Causes and factors associated with late mortality after the Fontan operation are summarised in this study. The presented information will aid in identifying patients at highest risk for mortality and guide our risk stratification efforts in this patient population.

In TOF patients who had undergone PVR, the best preoperative threshold to achieve mid-to-late RV normalization was RV ESV < 80 mL/m(2). Patients with preoperative RV ESV > 95 mL/m(2) were at increased risk for suboptimal haemodynamic outcome and adverse clinical events. Our findings may assist in timing of PVR.

In patients with repaired TOF, biventricular dysfunction on CMR imaging was associated with major adverse clinical outcomes. The quantified thresholds (RV EF <30% and LV EF <45%) may be implemented in noninvasive risk stratification.

Traditional atherosclerotic risk factors were associated with CAD in ACHD patients. In contrast, ischaemic stroke was related to factors (previous shunts, septal defects, mechanical valves) suggesting a cardioembolic aetiology. These findings may inform surveillance and prevention strategies.

In this large multicentre rTOF cohort, PVR was not associated with a reduced rate of death and sustained VT at an average follow-up of 5.3 years. Additionally, there were more events after PVR compared with no PVR in subjects not meeting consensus criteria.

The extent of QRS fragmentation is superior to QRS duration in predicting mortality in adult patients with TOF and may be used in risk stratification.

No abstract

In patients with rTOF, severe preoperative TR was predictive for adverse events after PVR. Close surveillance is warranted in these patients irrespective of postoperative TR.