Fontan Procedure

Fontan procedure is a palliative surgical procedure performed in childhood in patients with complex congenital heart disease where a biventricular repair is not feasible. These defects include but not limited to

  1. Hypoplastic left heart syndrome
  2. Tricuspid atresia
  3. Hypoplastic right heart syndrome
  4. Pulmonary atresia with intact ventricular septum
  5. Double inlet left ventricle
  6. Unbalanced atrioventricular canal not amenable for biventricular repair
  7. Double outlet right ventricle not amenable for biventricular repair

 

Surgery

Soon after a baby with hypoplastic ventricle is born, multiple surgeries done in a particular order are needed to increase blood flow to the body and bypass the poorly functioning side of the heart. The predominant ventricle becomes the main pumping chamber to the body. These surgeries do not cure this condition but helps restore heart function. Surgery is usually done in three separate stages:

  1. Norwood Procedure
    This surgery usually is done within the first 2 weeks of a baby’s life. Surgeons create a “new” aorta and connect it to the dominant ventricle. They also place a tube from either the aorta or the single ventricle to the vessels supplying the lungs (pulmonary arteries). Thus, the single ventricle can pump blood to both the lungs and the rest of the body. This can be a very challenging surgery. After this procedure, an infant’s skin still might look bluish because oxygen-rich and oxygen-poor blood still mix in the heart.
  2. Bi-directional Glenn Shunt Procedure
    This usually is performed when an infant is 4 to 6 months of age. This procedure creates a direct connection between the pulmonary artery and the vessel (the superior vena cava) returning oxygen-poor blood from the upper part of the body to the heart. This reduces the work the single ventricle has to do by allowing blood returning from the body to flow directly to the lungs.
  3. Fontan Procedure
    This procedure usually is done sometime during the period when an infant is 18 months to 3 years of age. Doctors connect the pulmonary artery and the vessel (the inferior vena cava) returning oxygen-poor blood from the lower part of the body to the heart, allowing the rest of the blood coming back from the body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart and an infant’s skin will no longer look bluish.

The Fontan circulation is unique as there is no pumping chamber, blood flows passively into the lungs resulting in increased venous pressure that overtime can result in pathological changes in the liver, gut, kindeys and lungs. Patients with Fontan procedures need lifelong care involving a team of specialists to address various problems that can result from this circulation. 

 

Symptoms

Symptoms of Fontan include the following:

  • Cyanosis
  • Shortness of breath, especially during physical activity.
  • Fatigue and weakness.
  • Rapid heart rate, arrhythmias or syncope.
  • Heart failure
  • Ascites

 

Diagnosis

Fontan patients need various diagnostic follow up:

  • Echocardiography (echo): This ultrasound test provides detailed images of the heart and is the primary tool for diagnosing Tetralogy of Fallot.
  • Electrocardiogram (ECG or EKG): This test records the electrical activity of the heart and may show signs of right ventricular hypertrophy.
  • Cardiac MRI or CT scan: These imaging tests can provide additional information about the heart’s anatomy and function.
  • Cardiac catheterization and EP studies.
  • Various tests for evaluation of liver and gut health.

 

When to consult a physician

Fontan patients should be monitored life long, even without symptoms.