CTICasesPentalogy of Fallot
Imaging findings
A 52-year-old patient presented with previously undiagnosed complex congenital heart disease. Cardiac CT demonstrated a right-sided aortic arch, a left-sided superior vena cava (SVC), a large perimembranous ventricular septal defect (VSD), an atrial septal defect (ASD), an overriding aorta, right ventricular hypertrophy, and supervalvular pulmonary stenosis with infovalvular narrowing. Additionally, numerous bronchial collaterals draining into the lungs were observed.
Key takeaways
Pentalogy of Fallot, comprising Tetralogy of Fallot (VSD, overriding aorta, RV hypertrophy, pulmonary stenosis) plus an ASD, can remain undiagnosed into adulthood. The severity of pulmonary stenosis can significantly impact the clinical presentation and longevity, with mild-to-moderate stenosis potentially balancing right-sided pressures and limiting shunting, allowing for prolonged survival without intervention. Coronotruncal rotational abnormalities are often associated with clockwise rotation of the aortic root.
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