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.............................................................................. • ECG often shows complete right bundle branch block dependent on the surgical approach. The QRS width may also be influenced by the degree of RV dilation. • Echocardiography provides assessment of residual RVOTO and PR, residual VSD, RV and LV size and function,241 TR, RV pressure, aortic root size, and AR. Strain measurements are helpful in quantifying the degree of electromechanical dyssychrony.243

• CMR is the method of choice for assessment of RV volume and function; PR; size, shape, and expansion of the PAs; infundibulum; the ascending aorta; the position of great vessels or conduits in relation to the sternum (resternotomy); and evaluation for residual shunt (Qp:Qs). Late gadolinium enhancement demonstrates fibrosis, the extent of which relates to other risk factors for VT and SCD.244 T1 mapping may have an emerging role. • CCT provides information on coronary arteries (particularly important for the assessment of the spatial relationship with the RVOT prior to TPVI or surgery), the extent of conduit calcification (percutaneous valve anchoring), and the presence of major aortic pulmonary collaterals (MAPCAs). CCT may also be considered as an alternative for RV quantification in patients unable to undergo CMR. • CPET assists timing of reintervention and provides prognostic information.23 • Holter monitoring, event recorder, and EP evaluation are required for selected patients (high risk, suspected or clinical arrhythmia, and/or before RVOT reoperation). Inducible sustained VT carries prognostic value for clinical VT and SCD.245 • Cardiac catheterization should be restricted to patients undergoing catheter-based interventions (i.e. relief of distal PA stenosis, transcatheter valve implantation) and when non-invasive evaluation is inconclusive. Before surgery, coronary angiography may visualize the coronary arteries, which is important to assess the spatial relationship with the RVOT prior to TPVI.

4.10.4 Late surgical/catheter interventional treatment PVRep and/or relief of RVOTO can be performed with low mortality risk in patients without heart failure and/or advanced ventricular dysfunction.246 PR is the most frequent reason for consideration of surgery. Optimal timing remains challenging. Longitudinal data are more important than single measurements to assist timing for reintervention.247 Normalization of RV size after reintervention becomes unlikely as soon as the end systolic index exceeds 80 mL/m2 and the end diastolic volume index exceeds 160 mL/m2,248250 but this cut-off for reintervention may not correlate with clinical benefit. A recent meta-analysis demonstrated that PVRep can improve symptoms and reduce RV volume, but a survival benefit still needs to be shown.251 Distal PA stenosis must be addressed, either at the time of surgery (including intra-operative stenting) or with a percutaneous approach. A biological pulmonary valve (xenograft or homograft) seems to have a mean lifespan of 1020 years,248,252,253 and future replacement could be performed by transcatheter valve-in-valve

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Figure 8 Management of repaired tetralogy of Fallot: long-term complications to address during follow-up. Ao = aorta; LV = left ventricle; RV = right ventricle; PA = Pulmonary artery; TV = tricuspid valve; RA = right atrium; RV = right ventricular; LA = left atrium; PA = pulmonary artery; PS = pulmonary stenosis; SCD = sudden cardiac death; VSD = ventricular septal defect. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026