Outcome of pulmonary valve replacements in adults after tetralogy repair: a multi-institutional study.

Objective: The purpose of this study was to assess the outcome of pulmonary valve replacement (PVR) in adults with moderate/severe pulmonary regurgitation after tetralogy repair, with particular emphasis on patient outcome, durability of valve repair, and improvement in symptomatology.

Design/setting/patients: The project committee of the International Society of Congenital Heart Disease undertook a retrospective multi-institutional analysis of PVR. Seven centers participated in submitting data on 93 patients >18 years of age who had the operation performed and follow-up obtained. The average age of PVR was 26+/- years (median 27 years). Time of follow-up after replacement was 3 years (range 4 days-28 years). OUTCOMES/MEASURES/RESULTS: Kaplan-Meier estimates of durability of PVR showed approximately 50% replacement at 11 years. There were two deaths at 6 and 12 months after valve replacement. Right ventricular (RV) size estimated by echocardiography from pre- to postoperative studies decreased in 81% (P < 0.001 testing for equal proportions), but RV systolic function increased in only 36% (P = 0.09). Ability index improved in 59% (P < 0.001) and clinical heart failure status improved in 57% with this problem before PVR. PVR did not improve arrhythmia status in a small group of patients.

Conclusions: PVR is associated with low mortality, decrease in RV size and improvement in ability index, and uncertain effects on RV systolic function. Average valve durability was approximately 11 years. Criteria for PVR that will preserve RV function are not clearly identified, and management of these patients remains a difficult enterprise.