Norwood Procedure with Closure of an Aortic Left Atrial Appendage Fistula

This video shows a rare case of hypoplastic left heart syndrome with a large aortic root to left atrial appendage fistula in a baby born with aortic atresia, a hypoplastic aortic arch, and an aberrant right subclavian artery from the descending aorta. The preoperative echocardiogram and CT scan demonstrated the presence of a large fistula from the aortic root into the left atrial appendage, just above the course of the left coronary artery. The patient underwent a Norwood procedure, using moderate hypothermia at 26 degrees Celsius, selective cerebral perfusion, ligation of the fistula, and reconstruction of the aortic arch with a pulmonary homograft patch. A 5 mm reinforced Gore-Tex tube was used to connect the right ventricle with the right pulmonary artery. The patient had an uneventful post-operative recovery.

Abnormal congenital aorto-cardiac communications are a heterogeneous group of anomalies that provide an abnormal connection between the aorta and other cardiac chambers or structures, including the atria, ventricles, the main pulmonary artery, and the coronary sinus. They are also classified as aorta atrial tunnels (including aorta right and, less commonly, left atrial tunnels) with and without coronary artery take-off from the so-called tunnel.

Differentiation of the aorta-atrial tunnel from coronary cameral fistula can be challenging [1]. In this case, the preoperative echocardiography highlighted the presence of a tunnel in close proximity with the left coronary artery but also suggested a dilated coronary system and could not rule out communications between the two systems. The CT scan clearly identified a separate origin of the aorto-atrial tunnel from the origin of the left coronary artery, and accurate dissection of the aortic root and the left atrial appendage during the operation also confirmed the CT scan findings.
 

References

1. Rad EM, Hijazi ZM, Pouraliakbar H, Mirzaaghayan MR, Zamani H. Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification. Pediatr Cardiol. 2021 Oct;42(7):1459-1477. doi: 10.1007/s00246-021-02671-5.

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