Complete repair of Tetralogy of Fallot with absent pulmonary valve including the role of airway stenting

Tetralogy of Fallot (TOF) with absent pulmonary valve (APV) represents an extreme form of tetralogy where pulmonary insufficiency and mild annular stenosis often results in massive pulmonary arterial (PA) dilatation. The aneurysmal left and right PAs often compress the adjacent trachea and bronchi, leading to airway obstruction and respiratory failure in infancy. Between 1991 and 1997, 11 patients underwent a single stage repair of TOF and APV using a valved (10 patients) or nonvalved (1 patient) homograft conduit and PA reduction arterioplasty. There was one (1/11 [9.1%]) perioperative and one (9.1%) late death. Both deaths were related to airway complications. Morbidity associated with postoperative respiratory complications and ventilator-dependency due to underlying tracheobronchomalacia is an important problem. Intermediate follow-up shows a high incidence of reintervention for conduit stenosis and/or insufficiency and tracheobronchial compression. These infants also required multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Stenting of the right and left main bronchi with balloon expandable metallic stents is a new experimental therapy that has been useful in two recent patients with respiratory failure despite satisfactory intracardiac repair. It may provide an attractive alternative therapy to prolonged mechanical ventilation with positive end expiratory pressure in patients with severe tracheobronchomalacia. Complete repair with a valved homograft conduit and reduction pulmonary arterioplasty in infancy at the time of diagnosis is the procedure of choice for infants with TOF with APV. With this approach the patient outcome is essentially determined by their airway status and airway management.