We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.

In the last 12 years, 21 patients age 1 month to 5 years (median, 7 months) underwent surgical treatment for severe airway obstruction associated with congenital heart disease. Plico-suspension of the pulmonary artery was carried out in 14 patients with documented severe left bronchial compression by a distended pulmonary artery together with repair of ventricular septal defects (11 patients), repair of large coronary artery fistula (1), division of ductus (1) and pulmonary artery banding (1), with subsequent significant relief of compression. Aortopexy was used in 2 patients with documented severe tracheal compression by a right aortic arch. Five patients had congenital tracheal stenoses (3 extensive, 2 localized). Two of 3 patients with associated complex intracardiac anomalies underwent concomitant repair of both lesions with the use of cardiopulmonary bypass and 1 had a tracheoplasty and pulmonary artery banding. Tracheal reconstruction with a cartilaginous graft together with repair of pulmonary artery sling was carried out in 2 infants. Nineteen patients are alive and well, at a mean follow-up of 87 months. In conclusion, we advocate early aggressive surgical intervention to both lesions to obtain a better result in the management of infants and small children with this difficult and often fatal combination.

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