A Novel Approach for Tetralogy of Fallot-Absent Pulmonary Valve Using Bioresorbable Airway Splints

Tetralogy of Fallot with absent pulmonary valve (ToF-APV) is associated with severe tracheobronchomalacia (TBM) and significant airway compromise. These patients often require early repair with right ventricle-to-pulmonary artery conduit, pulmonary arterioplasty, tracheostomy, and long-term ventilator support. A bioresorbable, 3D-printed airway splint has shown early success in treating severe TBM and has the potential to obviate the need for early repair with conduit and tracheostomy. A retrospective case series analysis was conducted on consecutive patients with ToF-APV and severe TBM who underwent airway splinting between 2012 and 2021. Clinical data was extracted from the medical record. Patients were grouped and analyzed according to their sequence of procedures. Eight patients with ToF-APV and severe TBM underwent airway splinting with a median follow up of 3.6 years (range 1.0-6.4). All patients were alive at the most recent follow-up. Five patients underwent complete cardiac repair first, and one patient underwent concurrent complete cardiac repair and airway splinting. All six of these patients required tracheostomy and long-term ventilator support, even after airway splinting. Five of six remained on ventilator support at the most recent follow up. Two patients underwent airway splinting before complete cardiac repair. Neither required tracheostomy nor prolonged ventilatory support (p = 0.036). Both were discharged home in the neonatal period and returned as infants to undergo elective ToF-APV repair. Patients with TOF-APV and severe TBM may be able to avoid early repair with conduit placement and tracheostomy by undergoing airway splinting prior to complete cardiac repair.