de Lange C: Imaging of complications following Fontan circulation in children - diagnosis and surveillance. Pediatr Radiol. 50(10):1333-48, 2020
Dillman JR et al: Imaging of Fontan-associated liver disease. Pediatr Radiol. 50(11):1528-41, 2020
Ibe DO et al: Pearls and pitfalls in pediatric Fontan operation imaging. Semin Ultrasound CT MR. 41(5):442-50, 2020
Udink Ten Cate FEA et al: Imaging the lymphatic system in Fontan patients. Circ Cardiovasc Imaging. 12(4):e008972, 2019
Files MD et al: Pathophysiology, adaptation, and imaging of the right ventricle in Fontan circulation. Am J Physiol Heart Circ Physiol. 315(6):H1779-88, 2018
Ginde S et al: Imaging adult patients with Fontan circulation. Curr Opin Cardiol. 32(5):521-8, 2017
Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
Burkhart HM et al: The Fontan operation: is timing everything? Semin Thorac Cardiovasc Surg. 27(2):175-6, 2015
Navarro-Aguilar V et al: Fontan procedure: imaging of normal post-surgical anatomy and the spectrum of cardiac and extracardiac complications. Clin Radiol. 70(3):295-303, 2015
Harris MA et al: Pre-Fontan cardiac magnetic resonance predicts post-Fontan length of stay and avoids ionizing radiation. J Thorac Cardiovasc Surg. 138(4):941-7, 2009
Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
Goo HW et al: Time-resolved three-dimensional contrast-enhanced magnetic resonance angiography in patients who have undergone a Fontan operation or bidirectional cavopulmonary connection: initial experience. J Magn Reson Imaging. 25(4):727-36, 2007
Rodríguez E et al: Postoperative imaging in cyanotic congenital heart diseases: part 1, normal findings. AJR Am J Roentgenol. 189(6):1353-60, 2007
Festa P et al: The role of magnetic resonance imaging in the evaluation of the functionally single ventricle before and after conversion to the Fontan circulation. Cardiol Young. 15 Suppl 3:51-6, 2005
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References
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KEY FACTS
Terminology
Imaging
TERMINOLOGY
Synonyms
Total cavopulmonary connection
Definitions
Goal is to direct systemic venous return to pulmonary circulation directly, bypassing right heart
Initially described by Dr. Francis Fontan in 1968
Used in single ventricle physiology
Tricuspid atresia
Hypoplastic left heart syndrome
Double inlet ventricle
Some heterotaxies
Stage 3 of Norwood procedure
Typically performed between 18-36 months of age
Originally described as extracardiac valved conduit between right atrium & left pulmonary artery
In combination with Glenn shunt, creates physiologic correction of blood flow
Superior vena cava (SVC) blood is directed to right pulmonary artery & inferior vena cava (IVC) blood is directed to left pulmonary artery
Complications of classic Fontan
Right atrial enlargement & hypertension
Impaired ventricular function
↓ pulmonary vascular blood flow
Tricuspid valve insufficiency
In order to preserve ventricular & pulmonary vascular function, modified Fontan is now favored
Lateral tunnel Fontan
Intraatrial tunnel created in right atrium using prosthetic material
IVC is anastomosed to caudal aspect of tunnel
Pulmonary artery is anastomosed to cephalad aspect of tunnel
Extracardiac conduit Fontan (typically favored)
IVC is divided from right atrium
Synthetic graft tube is connected to IVC inferiorly & right pulmonary artery superiorly; travels along side of right atrium
In either form, IVC blood directed to pulmonary circulation, bypassing right heart structures
Small fenestration is often created between Fontan circuit & right atrium
de Lange C: Imaging of complications following Fontan circulation in children - diagnosis and surveillance. Pediatr Radiol. 50(10):1333-48, 2020
Dillman JR et al: Imaging of Fontan-associated liver disease. Pediatr Radiol. 50(11):1528-41, 2020
Ibe DO et al: Pearls and pitfalls in pediatric Fontan operation imaging. Semin Ultrasound CT MR. 41(5):442-50, 2020
Udink Ten Cate FEA et al: Imaging the lymphatic system in Fontan patients. Circ Cardiovasc Imaging. 12(4):e008972, 2019
Files MD et al: Pathophysiology, adaptation, and imaging of the right ventricle in Fontan circulation. Am J Physiol Heart Circ Physiol. 315(6):H1779-88, 2018
Ginde S et al: Imaging adult patients with Fontan circulation. Curr Opin Cardiol. 32(5):521-8, 2017
Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
Burkhart HM et al: The Fontan operation: is timing everything? Semin Thorac Cardiovasc Surg. 27(2):175-6, 2015
Navarro-Aguilar V et al: Fontan procedure: imaging of normal post-surgical anatomy and the spectrum of cardiac and extracardiac complications. Clin Radiol. 70(3):295-303, 2015
Harris MA et al: Pre-Fontan cardiac magnetic resonance predicts post-Fontan length of stay and avoids ionizing radiation. J Thorac Cardiovasc Surg. 138(4):941-7, 2009
Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
Goo HW et al: Time-resolved three-dimensional contrast-enhanced magnetic resonance angiography in patients who have undergone a Fontan operation or bidirectional cavopulmonary connection: initial experience. J Magn Reson Imaging. 25(4):727-36, 2007
Rodríguez E et al: Postoperative imaging in cyanotic congenital heart diseases: part 1, normal findings. AJR Am J Roentgenol. 189(6):1353-60, 2007
Festa P et al: The role of magnetic resonance imaging in the evaluation of the functionally single ventricle before and after conversion to the Fontan circulation. Cardiol Young. 15 Suppl 3:51-6, 2005
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