Single Ventricle Roadmap

Children with single ventricle physiology must undergo a series of surgeries prior to undergoing the Fontan procedure. These children require lifelong cardiac care and can expect to experience multiple surgical and cardiac catheter-based procedures, recurrent hospitalizations, daily medications, and frequent outpatient follow-up. Our single ventricle team is uniquely equipped to help these patients and their families face these challenges.

The image below explains the path that many single ventricle patients may take. Ver esta información en espanól.

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Step 1: Aorto-pulmonary Shunts, Pulmonary Artery Banding or Stage 1 Norwood Procedure

Some babies with single ventricle physiology may require additional blood flow to the lungs. A surgical shunt procedure may be performed to direct blood from the aorta to the lungs. Other babies may have too much circulation to the lungs, and require a pulmonary artery banding procedure.

Patients with hypoplastic left heart syndrome require additional surgery to enlarge the aorta, which is done through a surgical operation called the Norwood procedure. The Norwood procedure is performed as soon as the baby is physically ready after birth. The heart typically works very hard after this surgery, and these babies require careful monitoring and feeding support.

Once patients are stable for discharge home, they are enrolled in our Interstage Home Monitoring (IHM) Program coordinated through the High Acuity Transition (HeArT) Clinic by a dedicated single ventricle nurse practitioner. In addition, all of our babies undergoing cardiac surgery receive follow-up evaluations in our Neurodevelopmental Outcomes Center under the direction of Raye-Ann deRegnier, MD, and Bradley S. Marino, MD.

Step 2: Glenn Procedure

This operation connects the superior vena cava, the blood vessel that carries deoxygenated blood from the head and neck back to the heart, directly to the pulmonary circulation system. This diverts half of the body’s deoxygenated “blue” blood directly into the lungs, bypassing the heart. If a prior surgical shunt was performed, this shunt is taken down.

The Glenn procedure is usually performed between three months and one year of age, depending on the health of the child. This surgery relieves part of the single ventricle’s volume and work load, but the child will still be considered “blue.”

Step 3: Fontan Procedure

The last step in the stages of Fontan surgeries connects the inferior vena cava, the blood vessel that carries deoxygenated blood from the lower body, to the pulmonary circulation. In our center, this is performed via an extracardiac Gore-Tex® tube graft, and is called the extracardiac Fontan operation. The procedure allows all the deoxygenated blood returning from the body to bypass the heart and go directly into the pulmonary circulation. After the oxygenated blood returns to the heart, the single ventricle then supplies the body with oxygenated blood, a function traditionally done by the left ventricle. After this procedure, the child will be “pink” and typically have oxygen saturation of more than 95%. Learn about Lurie Children's Fontan Conversion Program.

Physicians usually wait until a patient is a few years old or weighs about 30 pounds before performing the Fontan surgery. After recovering and being discharged from the hospital, patients initially have frequent follow-up appointments. These children require ongoing cardiac evaluations throughout life and may require additional procedures later in childhood or as adults.

The Fontan procedure has been performed since 1968 for “blue” babies with one functional ventricle, directing deoxygenated blood to the lungs, bypassing the heart and allowing oxygenated blood to be pumped by the single ventricle to the body. Originally designed for patients with a single left ventricle, the Fontan procedure has been used for patients with hypoplastic left heart syndrome since the early 1980s.