also be addressed at the time of surgery. The indication for aortic root surgery does not differ from the general population.254 TPVI techniques have become an alternative to open heart surgery primarily in patients with RVOT conduit stenosis/regurgitation, but also in selected patients with native RVOT regurgitation/stenosis. TPVI, when technically feasible, provides outcomes comparable to surgical PVRep and is intended to extend the lifetime of a conduit, hence reducing the number of reoperations during a patient’s lifetime.255 Stent fracture  the initially most common complication  has become less of a problem with careful preparation of the ‘landing zone’ using additional stents. Best long-term results were reported when a residual gradient of <15 mmHg could be achieved.256 Uncommon complications, occurring in <2% of patients, include conduit rupture and coronary artery compression. The risk of endocarditis after TPVI remains a concern with an annual incidence rate of 23%.257,258 Since coronary artery compression may be lifethreatening, a balloon test to exclude potential coronary artery compression must be performed prior to TPVI, although this test carries a risk for conduit rupture. In case of severe circumscript conduit calcification, TPVI should only be performed if a CCT scan shows sufficient distance between the conduit and the coronary arteries. The strong link between slowly conducting anatomical isthmuses and sustained monomorphic VT, and the potential loss of accessibility to the anatomical isthmus by catheter ablation after surgical PVRep or after transcutaneous insertion of valves in patch augmented RVOTs, has important implications for patients who undergo reinterventions.259 Whether pre-interventional mapping and preventive ablation of slowly conducting anatomical isthmuses before or during intervention in patients without documented spontaneous sustained VT is beneficial is under investigation.

4.10.5 Indications for electrophysiological testing and implantable cardioverter defibrillator An ICD should be implanted for secondary prevention of SCD (patients with cardiac arrest or sustained VT) (IC recommendation). ICD implantation for primary prevention remains controversial, and no ideal risk stratification scheme has so far been developed. Patients with unexplained syncope and impaired ventricular function or other risk factors for SCD should undergo haemodynamic and EP evaluation. In the absence of a reversible cause, ICD implantation should be considered (see section 3.4.2).260,261

Recommendations for intervention after repair of tetralogy of Fallot

Recommendations Classa Levelb

PVRep is recommended in symptomatic patients with severe PRc and/or at least moderate RVOTO.d I C In patients with no native outflow tract,e catheter intervention (TPVI) should be preferred if anatomically feasible. I C PVRep should be considered in asymptomatic patients with severe PR and/or RVOTO when one of the following criteria is present. • Decrease in objective exercise capacity. • Progressive RV dilation to RVESVi >_80 mL/

m2, and/or RVEDVi >_160 mL/m2 f, and/or progression of TR to at least moderate. • Progressive RV systolic dysfunction. • RVOTO with RVSP >80 mmHg. IIa C

Continued VSD closure should be considered in patients

with residual VSD and significant LV volume overload or if the patient is undergoing pulmonary valve surgery. IIa C In patients with sustained VT who are undergoing surgical PVRep or transcutaneous valve insertion, pre-operative catheter mapping and transsection of VT-related anatomical isthmuses before or during the intervention should be considered. IIa C Electrophysiologic evaluation, including programmed electrical stimulation, should be considered for risk stratification for SCD in patients with additional risk factors (LV/RV dysfunction; non-sustained, symptomatic VT; QRS duration >_180 ms, extensive RV scarring on CMR). IIa C ICD implantation should be considered in selected TOF patients with multiple risk factors for SCD, including LV dysfunction, non-sustained, symptomatic VT, QRS duration >_180 ms, extensive RV scarring on CMR, or inducible VT at programmed electrical stimulation. IIa C Catheter ablation or concomitant surgical ablation for symptomatic monomorphic sustained VT may be considered in those with a preserved biventricular function as an alternative to ICD therapy, provided that the procedure is performed in highly experienced centres and that established ablation endpoints have been reached (e.g. non-inducibility, conduction block across ablation lines). IIb C

CMR = cardiovascular magnetic resonance; ICD = implantable cardioverter defibrillator; LV = left ventricle/ventricular; PR = pulmonary regurgitation; PVRep = pulmonary valve replacement; RV = right ventricle/ventricular; RVESVi = right ventricular end systolic volume indexed; RVEDVi = right ventricular end diastolic volume indexed; RVOT = right ventricular outflow tract; RVOTO = right ventricular outflow tract obstruction; RVSP = right ventricular systolic pressure; SCD = sudden cardiac death; TOF = tetralogy of Fallot; TPVI = transcatheter pulmonary valve implantation; TR = tricuspid regurgitation; VSD = ventricular septal defect; VT = ventricular tachycardia. aClass of recommendation. bLevel of evidence. cRegurgitant fraction by CMR >3040%. dPeak velocity >3 m/s. ePatients with previous RVOT surgery using homografts, bovine jugular vein grafts, bioprostheses/conduits. fConfirmed by repeated measurements. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026