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The success story of patients born with tetralogy of Fallot (ToF) is closely linked to significant progress in the treatment of congenital heart disease that resulted in low early mortality and a favourable course during the first two decades of life. Consequently, the majority of patients with repaired ToF survive to adulthood and participate in regular work and social life. The first outcome studies of larger cohorts of patients, however, demonstrated a significant risk of sudden death and malignant arrhythmia in the long-term follow-up.1 As a consequence of surgical relief of right ventricular (RV) outflow tract obstruction, pulmonary regurgitation (PR) resulted as the predominant, residual haemodynamic lesion causing progressive RV enlargement, functional impairment and probably sudden cardiac death. Therefore, pulmonary valve replacement (PVR) was considered necessary in order to abolish valvular insufficiency and to allow reverse remodelling of the dilated RV to normal values. Unfortunately, as RV volumes and function did not recover in all patients and adverse events occurred also frequently following PVR, extensive discussion evolved regarding the optimal time point for PVR taking into account the risk of irreversible RV remodelling versus degeneration of the implanted valve prosthesis over time.
Cardiac MR (CMR) imaging has contributed substantially to the understanding of the pathophysiological consequences of PR due to its ability for reliable quantification of PR and volumetric RV assessment in this population. Consequently, current recommendations for PVR are largely based on CMR studies investigating the effect of PVR on RV …
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.