Treatment

Medication and several procedures may be needed to treat your baby's pulmonary atresia with ventricular septal defect.

Procedures. Your baby may need one or more procedures to improve heart function and blood flow. There are several treatment pathways depending on the anatomy of the pulmonary arteries and the presence or absence of the MAPCAs.

• Stent placement. Placement of a rigid tube (stent) between the aorta and pulmonary artery (ductus arteriosus) allows blood to flow to your infant's lungs.
• Systemic-to-pulmonary artery shunt. This is a surgical procedure that may be needed in the first few days of life to increase blood flow to the lungs by creation of a connection (shunt) between one of the arteries and the pulmonary artery using a small tube of synthetic material. One example of such procedure is the Blalock-Taussig shunt (BT shunt).

Complete repair

• Neonatal complete repair. If your baby's heart has well-developed pulmonary arteries and no MAPCAs, your baby's surgeon can perform the complete repair in one stage during the neonatal period. This will include closure of the ventricular septal defect and placement of a valve conduit (tube) between the right ventricle and the pulmonary artery.
• One-stage complete repair. In this procedure, your baby's surgeon connects all the MAPCAs together to create new pulmonary arteries and then completes the repair with closure of the VSD and placement of the conduit. This is usually performed between 4 and 6 months of age. This procedure may also be called unifocalization.

Staged repair

• Staged unifocalization. If the MAPCAs are small or have multiple areas of narrowing, they may be connected in stages. This allows the arteries to grow to adequate size prior to the complete repair. The source of blood flow to the lungs will be provided through a small shunt from the aorta to the newly constructed pulmonary arteries. After a few months, your baby's heart will be evaluated with a cardiac catheterization and cardiac CT scan to determine the suitability and timing of the complete repair.
• Catheter interventions for the pulmonary artery branches. This procedure is done to assess the anatomy of your baby's heart and reconstruct pulmonary artery branches and dilate or stent the areas that have narrowing in them.