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Clarifying the Anatomy of Tetralogy of Fallot with S-shaped Ascending Aorta

Lookup NU author(s): Professor Bob Anderson

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Abstract

© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2024.We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta. Computed tomographic angiography was used for the assessment of cardiac morphology. Out of the 21 patients, 18 (86%) had a right aortic arch, 2 (9%) had a left aortic arch, and the remaining patient (5%) had a double aortic arch. Patients with a right aortic arch, compared to age and sex-matched patients with a right aortic arch but normally oriented ascending aorta, had lesser aortic override (29.3 ± 14% vs 54.8 ± 13.2%; p = 0.0001) and a wider ascending aorta (25.2 ± 6.9 vs 18.0 ± 3.2 mm; p = 0.0003). The S-shaped ascending aorta was located posteriorly, with a higher sterno-aortic distance (25.5 ± 7.7 vs 9.9 ± 4.5 mm; p = 0.0001). The ascending aorta among patients with tortuosity was longer (4.12 ± 1.7 vs 3.07 ± 0.82, p = 0.03) but with similar tortuosity index (1.22 ± 0.19 vs 1.15 ± 0.17, p = 0.23). Of the cases with right aortic arch and S-shaped ascending aorta, 16 (89%) had extrinsic compression of the right pulmonary artery (p = 0.0001), while 7 (39%) had crossed pulmonary arteries (p = 0.008), with no such findings among those with normally oriented ascending aorta. Tetralogy of Fallot with an S-shaped ascending aorta is a variant with lesser aortic override and a more posteriorly located ascending aorta. Compression of the right pulmonary artery and crossed pulmonary arteries is frequent in the presence of a right-sided aortic arch. These findings have important implications for optimal diagnosis and surgical repair. Graphical Abstract: (Figure presented.).

Publication metadata

Author(s): Gupta SK, Mukherjee A, Pandey NN, Ramakrishnan S, Kothari SS, Saxena A, Anderson RH

Publication type: Article

Publication status: Published

Journal: Pediatric Cardiology

Year: 2025

Volume: 46

Pages: 704-712

Print publication date: 01/03/2025

Online publication date: 22/04/2024

Acceptance date: 01/04/2024

ISSN (print): 0172-0643

ISSN (electronic): 1432-1971

Publisher: Springer

URL: https://doi.org/10.1007/s00246-024-03490-0

DOI: 10.1007/s00246-024-03490-0

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