Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Abstract

The absent pulmonary valve syndrome occurs in 3–6 % of patients with tetralogy of Fallot. This syndrome is physiologically distinctive from other forms of tetralogy of Fallot, due to tracheobronchial compression resulting from massive dilatation of the main pulmonary artery and its first and second-order branches, and from the abnormal branching of segmental arteries. Symptomatic newborns and infants, in particular, have a poor prognosis because of severe central pulmonary artery dilatation and bronchial compression. Here, one-stage early primary repair is the method of choice. Apart from the correction of tetralogy of Fallot, surgery must address the dilated pulmonary arteries in order to minimize the incidence of postoperative and long-term bronchial compression and peripheral lung damage. The traditional approach has focused on plication and reduction of the anterior and/or posterior wall of the normally positioned pulmonary arteries. An alternative approach advocated by some groups involves translocation of the pulmonary artery anterior to the aorta away from the trachea and bronchial tree, which may reduce or eliminate bronchial compression by the pulmonary artery. Insertion of a valved homograft or other suitable conduit should be considered particularly in symptomatic newborns and infants. Failure of treatment may be expected in symptomatic patients, in whom the pathology of the airways extends beyond the proximal pulmonary arteries.