Francis FONTAN
Francis M. Fontan a effectué la première opération de Fontan en 1968.
L'opération de Fontan est une opération du coeur employée pour
traiter des défauts de cardiopathies congénitale
(défauts cardiaques de naissance) comme l'atrésie tricuspide,
le syndrome gauche hypoplastic de coeur (HLHS),
l'atrésie pulmonaire et le ventricule unique.
Explication du Principe De Fontan
Nous sommes Habitués de penser que le coeur a quatre
chambres (deux oreillettes et deux ventricules). Ces quatre chambres
agissant dans l'unison et maintiennent la circulation du sang.
Pour comprendre la circulation de Fontan, vous devez imaginer
un coeur sans ventricule droit .
Comment le coeur peut-il fonctionner sans ventricule droit?
C'est exactement ce que Dr.Fontan a prouvé avec son opération.
Dans sa première intervention, il a relié l'oreillette droite
directement à l'artère pulmonaire, et a fermé l'ASD.
Le sang entrant des veines vers l'oreillette droite passe à
travers ce raccordement chirurgical dans l'artère pulmonaire et dans les poumons.
sans passer dans le ventricule droit.
Le sang n'est pas propulsé dans les poumons par le ventricule droit.
C'est vraiment ce qui rend ce procédé unique.
Normalement le ventricule droit fait office de pompe.
Dans cette situation le sang coule PASSIVEMENT dans les poumons pour s'oxygéner
(sans être propulsé par le ventricule droit).
Dans une circulation de type de Fontan, le ventricule gauche
pompe le sang dans l'aorte et les artères.
Ce sang coule d'abord
rapidement dans les différents organes. Cette force pousse le
sang à travers des capillaires, et les veines, mais avec un peu moins
force.
Lentement, le sang entre dans l'oreillette droite, et
passe à travers le raccordement chirurgical dans les poumons.
Francis FONTAN
The Fontan Principle
What is a Fontan-type circulation ?
Let me take some time to explain this, as it is an integral part of the entire operation for conditions like tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome ( HLHS ) and other single ventricle pathology.
We - you, me, cardiologists, surgeons, and everyone else - have been accustomed to thinking of the heart as having four chambers - two atria and two ventricles. These four chambers acting in unison maintain the circulation of blood.
To understand the Fontan circulation, you must make a "leap of imagination". In your mind, eliminate the right ventricle from the heart ! Tough isn't it ? And how can the heart possibly work without a right ventricle ?
Illogical as it may seem, this however was exactly what Dr.Fontan proved with his operation. In his original repair, he connected the right atrium directly to the pulmonary artery, and closed the ASD. Blood entering the right atrium from the veins passed across this surgical connection into the pulmonary artery and to the lungs. It completely bypassed the right ventricle.
Wait a minute ! There must be a flaw in this somewhere. How can the blood enter the lungs if it is not PUMPED IN by the right ventricle ? Well, that really is what makes this procedure unique. Normally the right ventricle will do the pumping. But in tricuspid atresia - and many other conditions in which a Fontan operation is performed - there is NO right ventricle. So blood flows PASSIVELY into the lungs - without being propelled into them by a right ventricle.
Why is lung blood flow so important ?
Because it is the only place in the body that blood can be purified by the addition of oxygen. So when lung blood flow is very low, oxygen supply is reduced to the entire body. This has many harmful effects, since no organ can perform its work normally without oxygen for energy.
So where does the energy for blood flow to the lungs come from ?
First, you must understand that any fluid flowing in a tube will continue to move, becoming slower and slower, until the resistance offered by the tube makes it stop. In a Fontan type circulation, the left ventricle pumps blood into the aorta and arteries. This blood flows at first rapidly into the different organs. The very same force pushes the blood across capillaries, and through the veins, but with lesser force. Slowly, blood enters the right atrium, and then passes across the surgical connection into the lungs - all the while unaided by a right ventricle.
But by its very nature, this flow depends on many factors. For instance, if the blood vessels in the lung are thick walled and narrow before surgery, they will offer very high resistance to passive blood flow. In such a state, the Fontan operation cannot be performed, or will have a high risk of failure, since the extra energy needed to maintain lung blood flow is not available.
Even normally a small amount of resistance will exist across the lung blood vessels. After a Fontan operation, the pressure in the veins will therfore be higher than normal, in order to overcome this resistance and maintain lung blood flow. The elevated pressure in the veins has a few ill effects.
First, there may be swelling of the entire body due to fluid from the blood leaking out of the vein walls.
There may be facial puffiness, fluid accumulation in the abdomen (ascites) or chest (pleural effusion).
Sometimes even absorption of nutrients from the intestines is affected.
source: Dr.Mani Sivasubramanian is a specialist children's heart surgeon and is Assistant Professor of Pediatric Cardiac Surgery at the Institute of Child Health and Hospital for Sick Children, India. English version: http://www.fontanoperation.com/fontan.htm