Late pulmonary valve replacement after correction of Fallot's tetralogy

Background: The aim of this study was to investigate necessity and outcome of late pulmonary valve replacement (PVR) after repair of tetralogy of Fallot (TOF). Methods: Hospital records from patients operated on for TOF at our institution between 1960 and 2002 were reviewed and patients were interviewed by questionnaires. Results: Out of 411 long-term survivors after TOF-repair, 47 (11.4%) patients required reoperation after 13.2 +/- 7.4 years. Preoperative right ventricular (RV) dilatation was present in 36 (76.6%) patients including 16 (34%) with impaired RV function. Isolated PVR was performed in 12 patients (25.5%). Additional procedures were necessary in 35 patients (74.5%), including closure of residual defects (VSD, n = 11), tricuspid valve replacement (n = 1) and repair (n = 3). Obstructive right ventricular or pulmonary artery lesions (34 patients, 72.3%) were all surgically addressed. RV pressure decreased from 61.1 +/- 27.7 to 42.9 +/- 13.3 mmHg (p < 0.01) after PVR. RV size was reduced and RV function improved compared to preoperative values. Early mortality after reoperation was 2.1% (n = 1) with one patient dying from biventricular failure. There was no late mortality. Conclusions: PVR after Fallot repair is frequently required because of progressive RV enlargement with dysfunction. It can be performed with relatively low risk, even in the setting of multiple reoperation. Obstructive lesions (RVOTO, PA stenosis) and residual defects are frequently observed in patients needing late PVR and may play a crucial role in the development of RV failure. Timely valve replacement with repair of all obstructive lesions proximal and distal to the implanted valve is the key to preserving RV function.