CTICasesCongenital Pulmonary Atresia with Glenn and Potts Shunts and RV-Dependent Coronary Circulation
Imaging findings
CT in a 20-year-old female with pulmonary atresia and tricuspid atresia shows a single ventricle configuration (large LV), a patent Glenn shunt, a right pulmonary artery stent, and a Potts shunt (anastomosis of the right/left pulmonary artery to the ascending aorta acting as a right-to-left shunt). It also shows transpleural venous collaterals and a dilated right coronary artery demonstrating a fistula with a blind-ending, high-pressure right ventricle (RV-dependent coronary circulation).
Key takeaways
Pulmonary atresia with intact ventricular septum and tricuspid atresia is palliated with a single ventricle pathway (Glenn and Fontan). High PA pressures can preclude Fontan, leaving the patient with a Glenn shunt and a Potts shunt (surgical right-to-left shunt to decompress the PA). The blind RV can develop extremely high pressures, leading to ventriculocoronary arterial fistulas and RV-dependent coronary circulation, where decompressing the RV can cause coronary steal.
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