An Optimized Rerouting Technique for Inferior PAPVC and Hypoplastic Aortic Arch Repair

This video is part of CTSNet’s 2025 Resident Video Competition. Watch all entries into the competition, including the winning videos.  

The authors present the case of a 4-month-old male infant weighing 2.8 kg who first presented with severe heart failure symptoms. Initial echocardiography revealed a tight coarctation segment and hypoplasia of the distal aortic arch. Cardiac computed tomography (CT) confirmed distal arch hypoplasia (z-score −4) and severe coarctation of the aorta.  

The imaging also demonstrated an anomalous pulmonary vein (PV) draining the entire right lung, descending in a craniocaudal direction to join the inferior vena cava (IVC) at an acute angle in a scimitar-like fashion, but without other features of scimitar syndrome such as sequestration or right lung hypoplasia. The hepatic veins, however, were draining in close proximity to the PV ostium. 

The multidisciplinary team (MDT) recommended immediate surgery to repair the hypoplastic aortic arch and coarctation, as well as rerouting the anomalous PV through the atrial septal defect (ASD) to the left atrium, ensuring unobstructed drainage of the PV, hepatic veins, and IVC. 

A standard median sternotomy was performed. The aorta was cannulated below the innominate artery, and bicaval venous cannulation was established. The aorta was extensively mobilized prior to cross-clamping. Following antegrade cardioplegia, a right atriotomy was performed, and the ostia of the PV, hepatic veins, and IVC were identified.  

The IVC cannula was removed for improved exposure, and the IVC was drained using a pump sucker. The right atriotomy was then extended anterior to the IVC cannulation site. The anomalous PV was identified from the right pleural side and marked with stay sutures. The right PV–atrial junction was incised, and the incision was extended toward the ASD on the atrial side. The medial aspect of the anomalous PV was also incised to create a wider orifice.  

The widened PV and atrial opening were then anastomosed, creating a broader ostium positioned closer to the left atrium and reducing the acute angulation of the anomalous PV. This approach eliminated the need to oversize the pericardial patch used to baffle the PV to the left atrium. After baffling the PV to the left atrium with an autologous pericardial patch, the IVC was augmented with another autologous pericardial patch, after which the IVC cannula was reinserted. A small ASD was intentionally left open for placement of a left ventricular (LV) vent until the completion of the aortic arch repair. 

Repair of the aortic arch required deep hypothermic circulatory arrest. Antegrade cerebral perfusion was achieved by redirecting the aortic cannula into the innominate artery and snaring all arch vessels. The patent ductus arteriosus (PDA) was divided, and the descending aorta was mobilized. The coarctation segment was resected, and the distal arch stump (distal to the left subclavian artery) was closed. The descending aorta was then anastomosed to the distal ascending aorta in an extended end-to-side fashion.  

The aortic cannula was redirected into the neo-arch, and the arch vessels were unsnared. The LV vent was removed, the residual ASD was closed, and the right atrium was closed. The patient was weaned smoothly from cardiopulmonary bypass and transferred to the intensive care unit (ICU), where he had an uncomplicated postoperative course. Postoperative CT demonstrated a uniform repair of the aortic arch, successful rerouting of the right PV to the left atrium, and unobstructed drainage of the IVC and hepatic veins. 

References

1. A Afifi, M Nagy, H Hosny, N Shehata, W Simry, Y Raef, W El Mozy, M Abou Gamrah, H Aguib, M Yacoub, P2789
2. A new technique to restore shape and pattern of flow in hypoplastic aortic arch, European Heart Journal, Volume 40, Issue Supplement_1, October 2019, ehz748.1104, https://doi.org/10.1093/eurheartj/ehz748.1104

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.