CTICasesUncorrected Tetralogy of Fallot in an Adult with Major Aortopulmonary Collateral Arteries
Imaging findings
4D flow MRI demonstrates an overriding aorta, right ventricular hypertrophy, and a ventricular septal defect with aortic regurgitation directed toward the right ventricle. A very dysplastic, doming pulmonary valve is seen with significant stenosis. CT angiography confirms a severely stenotic pulmonary outflow tract and dysplastic pulmonary valve, and additionally demonstrates major aortopulmonary collateral arteries arising from the ascending aorta supplying the left hilum and from the descending aorta supplying the right pulmonary artery.
Key takeaways
In uncorrected tetralogy of Fallot surviving into adulthood, the stenotic pulmonary valve paradoxically may allow survival by increasing right ventricular pressure and maintaining preferential flow through the aorta while still providing enough pulmonary perfusion for gas exchange. Major aortopulmonary collateral arteries can develop even without complete pulmonary atresia, as seen here with only severe stenosis rather than atresia. Surgical correction with collateral takedown is planned in these cases.
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