Long-term outcomes of pulmonary valve replacement in patients with repaired tetralogy of Fallot

OBJECTIVES: The objectives of this study were to evaluate long-term outcomes of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (TOF) and to identify the factors associated with adverse clinical events (ACEs). METHODS: A total of 190 patients who underwent PVR between 1998 and 2015 after repair of TOF were retrospectively analysed. ACE was defined as all-cause death, heart transplantation or new-onset sustained arrhythmia. Univariable Cox proportional hazards regression analysis was used to identify the factors associated with ACE after PVR. RESULTS: The median age at PVR was 19 years. Preoperative magnetic resonance imaging (MRI) was performed in 143 (75%) patients, and the median right ventricular (RV) end-diastolic and end-systolic volume index was 164 and 82 ml/m², respectively. The follow-up completeness was 94%, and the median follow-up duration was 9.8 years. The transplantation-free survival and freedom from ACE at 15 years was 95% and 90%, respectively. The factors associated with ACE were older age at PVR, older age at TOF repair, New York Heart Association functional class III or IV, presence of tachyarrhythmias, longer cardiopulmonary bypass time and concomitant arrhythmia surgery. In a subgroup analysis of 143 patients with preoperative MRI data, larger RV end-systolic volume index, larger left ventricular end-systolic volume index and lower left ventricular ejection fraction were associated with ACE. CONCLUSIONS: Long-term outcomes of PVR in patients with repaired TOF were satisfactory. Proactive PVR before the onset of advanced symptoms, tachyarrhythmias and ventricular dysfunction may further improve the long-term survival of this patient population.