Tetralogy of Fallot with Absent Pulmonary Valve and Bronchial Compression: Treatment with Endobronchial Stents

Abstract

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac lesion. The lesion includes ventricular septal defect, overriding aorta, and absence of the pulmonary valve, with resultant pulmonary incompetence. It has been suggested that the pulmonary incompetence induces intrauterine dilatation of the pulmonary artery, which leads to tracheobronchial compression. One of the presenting features in infants with APVS is severe airway obstruction, which may be difficult to manage. We report an infant who benefited from bilateral endobronchial endoscopic stent placement.

Pediatric bronchoscopy

“…145,146 Also, children with severe tracheobronchomalacia or obstructing malignancies have been successfully treated with temporary stents, 147 but serious questions remain as to their long-term safety, particularly when extrinsic compression of the airway by a vessel is to be relieved by the stent. 150 However, their use remains experimental at present and should only be used as a last resort. 150 However, their use remains experimental at present and should only be used as a last resort.…”

Successful stenting of congenital bronchial stenosis in infancy

“…The authors could ®nd only three previous reports of therapeutic stent placement in infants [1,5,9]. Two of them developed tracheobronchial stenosis as a complication of congenital heart anomalies which caused extrinsic vascular compression of the airway.…”

Bronchial compression due to stent placement in pulmonary artery in a child with congenital heart disease

“…In addition to this, the preexisting tracheomalacia may have been an important factor in the development of bronchial compression (4), as well as the structural deterioration from the multiple surgery performed in this patient. The options to resolve the bronchial stenosis were basically two: to place an endobronchial stent (2, 5, 6) or to remove the stents placed in the left pulmonary artery. It was decided to remove the stents because an endobronchial stent could have produced necrosis in the pulmonary artery by compression.…”