Fontan circulation

Case contributed by Adam Krajewski , 9 May 2020
Diagnosis certain
Changed by Joachim Feger, 10 May 2020

Updates to Study Attributes

Caption was added:
Initial ventilation/perfusion scan
Findings was changed:

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. 

Updates to Study Attributes

Findings was changed:

Left pulmonary artery stent and median sternotomy wires are noted. There is central

Central vascular crowding. 

Caption was added:
Chest x-ray after pulmonary stent artery placement

Updates to Study Attributes

Caption was added:
Ventilation/perfusion scan after left pulmonary artery stent placement

Updates to Study Attributes

Caption was added:

Updates to Case Attributes

Body was changed:

History of tricuspid atresia type IA status post right-sided cavopulmonary anastomosis, extracardiac Fontan, and left pulmonary artery stent placement. 

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. The right-sided cavopulmonary anastomosis allows a passive venous return to the pulmonary arteries. Unilateral flow to the right pulmonary artery may be seen, as in this case, mimicking a pulmonary embolism on perfusion scan. The extracardiac conduit allows flow from the IVC to both pulmonary arteries without the mechanical help of the right ventricle. Repeat perfusion images following pedal injection showed homogeneous perfusion to both lungs.

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

  • -<p>History of <a title="Tricuspid atresia" href="/articles/tricuspid-atresia">tricuspid atresia</a> type IA status post right-sided cavopulmonary anastomosis, extracardiac <a title="Fontan procedure" href="/articles/fontan-procedure">Fontan</a>, and left pulmonary artery stent placement. </p><p>The initial <a title="VQ scan" href="/articles/vq-scan-2">VQ scan</a> performed with a right upper extremity IV line demonstrates a complete lack of perfusion to the left lung and normal ventilation imaging. The right-sided cavopulmonary anastomosis allows passive venous return to the pulmonary arteries. Unilateral flow to the right <a title="Pulmonary artery" href="/articles/pulmonary-trunk">pulmonary artery</a> may be seen, as in this case, mimicking a pulmonary embolism on perfusion scan. The extracardiac conduit allows flow from the IVC to both pulmonary arteries without the mechanical help of the right ventricle. Repeat perfusion images following pedal injection showed homogeneous perfusion to both lungs.</p><p>Case prepared with the help of Dr. Suzanne Parets and Dr. Samuel Valle.</p>
  • +<p>History of <a href="/articles/tricuspid-atresia">tricuspid atresia</a> type IA status post right-sided cavopulmonary anastomosis, extracardiac <a href="/articles/fontan-procedure">Fontan</a>, and left pulmonary artery stent placement. </p><p>The initial <a href="/articles/vq-scan-2">VQ scan</a> performed with a right upper extremity IV line demonstrates a complete lack of perfusion to the left lung and normal ventilation imaging. The right-sided cavopulmonary anastomosis allows a passive venous return to the pulmonary arteries. Unilateral flow to the right <a href="/articles/pulmonary-trunk">pulmonary artery</a> may be seen, as in this case, mimicking a pulmonary embolism on perfusion scan. The extracardiac conduit allows flow from the IVC to both pulmonary arteries without the mechanical help of the right ventricle. Repeat perfusion images following pedal injection showed homogeneous perfusion to both lungs.</p><p>Case prepared with the help of Dr Suzanne Parets and Dr Samuel Valle.</p>

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