Tetralogy of Fallot with absent pulmonary valve syndrome and severe diffuse tracheobronchomalacia in an infant: the value of modified Lecompte manoeuver and reduction pulmonary arterioplasty

Abstract

An 8-week-old, 3.4-kg infant, who was diagnosed prenatally with tetralogy of Fallot and absent pulmonary valve syndrome, was intubated after birth and failed extubation due to severe tracheobronchomalacia. He was deemed inoperable prior to being transferred to our institution. The left pulmonary artery was severely aneurysmal to the point of occupying almost the entire left upper lobe. Standard tetralogy of Fallot repair was performed together with bilateral reduction pulmonary arterioplasties and a modified Lecompte manoeuvre. No interventions were done directly to the airways. In this video tutorial, we demonstrate the technique used and the outcomes.

Keywords: Absent pulmonary valve syndrome; Infant; Modified Lecompte Maneuver; Reduction Pulmonary Arterioplasty; Tetralogy of Fallot; Tracheobronchomalacia.