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 (TAPVC) is a simple yet challenging surgical procedure, especially when done in a neonate. The goal of the surgery is to create an unobstructed communication of the pulmonary veins to the left atrium. It is a standard, time tested, technique to treat this important congenital cardiac defect.

This video demonstrates a surgical repair of a cardiac type of TAPVC by unroofing the coronary sinus.

After standard cardiac anesthesia, median sternotomy is performed. The pericardium is opened and a patch is prepared for the atrial septal defect (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. The 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 atrioventricular (AV) groove and stay sutures are taken. The native interatrial septum is excised enlarging the ASD/ patent foramen ovale (PFO). The coronary sinus is usually 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 channeling the pulmonary venous return into the left atrium. In our practice, we usually perforate the patch to leave a patent foramen ovale (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. This is a simple and reproducible method to treat cardiac type total anomalous pulmonary venous connection draining into coronary sinus.

References

1. 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.

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