Abstract
Truncus arteriosus is a cyanotic congenital heart defect where there is lack of wall development in the truncoconal root which impairs the creation of separate aortic and pulmonary valves, resulting in the single truncal valve. Other associated findings are common and include truncal valve anomaly (truncal valve regurgitation or stenosis) and aortic arch anomalies (right aortic arch, interrupted aortic arch, and hypoplastic aortic arch) and ventricular septal defects. A considerable number of truncus arteriosus patients have 22q11.2 deletions and may present with other clinical features of DiGeorge syndrome. In majority of the cases, the truncal valve is trileaflet. The diagnosis of truncus arteriosus is generally made by echocardiography. However, if echocardiographic evaluation is limited due to suboptimal acoustic windows or associated aortic arch interruption, cardiac CT imaging delineates aortic arch, pulmonary arteries, and coronary artery anatomy before surgical intervention. The outcome of truncus arteriosus without surgical repair is generally poor.
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Schoeneberg, L., Adebo, D.A. (2021). Truncus Arteriosus . In: Adebo, D.A. (eds) Pediatric Cardiac CT in Congenital Heart Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-74822-7_19
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DOI: https://doi.org/10.1007/978-3-030-74822-7_19
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