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 e xtreme form of tetralogy where pulmonary insufficiency and mild annular ste nosis often results in massive pulmonary arterial (PA) dilatation. The aneu rysmal left and right PAs often compress the adjacent trachea and bronchi, leading to airway obstruction and respiratory failure in infancy. Between 1 991 and 1997, 11 patients underwent a single stage repair of TOF and APV us ing a valved (10 patients) or nonvalved (1 patient) homograft conduit and P A reduction arterioplasty. There was one (1/11 [9.1%]) perioperative and on e (9.1%) late death. Both deaths were related to airway complications. Morb idity associated with postoperative respiratory complications and ventilato r-dependency due to underlying tracheobronchomalacia is an important proble m. Intermediate follow-up shows a high incidence of reintervention for cond uit stenosis and/or insufficiency and tracheobronchial compression. These i nfants also required multiple hospitalizations for recurrent respiratory in fections secondary to their tracheobronchomalacia. Stenting of the right an d left main bronchi with balloon expandable metallic stents is a new experi mental therapy that has been useful in two recent patients with respiratory failure despite satisfactory intracardiac repair. It may provide an attrac tive alternative therapy to prolonged mechanical ventilation with positive end expiratory pressure in patients with severe tracheobronchomalacia. Comp lete repair with a valved homograft conduit and reduction pulmonary arterio plasty in infancy at the time of diagnosis is the procedure of choice for i nfants with TOF with APV. With this approach the patient outcome is essenti ally determined by their airway status and airway management.