Utilizing the RAA Valve for the Repair of Tetralogy of Fallot With Pulmonary Atresia Following RVOT Stenting

Tetralogy of Fallot is the most common cyanotic congenital heart disease. In patients with a small pulmonary annulus, repair often requires a transannular patch, resulting in progressive pulmonary regurgitation and eventual right ventricular dilation and dysfunction.

To address these limitations, valve-sparing and right ventricular outflow tract reconstructive techniques are increasingly being used. Pulmonary valve reconstruction with right atrial appendage tissue is a novel approach that may provide improved valve competency and growth potential as a native autologous tissue.

In pulmonary atresia, the anatomy is more complex, with an absent or severely hypoplastic right ventricle–pulmonary artery connection. Even after neonatal right ventricular outflow tract (RVOT) stenting, the pulmonary valve region often remains small and nonfunctional.

The authors present the case of a six-month-old infant with tetralogy of Fallot who underwent complete repair using a right atrial appendage valve reconstruction in the pulmonary outflow tract. The patient had previously undergone neonatal RVOT stenting for pulmonary atresia with duct-dependent pulmonary circulation.

The operation was technically challenging due to near-complete RVOT obstruction, interference from the prior stent, complete loss of the native pulmonary valve, and severe hypoplasia of the pulmonary annulus and main pulmonary artery. Despite these complexities, the repair was completed successfully, resulting in a favorable immediate outcome.

References

1. Amirghofran A, Edraki F, Edraki M, Ajami G, Amoozgar H, Mohammadi H, Emaminia A, Ghasemzadeh B, Borzuee M, Peiravian F, Kheirandish Z, Mehdizadegan N, Sabri M, Cheriki S, Arabi H. Surgical repair of tetralogy of Fallot using autologous right atrial appendages: short- to mid-term results. Eur J Cardiothorac Surg. 2021 Apr 13;59(3):697-704.
2. Almousa A, Behrmann A, Miller P, Bhattacharya S, Nath D, Miller JR, Eghtesady P. Use of right atrial appendage tissue for pulmonary valve reconstruction in tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2025;28:60–67.

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