Long-term outcome of repaired tetralogy of Fallot: Survival, tachyarrhythmia, and impact of pulmonary valve replacement

Background: Pulmonary valve replacement (PVR) is recommended for severe pulmonary regurgitation in repaired tetralogy of Fallot (rTOF).

Objective: The purpose of this study was to investigate the event rate and effectiveness of PVR.

Methods: A retrospective study of tetralogy of Fallot patients who survived total repair from 1970 to 2020 was conducted.

Results: We identified 1744 rTOF patients; 86.6% with classic rTOF, 11.5% with pulmonary atresia, 0.8% with endocardial cushion defect, and 1.1% with absent pulmonary valve. Annual risks of tachyarrhythmia/sudden cardiac arrest (SCA) increased to 0.295% and 1.338% in patients aged 10-30 and 30-60 years, respectively, without sex predominance. PVR (223 surgical and 39 percutaneous) event rate was 34.7% ± 2.1% by 30 years after repair (annual risk: 1.57% between 10 and 30 years after repair). The second PVR rate was 9.9% ± 4.1% by 20 years after the first PVR. Tachyarrhythmia/SCA risk was higher in PVR patients than in No PVR patients and was reduced in PVR patients without tachyarrhythmia/SCA before PVR. However, survival in patients with ventricular tachyarrhythmia/SCA still was better after PVR. At PVR, 13% of patients had tachyarrhythmia/SCA, which was the major predictor of events after PVR. Before PVR, although the ventricular tachyarrhythmia/SCA risks included QRS duration >160 ms and New York Heart Association functional class III or IV, supraventricular tachyarrhythmia was associated with PVR age ≥28 years and N-terminal pro-brain natriuretic peptide >450 pg/mL.

Conclusion: Tachyarrhythmia/SCA occurrence and the need for PVR increased with age during young adulthood. PVR reduced subsequent arrhythmias only in those patients without arrhythmias before PVR.