Pulmonary regurgitation: not a benign lesion

Pulmonary regurgitation (PR) is a common complication after surgical or percutaneous relief of pulmonary stenosis and following repair of tetralogy of Fallot. Significant PR is usually well tolerated in childhood. However, in the long term, chronic PR has a detrimental effect on right ventricular (RV) function and exercise capacity and leads to an increased risk of arrhythmia and sudden cardiac death (SCD). Recent advances in non-invasive imaging and, in particular, wider availability of cardiovascular magnetic resonance (CMR), have improved the assessment of PR and RV function in these patients. This in turn has facilitated decision making on the optimal timing for elective pulmonary valve replacement (PVR), which should be performed before irreversible RV dysfunction ensues.