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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 directed to right pulmonary artery & inferior vena cava (IVC) blood 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 now favored
- Lateral tunnel Fontan
- Intraatrial tunnel created in right atrium using prosthetic material
- IVC anastomosed to caudal aspect of tunnel
- Pulmonary artery anastomosed to cephalad aspect of tunnel
- Extracardiac conduit Fontan (typically favored)
- IVC divided from right atrium
- Synthetic graft tube 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
- Lateral tunnel Fontan
- Small fenestration often created between Fontan circuit & right atrium
- Prevents volume overload to lungs
- Buffers any ↑ in systemic venous pressure
- Excellent early, mid, & late-term outcomes, with mortality rates < 5-10%
IMAGING
General Features
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
Selected References
- 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
Tables
Tables
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 directed to right pulmonary artery & inferior vena cava (IVC) blood 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 now favored
- Lateral tunnel Fontan
- Intraatrial tunnel created in right atrium using prosthetic material
- IVC anastomosed to caudal aspect of tunnel
- Pulmonary artery anastomosed to cephalad aspect of tunnel
- Extracardiac conduit Fontan (typically favored)
- IVC divided from right atrium
- Synthetic graft tube 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
- Lateral tunnel Fontan
- Small fenestration often created between Fontan circuit & right atrium
- Prevents volume overload to lungs
- Buffers any ↑ in systemic venous pressure
- Excellent early, mid, & late-term outcomes, with mortality rates < 5-10%
IMAGING
General Features
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
Selected References
- 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
