CTICasesTetralogy of Fallot with Pulmonary Atresia ("Super Tet") and Right Aortic Arch
Imaging findings
In a two-week-old with known congenital heart disease, cardiac CT shows a right aortic arch with mirror-image branching and a possible pseudo-bovine variant, complete atresia of the proximal pulmonary artery, and a large ventricular septal defect with a single great vessel arising across both ventricles. A large ductus arteriosus supplies both pulmonary arteries, coursing between the descending aorta and the airway; the right mainstem bronchus is mildly compressed between the ductus and the descending aorta, and a small posterior diverticulum off the proximal right mainstem bronchus is noted, favored to represent a cardiac bronchus.
Key takeaways
Complete pulmonary artery atresia with a VSD and ductal supply to both pulmonary arteries represents an extreme form of tetralogy of Fallot physiology ("super Tet"), and the ductus arteriosus must be kept patent until surgical repair (VSD closure with an RV-to-pulmonary-trunk conduit); an incidental posterior bronchial diverticulum in this location most likely represents a blind-ending cardiac bronchus.
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