Simultaneous complex single ventricle palliation and tracheoplasty for heterotaxy syndrome

Although isomerism of the bronchial tree is an integral part of hetrotaxy syndrome, the association of congenital tracheal stenosis is rare in this group of disorders, and it has not yet been thoroughly described in the literature. This condition is potentially life-threatening and precludes single ventricle palliation. This report presents the case of a 5-month old infant with symptomatic congenital tracheal stenosis, functionally univentricular heart and extracardiac total anomalous pulmonary venous connection (TAPVC). The condition was successfully treated with bidirectional Glenn anastomosis, central pulmonary artery plasty, repair of TAPVC and slide tracheoplasty.