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Fontan’s Operation

Fontan’s operation is a palliative surgical procedure used to treat congenital heart defects known as single ventricle anomalies. This term is used to describe a group of cardiac defects that differ from each other, but share the common feature that only one of the two ventricles is of adequate size to function normally. Some of the anomalies include tricuspid atresia, hypoplastic right heart syndrome, and double inlet left ventricle. All of these defects will generally undergo staged reconstructive procedures ultimately resulting in a “Fontan circulation.”

In the normal heart, each ventricle (lower chamber of the heart) does a separate job. The right ventricle pumps blood to the lungs, and the left ventricle pumps blood to the body. In a single ventricle heart, there is only one ventricle large enough to do the normal job of pumping the blood. This requires assigning the single ventricle to do the harder work of the heart which is to pump blood to the body. The job of getting blood to the lungs must be done without a pump. Different procedures may be necessary to achieve this goal depending on each patient and their needs.

The type of operation needed is quite varied depending on the cardiac defect. Generally, it takes three stages to achieve Fontan circulation. The first stage, usually performed in the first weeks of life, involves regulating the amount of blood flow to the lungs. If blood flow to the lungs is excessive, then a band can be placed around the pulmonary artery (the main lung vessel) to control the flow and protect the lungs from high pressure. This procedure is called pulmonary artery (PA) banding. If there is an inadequate supply of blood to the lungs, then a Blalock-Taussig shunt procedure would take place. This involves using a patient’s blood vessel or a shunt made from synthetic material and connecting it between the subclavian (a branch of the aorta) and the pulmonary artery.

The next stage is performed around 4-12 months of age. During the Glenn operation (hemi-Fontan), the superior vena cava (vessel that drains blood from the head and upper body back to the heart) is removed from the heart and sewn to the pulmonary artery. The shunt and band placed in a previous procedure may be removed at this time.

The third and final stage is the Fontan completion operation. This procedure is usually done around 2-3 years of age. During the operation, the inferior vena cava (vessel that returns blood to the heart from the lower part of the body) is connected to the pulmonary artery. Until now, this blood has bypassed the lungs and has been pumped to the body resulting in oxygen levels lower than normal. The two most common methods of the Fontan operation are the lateral tunnel and extra-cardiac technique. In the lateral tunnel method, a tunnel-like patch is placed inside the right atrium. It is then attached to the pulmonary artery directing the blood returning from the inferior vena cava. In the extra-cardiac method, the inferior vena cava is connected to a synthetic tube and is sewn to the pulmonary artery, routing the blood flow outside the heart.

Preparation
  • The patient will be prepared for physical cleanliness, and refrain from wearing any metallic ornaments, holy threads and nail lacquer.
  • Stop some medications at least 7-10 days before operation such as aspirin or some heart medicines which will be instructed by the physician.
  • Inform the physician if the patient or siblings have a record of easy bleeding, difficult blood clotting or bruise on the body.
  • The operation should be pending if the patient has a cold or fever.
  • If the patient has tooth decay, please see the dentist before undergoing the operation to avoid the risk of respiratory and heart infections. Small children and children with cyanosis are excluded as they might encounter complications during dental treatment.
  • The patient should refrain from food and drink according to the anesthetist’s scheme.
After surgery, the patient will be in an intensive care unit. A number of systems are used to monitor the patient's heart rate and rhythm, blood pressure, temperature, and breathing rate. Monitoring is gradually discontinued as the person recovers. Before leaving the hospital, the family will be instructed on how to care for their child at home. This includes information on medications, caring for the incision, and activity limitations.

The patient will require continuing care. The doctor will schedule routine check-ups to make sure the procedure was successful and to monitor any new problems. The Fontan operation is palliative, not curative. The limitations children experience due to their heart defect can vary greatly. There are children with Fontan circulations who have participated in competitive sports. Other children have significant limitations in regards to their exercise capacity. Most children fall somewhere in the middle of these extremes. Many cases result in normal or near-normal growth, development, exercise tolerance, and good quality of life.

Let the doctor know if the patient develops problems, such as:
  • Fever
  • Redness, swelling, or bleeding from the incision
  • Rapid breathing
  • Excessive fatigue or tiredness
  • Decreased appetite or refusal to eat
  • Increased pain
The risks for Fontan’s operation include the following:
  • Bleeding
  • Infection
  • Arrhythmias (irregular heartbeat)
  • Adverse reaction to the medication
  • Heart failure
  • Blood clots causing stroke or pulmonary embolism
  • Pleural (fluid around the lungs) and pericardial (fluid around the heart) effusions
  • Death
Before the procedure
  • You should plan to stay in Thailand for at least 2-3 weeks through the duration of your treatment.
  • It is recommended that you stay in a hotel close to the hospital for convenience in traveling to the hospital before and after the procedure or from the day of the procedure to the day of your follow-up appointment.
After the Procedure
  • At your follow-up appointment you will undergo a physical examination and your wound will be checked. You will receive documentation regarding your surgery or procedure and all other relevant documentation for traveling.
  • Clean the incision site by bathing and using soap daily. Pat the incision dry. Do not apply any cream, lotion, or powder to the site. Please contact your doctor immediately if you notice any signs of infection.
  • When traveling by air, if you are seated in Economy Class, please choose an exit row or bulkhead seat for convenience in getting up and moving around every 15-30 minutes. Flex your ankles regularly to prevent deep vein thrombosis.
  • Please take all medication prescribed by your doctor. Carry the appropriate dosage of mediation in your carry-on luggage when you travel as well as a few extra doses in case of an emergency. Carry the prescription for all your medication to avoid problems at the airport.
The success of the procedure depends on a number of factors and post-procedure care. Please discuss the likelihood of success with your doctor before the procedure.
 
What if this procedure is not performed?
Fontan’s operation is used to improve the blood and oxygen flow to the heart. Without surgical intervention, the patient will not survive.
 
Medications and other palliative procedures until the Fontan’s operation can be performed.

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