Treatment is surgical. Systemic-pulmonary anastomosis is indicated for patients with pulmonary arteries of adequate size. An open heart bypass from the right ventricle directly to the pulmonary artery using a non-valved conduit or aortic homograft has been utilised in some patients.
The pulmonary arteries must be of adequate size to allow a later operation to repair the VSD and place a conduit between the right ventricle and pulmonary artery.
In some cases there is stenosis or hypoplasia of the pulmonary artery and/or its main branches and reconstruction is difficult even after early systemic- pulmonary anastomotic procedures.
Last reviewed 01/2018