Patients with absent pulmonary valve syndrome (APVS) and tetralogy of Fallot represent a particular management's challenge. The distinctive feature of APVS is the airway obstruction caused by tracheobronchial compression that results from massive dilatation of the main pulmonary artery (PA) and its first- and second-order branches. Consequential tracheomalacia and bronchomalacia determine the timing and severity of respiratory compromise, as well as the morbidity and mortality of these patients. Although several techniques have been proposed for decompression of the airways, the method of choice is still controversial. Translocation of the PA anterior to the aorta and away from the airways has the potential to reduce or eliminate bronchial compression. Overall, the intermediate-term functional outcomes using this technique have been encouraging, with zero mortality even in the youngest age group of patients and the disappearance of respiratory symptoms in the majority of them. Nevertheless, failure of the treatment could be expected in symptomatic patients in whom the pathology of the airways extends beyond the proximal PAs.
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