Chest X-ray demonstrates dilated central pulmonary arteries and normal peripheral markings . Lobar hyperinflation may also be seen. In addition to imaging the intracardiac anatomy with echocardiography , MRI may help identify aortopulmonary collaterals and the relationships of vessels to tracheobronchial structures. Tracheal intubation and positive-pressure ventilation with high PEEP may open the collapsed airways for preoperative stabilization. Prone positioning may also alleviate some of the obstruction.
Surgical repair involves plication of the dilated pulmonary arteries.
Longitudinal incisions are made in the arteries' anterior and posterior walls. Strips of redundant aneurysmal tissue are excised, and the incisions are closed. The pulmonary regurgitation is addressed with a monocusp or a homograft valve. Older patients may tolerate some pulmonary regurgitation without valve placement. The remaining elements of the TOF repair, the VSD closure and transannular patching of the RV outflow tract, are performed.