A New Treatment Paradigm for Tetralogy of Fallot/Absent Pulmonary Valve with Significant Airway Compromise: Addressing the Airway First.

Abstract

Tetralogy of Fallot with absent pulmonary valve (TOF-APV) can manifest neonatally with severe respiratory insufficiency secondary to tracheobronchomalacia (TBM). Typical treatment of patients with TOF-APV with significant respiratory compromise is directed at the underlying heart defect rather than the airway. Complete repair often does not ameliorate airway issues and subsequently requires a tracheostomy or ventilator. Given this lack of effective treatment, a bioresorbable, patient-specific, 3-dimensionally printed splint was developed that provides external support to the airways. Eight patients with TOF-APV and TBM underwent airway splinting. Cohort 1 underwent splinting after (n = 5) or concurrently with (n = 1) a complete repair with a right ventricle-to-pulmonary artery conduit. Cohort 2 underwent splinting before a complete repair (n = 2). All cohort 1 patients required tracheostomy and long-term ventilator support. The cohort 2 patients were discharged home after splinting and returned to undergo elective tetralogy of Fallot (TOF) repair without a conduit. Patients with TOF-APV and airway compromise may avoid early repair with a conduit and tracheostomy with initial airway splinting. They are then able to undergo elective TOF repair without a conduit.