Fontan circulation (CT)

Case contributed by Mujeeb Khader
Diagnosis almost certain


Complex congenital heart disease: congenitally corrected L-TGA, small left ventricle, hypoplastic left heart syndrome (HLHS) - aortic arch hypoplasia. Status post total cavopulmonary anastomosis (TCPC) and stenting of TCPC-LPA

Patient Data

Age: 10 years
Gender: Male

Fontan Circulation

  • side by side great vessels with Damus-Kaye-Stansel (DKS) anastomosis, no coarctation, the original aorta is to the neo-aortic valve and main pulmonary artery
  • normal pulmonary venous return excessive and congested
  • smallish left pulmonary artery, with a long segment
  • Fontan circuit return to the pulmonary artery, no significant narrowing seen, fenestration stent in place.
  • multiple major aortopulmonary collateral arteries (MAPCAs) from descending aorta to both sides of the lungs mainly to the right lung, possible to the pulmonary venous system
  • superior vena cava returns to the pulmonary artery (after previous Glenn procedure)
  • hepatic vein and inferior vena cava join the Fontan conduit
  • dilated inferior vena cava and hepatic veins

Case Discussion

An example of a Fontan circulation in a patient with a Damus-Kaye-Stansel anastomosis for complex congenital heart disease with L-TGA and hypoplastic left heart syndrome (HLHS).

Simultaneous injection of iodinated contrast by proper selection of injection sites (through upper and lower extremities) or dual injection protocol method providing timing of contrast administration, and initiation of scanning which allows denser opacification of the entire Fontan circuit which successfully optimize and uniform simultaneous contrast enhancement of the Fontan pathway and pulmonary arteries.

Differential timing of opacification of the superior vena cava (SVC) and inferior vena cava (IVC), incomplete mixing in the Fontan circuit, and differential streaming of contrast into pulmonary arteries result in inhomogeneous opacification of the Fontan pathway.

Difficulties in enhancing both the pulmonary arteries and the aorta in the same phase in the setting of Total Cavo Pulmonary Connection (TCPC) with possible swirling artifacts, un-opacified hepatic venous inflow, or incomplete mixing. Another option is delayed scanning when the venous blood returns to the Fontan pathway following systemic circulation.

In patients with an atriopulmonary Fontan connection, significant ventricular dysfunction, or severe atrioventricular valve regurgitation, due to slow circulation, a one-minute-delayed scan (60 or 90 sec) usually provides adequate contrast opacification of the intrathoracic vasculature with only minor inhomogeneity with clearly visualized lateral tunnel between Fontan conduit and right atrium.


Special thanks and appreciation is extended to Dr Ajay Prashanth Dsouza (Consultant Radiologist and HOD) Dr Muhammad Anwar (Consultant Radiologist), Dr Mohamed Sulaiman (Pediatric Cardiologist), Jukha Shater Ali Ali AL Badawi (Radiology Manager), Diagnostic Imaging Department, Al Jalila Children's Specialty Hospital, Dubai, UAE for contributing this case.

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