Intubation Prior to Tetralogy of Fallot with Absent Pulmonary Valve Surgery: A Tale of Two Ventilation Pathways

Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare form of congenital heart disease that has both cardiac and respiratory implications. While some patients are asymptomatic at birth, others have significant airway disease requiring mechanical ventilation and urgent surgical repair.

To compare the relationship between preoperative mechanical ventilation on durations of both total invasive mechanical ventilation and non-invasive ventilation in patients undergoing TOF/APV repair. Secondary aims include comparing echocardiographic parameters with clinical predictors and outcomes.

Retrospective chart review of 23 neonates and infants less than one year-old undergoing initial repair for TOF/APV. Patients were stratified by whether they required mechanical ventilation for cardiopulmonary stabilization in the immediate preoperative period. Patients were assessed for the durations of invasive and non-invasive ventilation. Echocardiographic parameters including main pulmonary artery, branch pulmonary arteries, and pulmonary valve z-scores were compared to clinical outcomes as well.  RESULTS: Patients intubated preoperatively had significantly longer initial intubations (383.28 [211.47, 500.30] vs. 33 [23.93, 140.0] hours, p < 0.001) and total duration of intubation (599.82 [441.83, 3324.22] vs. 33 [23.93, 141.17] hours, p < 0.001). Patients intubated preoperatively had significantly longer durations of non-invasive ventilation (173.33 [89.33, 585] vs. 0 [0, 72.57] hours, p = 0.004). Only LPA z-scores were associated with the need for preoperative intubation. Preoperative intubation was associated with longer CICU and hospital length of stay.  CONCLUSIONS: In patients with TOF/APV, the need for preoperative intubation is a predictor of postoperative respiratory complexity and length of stay. Echocardiographic data must be interpreted within the broader clinical context. Recognizing the potential implications in patients requiring preoperative respiratory support may help clinical teams anticipate postoperative ventilatory needs, inform perioperative planning, and guide family counseling.