Fontan circulation

Case contributed by Dr Adam Krajewski

Presentation

Shortness of breath and hypoxia. History of cardiac surgery.

Patient Data

Age: 25 years
Gender: Female
Nuclear medicine

Initial ventilation/perfusion scan performed with a right upper extremity IV line.

Ventilation images: Homogeneous distribution on a single breath and equilibrium images with no abnormal retention during the washout phase.

Perfusion images: Complete lack of perfusion to the left lung. No segmental perfusion defect in the right lung. 

X-ray

Cotemporaneous chest radiograph: Central vascular crowding and enlarged right pulmonary artery. Median sternotomy wires and left pulmonary artery stent are seen. A review of the patient's chart revealed that the left pulmonary artery stent was placed 21 years prior.

Nuclear medicine

Repeat ventilation/perfusion scan the same day

The same day a repeat ventilation/perfusion scan was performed with a right foot IV line.

Radiopharmaceutical was injected in the right foot.

Following the injection in the right foot, the perfusion images now show homogeneous activity in both lungs. No focal perfusion defects are present. The right lung activity is slightly more intense than the left lung consistent with the residual activity from the previous injection earlier in the day.

MRI

MRA of the chest

MRA of the chest performed one year prior illustrates a Fontan circulation

  • the superior vena cava is connected to the right pulmonary artery (right-sided cavopulmonary anastomosis)
  • the right-sided cavopulmonary anastomosis (SVC to RPA) allows a passive venous return to the pulmonary arteries
  • the extraatrial conduit connects the IVC to both the right and left pulmonary arteries
  • the extraatrial conduit allows flow from the IVC to both pulmonary arteries without the mechanical help of the right ventricle (bypassing the right ventricle)
  • Blooming artifact within the LPA related to left pulmonary artery stent

 

Annotated image

1. The superior vena cava is connected to the right pulmonary artery (right-sided cavopulmonary anastomosis).

2. The right-sided cavopulmonary anastomosis (SVC to RPA) allows a passive venous return to the pulmonary arteries. There is a preferential venous return to the right pulmonary artery (red arrow) due to venous admixture from the extraatrial conduit (blue arrows).

3. The extraatrial conduit connects the IVC to both the right and left pulmonary arteries. 

4. The extraatrial conduit allows flow from the IVC to both pulmonary arteries without the mechanical help of the right ventricle (bypassing the right ventricle). There is venous admixture to both the RPA and LPA (blue arrows).

5. Left pulmonary artery stent.

Terms:

  • IVC conduit: Inferior vena cava conduit (synonyms include external cardiac conduit or extraatrial conduit1)
  • SVC: superior vena cava
  • LPA: left pulmonary artery
  • RPA: right pulmonary artery

Case Discussion

Key teaching points:

  • Fontan circulation may mimick a pulmonary embolism on a perfusion scan.
  • Due to passive venous return to the pulmonary circulation, pedal injection during perfusion scan may reveal normal perfusion to both lungs.

Patient with a history of tricuspid atresia type IA status post right-sided cavopulmonary anastomosis, extracardiac Fontan, and left pulmonary artery stent placement during childhood. 

The initial VQ scan performed with a right upper extremity IV line demonstrates a complete lack of perfusion to the left lung and normal ventilation imaging. Unilateral flow to the right pulmonary artery may be seen, as in this case, mimicking a pulmonary embolism on perfusion scan.

Repeat perfusion images the same day following pedal injection shows homogeneous perfusion to both lungs.

Case prepared with the help of Dr Suzanne Parets and Dr Samuel Valle.

PlayAdd to Share

Case information

rID: 77248
Published: 9th May 2020
Last edited: 6th Oct 2020
System: Vascular, Cardiac
Inclusion in quiz mode: Included
Institution: Hartford Hospital