Interatrial Baffle Augmentation of Persistent Left Superior Vena Cava to Right Atrium

Persistent left superior vena cava is commonly associated with an unroofed coronary sinus and requires rerouting to the systemic right atrium during the repair of concurrent cardiac lesions [1]. The interatrial baffle was first described by Rastelli and associates at the Mayo Clinic, and has been the traditional approach to surgical correction of persistent left superior vena cava [2]. Our case highlights successful anastomosis between the left superior vena cava and the right atrium with concurrent closure of the secundum atrial septal defect.

This is a case presentation of a 21-month-old infant female with a history of Pentalogy of Cantrell consisting of: ectopia cordis, Tet-type double outlet right ventricle, two sub-aortic and inlet ventricular septal defects, a secundum atrial septal defect, left superior vena cava, and an unroofed coronary sinus. Due to the complexity of the condition, an intracardiac baffle technique was utilized to both shunt blood from the left superior vena cava to the right atrium in addition to closing the secundum atrial septal defect. Successful completion of the baffle patch would serve to direct deoxygenated venous flow from the head and neck to the right side of the heart as well as prevent mixing of blood at the atrial level of the heart to restore normal intracardiac physiology.

Upon opening the right atrium, several key structures are identified:  tricuspid valve, coronary sinus, both the superior and inferior vena cava, as well as the secundum atrial septal defect.  Importantly, the ostea of the left superior vena cava was visualize as it entered the left atrium high along its superior boarder, near the left atrial appendage.  .   Surgical patch material was brought to the surgical field and cut to create a 3-dimensional patch, used as both an intra-atrial baffling of the LSVC to the right atrium, as well as close the secundum ASD by folding it on itself. To achieve this, the tapered end of the  patch was anastomosed using 5-0 prolene sutures in a running continuous fashion to the osteal opening of the left superior vena cava in order to create the “floor of the baffling” as the left superior vena cava to the right atrium.  Prior to its completion, the baffle was probed to ensure  the native osteal opening of the LSVC was non-stenotic and unobstructed.  With the completion of this intra atrial shunt, attention was then drawn to the closing the remaining secundum atrial septal defect. The patch was then folded on itself to utilize a one-patch technique to close the atrial defect. The remaining portion of the patch (wide end) was sutured to the lateral rims of the secundum atrial septal defect to fully septate the left atrium from the right atrium.

Post-operative trans-esophageal echocardiogram indicated normal venous drainage without any gradient across the left superior vena cava baffle to right atrium.  Additional images show that the secundum atrial septal defect was fully closed without any obstruction of pulmonary venous return to the left side of the heart.  Post operative function and rhythm was preserved.  Cardiopulmonary bypass and cross clamp times were relatively short at 63 and 86 minutes.

When a persistent left superior vena cava is present without a bridging vein connection to the right superior vena cava, deoxygenated venous blood mixes within the left atrium.  To restore normal physiological flow, the surgical team is faced with several obstacles.  In this case report, surgical repair was achieved with a one patch intercardiac repair technique via interatrial baffling of the aberrant left superior vena cava to the right atrium, while also repairing the intra-atrial mixing associated with a secundum atrial septal defect. This technique offers several benefits including maximizing the use of native cardiac tissue, while avoiding the use of prosthetic conduits which are prone to thrombosis as well as stenosis as the child grows.

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1. Rudrappa, S.C., et al., Extracardiac Rerouting of Left Superior Vena Cava to Right Atrium. Seminars in Thoracic and Cardiovascular Surgery, 2019. 31(3): p. 571-572. 2. Zimand, S., et al., Left superior vena cava to the left atrium: do we have to change the traditional approach? The Annals of Thoracic Surgery, 1999. 68(5): p. 1869-1871.