Surgical progress has not influenced late risks for death, re-operation or PVR in adults with repaired tetralogy of Fallot. Instead, reduction of early surgical mortality to <2% is responsible for excellent late survival >90% overall. The constant risk of PVR is low and independent of repair type. Baseline morphologic features are important determinants of late outcome.

Objective: While there are no controlled studies, pulmonary valve replacement (PVR) after late tetralogy of Fallot repair is performed for patients with symptoms, arrhythmia or ventricular dysfunction. We sought to determine the impact of PVR on clinical outcomes. Methods: In a matched cohort study, 82 patients with PVR after tetralogy of Fallot repair without a history of ventricular arrhythmia were matched and compared with similarly followed non-PVR control subjects. A propensity-score adjusted analysis using repeated measures regression techniques was performed. Results: For the PVR subjects, the mean age was 28 years with a mean duration follow-up of 9 years. Before PVR, these subjects were significantly more likely than matched non-PVR subjects to have had non-ventricular arrhythmias, symptoms, lower functional class, longer QRS duration, lower right ventricular ejection fraction and higher right ventricular pressure, and reduced exercise duration but not aerobic capacity. During follow-up, sudden death occurred in four non-PVR subjects compared with no PVR subjects, with three episodes of ventricular tachycardia in the PVR subjects versus none in the non-PVR subjects ( p = 0.49). Symptoms and functional class improved in the PVR subjects with no change in the non-PVR subjects ( p < 0.001). The change in QRS duration was not significantly different between PVR and non-PVR subjects ( p = 0.48). Oxygen consumption at peak exercise did not significantly change in either group. For PVR subjects, there was a significant qualitative reduction in pulmonary ( p < 0.001) and tricuspid valve regurgitation ( p = 0.009) and right ventricular size ( p < 0.001) and dysfunction ( p < 0.001) noted on echocardiography. Conclusions: Symptoms and functional status are improved after late PVR, with a reduction in pulmonary and tricuspid valve regurgitation and right ventricular size and dysfunction. While a significant impact on arrhythmia was not detected, there were no sudden deaths in the PVR subjects. #