The Fontan procedure is a repair surgical strategy for congenital cardiac anomalies. It is not usually used in isolation, but in combination with other repair procedures in a staged manner in an attempt to correct the underlying cardiac pathology.
The procedure attempts to bypass the right heart. Systemic circulation is redirected into the pulmonary arteries.
It can be used in multiple situations, including:
- tricuspid atresia
- hypoplastic left heart (part of the overall repair strategy)
- other types of single ventricle physiology
The original procedure involved:
- closure of atrial septal defect (ASD) and tricuspid valve
- creating an anastomosis between the right atrial appendage and the main pulmonary artery
To improve flow dynamics, a "lateral tunnel" was introduced. The patient essentially received a bidirectional Glenn procedure:
- the superior vena cava (SVC) is divided
- the proximal and distal ends are anastomosed to the right pulmonary artery
- an atrial "baffle" is put in the right atrium to direct blood toward the right pulmonary anastomosis and away from the tricuspid valve
Currently, an extracardiac conduit directs blood flow from the inferior vena cava (IVC) at the inferior cavoatrial junction to the right pulmonary artery, bypassing the right atrium. Systemic flow from the SVC is directed into the right pulmonary artery with a Glenn procedure.
Treatment and prognosis
Although a palliative procedure, patients treated with the Fontan procedure can reach adulthood. The maximum duration of the procedure is not well-defined.
A patient with a failing Fontan repair can receive an extracardiac Fontan conduit, if they do not already have one. The definitive procedure is cardiac transplantation.
Intracardiac and central venous thromboses are major causes of morbidity and mortality in patients after Fontan procedure. It can occur in 1%-33% of patients who underwent the procedure 4.
History and etymology
The procedure was originally suggested in 1971 by F Fontan and E Baudet as a correction for tricuspid atresia.
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.
congenital heart disease
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- conotruncal cardiac anomalies
- pentalogy of Cantrell
- Shone syndrome
- congenital heart disease - chest x-ray approach
surgical repairs (mnemonic)
- arterial switch procedure
- Blalock-Taussig shunt
- double switch procedure
- Fontan procedure
- Glenn procedure
- Mustard repair
- Norwood procedure
- Pott shunt
- pulmonary artery banding
- Rastelli procedure
- Sano shunt
- Senning repair
- total repair of tetralogy of Fallot (TOF)
- unifocalisation procedure
- Waterston shunt
- 1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26 (3): 240-8. Free text at pubmed - Pubmed citation
- 2. d'Udekem Y, Iyengar AJ, Cochrane AD et-al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation. 2007;116 (11_suppl): I157-64. doi:10.1161/CIRCULATIONAHA.106.676445 - Pubmed citation
- 3. Fuster V, Walsh R, Harrington R. Hurst's the heart. McGraw-Hill Professional. ISBN:0071636463. Read it at Google Books - Find it at Amazon
- 4. Ghadimi Mahani M, Agarwal PP, Rigsby CK et-al. CT for Assessment of Thrombosis and Pulmonary Embolism in Multiple Stages of Single-Ventricle Palliation: Challenges and Suggested Protocols. Radiographics. 2016;36 (5): 1273-84. doi:10.1148/rg.2016150233 - Pubmed citation
- 5. Fredenburg TB, Johnson TR, Cohen MD. The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics : a review publication of the Radiological Society of North America, Inc. 31 (2): 453-63. doi:10.1148/rg.312105027 - Pubmed
- 6. Deal BJ, Jacobs ML. Management of the failing Fontan circulation. Heart (British Cardiac Society). 98 (14): 1098-104. doi:10.1136/heartjnl-2011-301133 - Pubmed