Repair of Cardiac Total Anomalous Pulmonary Venous Connection by Unroofing the Coronary Sinus in a Neonate

Surgical repair of a cardiac type Total Anomalous Pulmonary venous connection is simple yet challenging surgical procedure, especially when it is done in a neonate. The goal of the surgery is creating an unobstructed communication of the pulmonary veins to the left atrium . It is a time tested , standard technique to treat this important congenital cardiac defect.[1,2,3]

This is a video demonstration of surgical repair of Cardiac type of TAPVC by unroofing the the coronary sinus.

After standard cardiac anesthesia, median sternotomy is performed. Pericardium is opened and a patch is prepared for ASD to be closed later. The great arteries are then dissected and delineated. The ductus arteriosus, often large, is then looped. Heparin is administered .Cardiopulmonary bypass is initiated with an aorto bicaval cannulation. Ductus arteriosus is then ligated. Patient is cooled to moderate hypothermia.. Antegrade Del Nido. cardioplegia is administered after clamping the aorta.

The heart is then arrested. Right atriotomy is done parallel to the AV groove and stay sutures are taken. The native interatrial septum is excised enlarging the ASD/ PFO. The coronary sinus is usual large. Pulmonary veins openings into the coronary sinus are identified. The roof of the coronary sinus is then excised , creating an unobstructed pathway from the pulmonary veins to the left atrium. Rewarming is started.The untreated autologous pericardial patch harvested earlier is used to close the extended ASD, thereby channelling the pulmonary venous return into the left atrium. In our practice, we usually perforate the patch to leave a PFO. The heart is de-aired and the cross clamp released .The patient is gradually weaned off cardiopulmonary bypass.

The results of this technique are excellent. [1,2,3] This a simple and reproducible method to treat cardiac type total anomalous pulmonary venous connection draining into coronary sinus.

References

Lupinetti, F., Kulik, T., Beekman, R., Crowley, D. and Bove, E., Correction of total anomalous pulmonary venous connection in infancy. The Journal of Thoracic and Cardiovascular Surgery. 1993;106(5):880-885.

2. Hawkins, J., Clark, E. and Doty, D. Total Anomalous Pulmonary Venous Connection. The Annals of Thoracic Surgery. 1983;36(5):548-560.

3. Shi, G., Zhu, Z., Chen, J., Ou, Y., Hong, H., Nie, Z., Zhang, H., Liu, X., Zheng, J., Sun, Q., Liu, J., Chen, H. and Zhuang, J.Total Anomalous Pulmonary Venous Connection. Circulation. 2017;135(1):48-58