Surgical Outcomes of Repair for Tetralogy of Fallot With Absent Pulmonary Valve: Our Experience.

Background

Tetralogy of Fallot (TOF) with absent pulmonary valve (PV) accounts for 3% to 6% of TOF cases. It is physiologically distinct due to tracheobronchial compression caused by dilatation of the pulmonary arteries. We reviewed the surgical outcomes of TOF with absent PV at our center using various strategies.

Methods

A retrospective analysis was conducted on all patients diagnosed with TOF with absent PV who underwent surgery from May 2018 to December 2024. Data on demographics, surgical techniques, in-hospital mortality, ventilation duration, intensive care unit (ICU) and hospital stay, and early follow-up outcomes were collected. The surgical technique was individualized to the patient based on the severity of the clinical presentation, age, and radiologic evidence of tracheobronchial compression.

Results

Twenty-eight patients were included (median age: 9 months; median weight: 5 kg); six (21.4%) were neonates. Branch pulmonary artery reduction was performed in 75% (21/28) of cases. Surgical approaches included valveless transannular patch (5/28, 17.8%), 0.1 mm polytetrafluoroethylene (PTFE) bicuspid valve with transannular patch (12/28, 42.8%), PTFE bicuspid valve with LeCompte's maneuver (6/28, 21.4%), and valved conduit repair (5/28, 17.8%). One patient (3.6%) experienced in-hospital mortality. Median mechanical ventilation time was 11 h (range: 4-82 h) with neonates requiring prolonged ventilation (>72 h, median 74.5 h). Median ICU stay was 24 h, and median hospital stay was six days. One patient (3.5%) required reintervention during follow-up.

Conclusion

Tailoring surgical therapy to each patient's clinical presentation and anatomy yields reasonable outcomes in TOF with absent PV. Early presentation and severe airway compression needs an aggressive surgical strategy. Neonates demonstrated a need for prolonged ventilatory support.