Pulmonary atresia, double outlet right ventricle, ventricular septal defect, and complete absence of major pulmonary arteries were diagnosed in a 6 m/o infant by echocardiography and CT-angiography (Fig. 1a, b). Both lung hila were connected to the aorta through two separate ducti arteriosus. No large collateral existed, and the gap between the two lung hila was approximately 5 cm.
The first stage operation was performed to reconstruct the pulmonary arteries through a median sternotomy on normothermic beating heart cardio-pulmonary bypass.
To ensure that there are sufficient venous connections between the left and right upper body venous systems, the innominate vein was temporarily clamped for 5 min before cardio-pulmonary bypass. The venous pressure in the left side of clamping site was measured directly to check the increase not to exceed 5 mmHg which was considered as the significant pressure change. Then, both ducti arteriosus and their connection to the lung hila were dissected and exposed as well as the innominate vein in its entire course, and the hilar areas were cleared of any suspected residual ductal tissue. The innominate vein was transected at its connection to the left jugular and subclavian vein and connected to the left hilar opening with a wide anastomosis. Then it was transected in the right side at its junction with the superior vena cava, passed behind the ascending aorta, and anastomosed to the right hilum. A 5 mm shunt interposed between the ascending aorta and the conduit near the right hilum (Fig. 2a, b).
The cardio-pulmonary bypass was discontinued; pressures were measured again, which revealed a 5 mm difference between left and right venous systems.
The patient tolerated the procedure well with no facial or arm edema.
During the next 6 months, blood oxygen saturation gradually decreased. Echocardiographic and catheterization evaluations revealed shunt narrowing. Therefore, shunt stenting was performed, which resulted in an early increase in oxygen saturation. After 3 months, a shunt re-stenosis confirmed by CT angiography occurred. Besides, moderate stenosis of the innominate vein anastomosis to the right hilum was detected (Fig. 2a). Hence, considering all conditions, we decided to proceed to a total repair 9 months after the initial pulmonary artery reconstruction.
The operation was done through a median sternotomy on cardiopulmonary bypass and cardioplegic arrest. The quality of the innominate vein tissue was observed. The wall was thicker than the original, but the tissue was completely flexible, stretchable, and soft with no sign of fibrosis, calcification, or internal peeling formation. However, the internal lumen seemed to be relatively narrow despite elastic and stretchable wall tissue. The ventricular septal defect was closed after enlargement to overcome the severe overriding of the aorta, and an 18 mm Contegra graft (Medtronic Inc., Minneapolis, MN) was used to establish continuity between the right ventricle and the innominate vein. The Contegra graft inexorably crossed anteriorly towards the right side because the extremely large aorta was completely deviated to the left side.
The patient has generally been well since the final operation performed 4 years ago. A gradual, moderate increase in the gradient across pulmonary arteries was demonstrated in regular follow-up echocardiography. CT angiography confirmed a moderate narrowing of the distal anastomotic site of the Contegra graft on both sides. Consequently, bilateral stenting was performed after 3 years, followed by a re-dilatation 1 year later (Fig. 3). The patient is now in NYHA class I and good condition.