Repair of Scimitar Syndrome in a Two-Year-Old Via VRAT: Pitfalls of Intra-atrial Baffle

The patient was a 2-year-old, 12.8-kilogram girl who had the typical features of Scimitar Syndrome (SS) with a partial anomalous pulmonary venous connection (TAPVC) and a hypoplastic right lung. She also had pulmonary hypertension and an atrial septal defect (ASD). She had previously undergone transcatheter coil occlusion of systemic aortopulmonary collaterals and device closure of a patent ductus arteriosus. A decision was made to proceed to the operating room for repair via a vertical right axillary thoracotomy (VRAT). 
 
Through the VRAT approach, a 6 cm incision was made in the mid-axillary line. The right chest was entered through the fifth intercostal space. The coiled collaterals were identified, and an additional aortopulmonary collateral was found, which was doubly clipped and divided. The pericardium was opened anterior to the phrenic nerve, and cardiopulmonary bypass (CPB) was initiated through central aortic and bicaval cannulation. An oblique right atriotomy was performed, and a large ASD was visualized. A 0.4 mmm Gore-Tex patch was then used to create an intra-atrial baffle, directing the venous return from the scimitar vein to the left atrium via the atrial septal defect using running 5-0 Prolene sutures. A 4 mm fenestration was made, given the patient’s known pulmonary hypertension. A bovine pericardial patch was then used to augment the inferior vena caval/right atrial junction, and it was sewn in place using running 5-0 Prolene sutures. After coming off CPB, a gradient across the vein was noticed, and CPB was re-initiated. A new pathway was created using a flap from the posterior pericardium to bridge the gap between the scimitar vein and the left atrium. A new bovine pericardial patch was used to create a roof for the scimitar vein/left atrial connection, which was sewn in using running 5-0 Prolene sutures.  

The surgeons were satisfied with the results, and the patient was weaned off CPB for a second time without difficulty.  
 
CPB time was 257 minutes, and cross-clamp time was 196 minutes. The patient was extubated in the operating room and discharged on postoperative day four. 
 
The VRAT technique provides more than cosmetic superiority; it offers good postoperative results, even in complex cases such as repairing SS. 

References

1. Said SM, Mashadi A, Essa Y, Erb M. Fontan Conversion in an Adult With Hypoplastic Left Heart Syndrome: A 38-Year-Old Norwood Success. JACC Case Rep. 2024;29(17):102495. Published 2024 Sep 4. doi:10.1016/j.jaccas.2024.102495
2. Said SM, Burkhart HM, Dearani JA. The fontan connections: past, present, and future. World J Pediatr Congenit Heart Surg. 2012;3(2):171-182. doi:10.1177/2150135111434806

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