Fontan procedure
Updates to Article Attributes
The Fontan procedure is a repair strategy for congenital cardiac anomalies. It is not usually used in isolation, but combined with other repairs in a staged manner in an attempt to correct the underlying problem.
Rationale
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
Procedure
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.
History and etymology
The procedure was originally suggested in 1971 by F Fontan and E Baudet as a correction for tricuspid atresia.
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