Surgical Repair of Mixed Supracardiac Total Anomalous Pulmonary Venous Connection

The surgical technique used in this video is suitable for patients with mixed supracardiac total anomalous pulmonary venous connection (TAPVC) in whom the right and left pulmonary venous systems drain through separate vertical veins and can be adequately mobilized for direct anastomosis to the left atrium

Operative Steps

The operation began with a median sternotomy and excision of thymic tissue. The surgical team harvested and prepared an autologous pericardial patch. When present, the patent ductus arteriosus is divided and ligated. Extensive dissection of both pulmonary venous confluences and vertical veins was performed, mobilizing the veins to the level of the bronchi. Cardiopulmonary bypass was established using aortic, innominate vein, and inferior vena cava cannulation. The patient was cooled to 22°C, and the heart was arrested with antegrade cardioplegia.

The ascending aorta and main pulmonary artery were transected to obtain clear exposure of the left atrial roof. The right pulmonary venous confluence was opened, and a wide anastomosis was performed to the left atrial roof. The left pulmonary venous confluence was then opened, and a second wide anastomosis was created on the left atrial roof, adjacent to the first. The right atrium was opened, the atrial septal rims were excised, and the atrial septum was reconstructed using a fenestrated autologous pericardial patch. Finally, the ascending aorta and main pulmonary artery were reconstructed, and the patient was successfully weaned from cardiopulmonary bypass.

Instruments and Materials

• Standard neonatal/infant cardiopulmonary bypass setup
• Del Nido cardioplegia
• Glutaraldehyde-treated autologous pericardial patch
• Fine vascular instruments for pulmonary venous dissection
• Polypropylene suture
• Polytetrafluoroethylene (PTFE) suture

Tips and Pitfalls

It is important to dissect the pulmonary veins extensively to the level of the bronchi to minimize tension and prevent kinking. The surgeons recommend marking the intended venotomy while the vein is distended to avoid twisting during anastomosis. Transection of the ascending aorta and the main pulmonary artery provides excellent exposure of the left atrial roof and facilitates the construction of a large anastomosis in an anatomic orientation. Taking care to mark the posterior aspect of the aorta and the pulmonary artery before transection helps avoid twisting during reconstruction. It is essential to protect the phrenic nerve throughout the procedure. Polypropylene or PTFE sutures may reduce the risk of delayed fibrosis-related injury.

Outcome

In the illustrative case, postoperative echocardiography demonstrated unobstructed flow from both pulmonary venous confluences into the left atrium with laminar Doppler flow. The patient had an uncomplicated recovery and was discharged home on postoperative day seven.

Disclaimer

The information and views presented on CTSNet.org represent the views of the authors and contributors of the material and not of CTSNet. Please review our full disclaimer page here.