Fontan surgery - specialists and information

What is Fontan surgery?

Fontan surgery is a palliative surgical procedure for children with a congenital heart defect. It cannot cure the congenital heart disease, but it can improve the patient's condition.

During Fontan surgery, doctors adjust blood vessels so that they pump arterial and venous blood through the heart one after the other.

Several different variants have developed from the original Fontan operation. They are usually performed in two stages, relieve the strain on the heart and optimize blood flow.

Reasons for a Fontan operation

Fontan surgery is performed on patients who have a congenital heart disease and in whom only one ventricle is functional. This single ventricle must supply both the systemic circulation and the pulmonary circulation. This causes the blood from both circulatory systems to mix. As a result, the heart and lungs are considerably impaired and strained.

During the Fontan operation, the circulatory systems are separated from each other. The systemic venous blood from the systemic circulation reaches the lungs directly without passing through the heart. Here it is enriched with oxygen. The pulmonary venous blood reaches the atrium and from here the ventricle. The oxygen-rich blood is pumped into the systemic circulation. The aim of the operation is to provide the body with an almost normal supply of oxygen despite the presence of a ventricle.

The Fontan operation has only been around since the 1980s @ Pebo /AdobeStock

Procedure of the Fontan operation

Fontan surgery is performed in several steps:

1. the first step is to ensure the free inflow and outflow of blood to and from the heart. The Norwood operation, the Damus-Kaye-Stansel operation or the Giessen hybrid procedure can be used for this.

2 In the second step, doctors connect the superior vena cava directly to the pulmonary arteries. As a result, the blood from the upper half of the body no longer passes through the right heart. Doctors often use the Glenn anastomosis here if Fontan completion through an extracardiac tunnel is planned. The result of this step is to efficiently relieve the heart. The blood continues to mix. The volume in the heart drops to a normal level.

3 The third step is the complete separation of the circulatory system, which takes place when the child is between two and three years old.

Doctors also separate the inferior vena cava from the right ventricle and connect it to the pulmonary artery. In this way, they relocate and improve the supply to the lower half of the body. The pulmonary load increases and cardiac output decreases.

In the case of a hypotonic right heart, doctors use the remaining function. For this purpose, a surgical connection is made between the right atrium and the right ventricle. This procedure achieves a better distribution of the load and can increase the pumping capacity. Experts also call this method the ¾ heart or Fontan-Björk procedure.

The procedure just described represents the sequence of the various surgical interventions. However, there are other procedures for the individual steps that offer the best chances of success for the patient.

The decision as to which procedure is suitable for the patient is made by doctors after a thorough examination and taking all factors into account.

Fontan surgery is suitable for patients who only have one functionally effective ventricle @ alisaaa /AdobeStock

Which specialists perform Fontan surgery?

Trained and experienced cardiac surgeons perform Fontan surgery. Based on the initial situation, they determine which option is suitable to achieve the greatest success.

Aftercare following a Fontan operation

After the operation, the patient is admitted to the intensive care unit, where they are closely monitored and cared for by specialist medical staff.

The main aim is to wean the patient off artificial respiration so that the lungs can act as a suction organ to stabilize the circulation. For one child, this can be done in the operating room, while for others it takes longer.

Drainage helps to remove wound fluid and secretions. Nutrition can be given intravenously or via a gastric tube.

If the Fontan circulation is stable and there are no complications, the patient is discharged within a few weeks.

As the patient has been in hospital for a long time, initial difficulties in everyday life are to be expected. A fixed daily routine and regular check-ups are crucial. During the check-ups, doctors check the function of the heart and lungs as well as the healing of the surgical scar.

Patients can onlytake on more strenuous activities with the consent of their pediatrician or cardiac surgeon. Post-operative development, general healing and oxygen saturation are relevant here.

Special care and regular checks are necessary on an ongoing basis. Growth, puberty and any secondary illnesses can lead to new challenges and changes.

As Fontan surgery is a new procedure, it is not yet possible to estimate the long-term consequences. Nevertheless, the Fontan operation is a valuable method. Fontan surgery increases both the quality of life and the lifespan of the patient.

Complications, risks and prognosis

Possible treatment risks and complications during and after Fontan surgery include

• Protein-losing enteropathy
• effusions
• Rhythm disturbances
• cyanosis

Follow-up examinations are therefore important to detect and treat potential problems early.

Studies have shown that patients have an increased risk of liver damage. The prognosis is good in the short and medium term. However, sufficient information is still lacking for longer-term data.