The classic method of repair, also known as the Glenn operation, is to anastomose the SVC and the right pulmonary artery. The SVC is disconnected at the entrance of the atrium, and the beginning part of the right pulmonary artery is cut off. The two ends are anastomosed. However, this procedure will result in uneven distribution of the blood flow and has been replaced by a modified technique called bidirectional cavopulmonary anastomosis. Without disconnecting the right pulmonary artery, the SVC blood is discharged into the bilateral pulmonary artery. For ill children with interruption of the IVC, blood from the IVC flows through the azygos vein and the SVC into the bilateral pulmonary artery.

In patients with tricuspid atresia, if the ventricle (RV) from which the pulmonary artery originates develops in a certain degree, the RA and the RV can be connected with an extracardiac conduit, and then the blood from the RA can drain into the pulmonary artery through the RV. If no pulmonary stenosis is present, right ventricular involvement may help with the pump function. The RV participating in the pumping function might bring some disadvantages. RV-to-RA regurgitation is a common occurrence, unless the extracardiac conduit is an implanted valve. However, the implanted valve will increase the ejection resistance of the RA, which one should consider when planning to apply this procedure (Figs. 16.1 and 16.4).

This surgical approach has been widely used, with increasing indications. Initially, a prosthetic valve was implanted at the opening of the IVC; the general consensus now is that the valve is not necessary. The RA can be seen as a valve-free chamber with systolic function, playing a pumping function between the vena cava and the pulmonary artery. With a well-developed RA, sinus rhythm, and low pulmonary resistance, this operation can result in a good hemodynamic state. On the other hand, an RA with a thin wall will directly affect the operative outcomes. Therefore, patient-selection criteria are sinus rhythm, normal vena cava, RA with normal capacity, mean pulmonary artery pressure <15 mmHg, normal left atrioventricular valve function, and ventricular ejection fraction >0.6 (Fig. 16.3).

The SVC is anastomosed to the pulmonary artery. The IVC is connected to the pulmonary artery through the conduit in the RA, which allows blood to flow more smoothly and results in more reasonable hemodynamics and simplified technique.