CTICasesUnrepaired Tetralogy of Fallot with Pulmonary Atresia, MAPCAs, and Collateral Thrombosis
Imaging findings
CT angiogram of an adult with unrepaired Tetralogy of Fallot and pulmonary atresia shows a right-sided aortic arch, a large ventricular septal defect, and multiple large, dilated major aortopulmonary collateral arteries supplying both lungs. A surgically placed systemic-to-pulmonary shunt is noted. A subsequent CT shows new saddle-like filling defects in multiple major aortopulmonary collateral arteries, representing acute thrombosis or embolization, which resolved on follow-up imaging after anticoagulation.
Key takeaways
Patients with Tetralogy of Fallot and pulmonary atresia rely on major aortopulmonary collateral arteries for pulmonary blood flow. These collateral vessels can develop stenosis, thrombosis, or embolization, leading to acute respiratory decompensation. Anticoagulation can successfully resolve thrombosis within collateral vessels and associated stents.
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