CTICasesTetralogy of Fallot with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries (MAPCAs) in an Adult
Imaging findings
CT angiography in a 42-year-old demonstrates the classic features of tetralogy of Fallot with complete pulmonary atresia: overriding aorta, right ventricular hypertrophy, ventricular septal defect, and absence of a main pulmonary artery with numerous large major aortopulmonary collateral arteries (MAPCAs) supplying the pulmonary parenchyma bilaterally. A retro-aortic left brachiocephalic vein and right-sided aortic arch are additional incidental findings. The patient has survived uncorrected into adulthood.
Key takeaways
Tetralogy of Fallot with pulmonary atresia and MAPCAs is the most severe variant of TOF. MAPCAs arise from the descending aorta, intercostal arteries, or other systemic vessels and provide compensatory pulmonary blood supply, allowing survival into adulthood without surgical repair. The 22q11 deletion is associated with this severe form. Unrepaired adult cases are rare and eventually require heart-lung transplantation due to progressive pulmonary hypertension.
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