Staged single ventricle reconstruction is a series of open-heart surgeries to repair single ventricle heart defects. These heart defects are detected in your child during pregnancy or after delivery. This type of surgery improves blood flow and circulation to your child’s lungs by creating a better pathway for blood flow throughout the chest.
What Happens During Staged Single Ventricle Reconstruction Heart Surgery?
What Happens During Staged Single Ventricle Reconstruction Heart Surgery?
Staged reconstruction surgeries are spread out over a number of years, or “stages” to repair an underdeveloped side of the heart and allow it to pump blood fully throughout the body. The most common – and most complex – condition treated with staged reconstruction surgery is hypoplastic left heart syndrome (HLHS), but others may include:
- Tricuspid atresia
- Double outlet left ventricle (DOLV)
- Heterotaxy defects
- Other congenital heart defects
Staged reconstruction surgery to repair HLHS begins at birth and occurs in three staged operations:
- the Norwood Procedure
- the Glenn or Hemi-Fontan Procedure
- the Fontan Procedure
Norwood Procedure
Norwood Procedure
The first operation, the Norwood Procedure, occurs a few days after your child is born. Since the left side of the heart isn’t pumping blood properly, the Norwood Procedure reconstructs parts of your child’s heart to transform the fully developed side into the main pumping chamber. The right side of your child’s heart pumps blood to both the lungs and the entire body, allowing for proper blood flow and breathing until the next staged operation. Your child returns home in three to four weeks with new medicines and possibly a feeding tube.
Glenn or Hemi-Fontan Procedure
Glenn or Hemi-Fontan Procedure
Referred to as either Glenn or Hemi-Fontan Procedure, the second staged reconstruction surgery occurs within four to eight months of your child’s birth, once the blood pressure in your child’s lungs has decreased. A cardiac catheterization procedure is usually performed prior to surgery to reevaluate blood flow in the heart and lungs. In this operation the extra work of the right side of the heart is reduced as surgeons reconnect the superior vena cava – the large vein that moves deoxygenated blood from the upper body to the heart–directly to the lungs. De-oxygenated blood flows directly to the lungs instead of going through the heart first. Your child returns home usually 1-2 weeks after surgery with new medications.
Fontan Procedure
Fontan Procedure
The final staged reconstructive surgery, the Fontan Procedure, occurs when your child is between 18 months to three years old. This operation is the final step in reconnecting the heart’s circulation as surgeons return the inferior vena cava – the large vein that moves deoxygenated blood from the lower body to the heart – from the heart to the pulmonary artery. This allows all deoxygenated blood to travel directly to the lungs. Sometimes a small hole is added near the right wall to act as a relief valve while the lungs get used to the new blood flow from the lower body. Small holes may close on their own; larger ones can be closed later using cardiac catheterization. Your child may return home a week or two after surgery.
How Will My Child be Monitored After Surgery?
How Will My Child be Monitored After Surgery?
Our Interstage Home Monitoring Program is a convenient and accessible way to monitor your child at home and remain in close contact with your child’s care team to ensure any issues are addressed quickly.
In this program:
- You take home a scale and pulse oximeter to measure your child’s progress
- You receive an iPad with helpful at-home monitoring information that provides updates to your child’s care team in real-time
- An on-call nurse practitioner is available to answer any questions and provide reliable medical or problem-solving advice for both urgent and non-emergent issues
- Our Parent Mentor Program pairs experienced, trained parents with families whose children are newly diagnosed with single ventricle conditions to offer parent-to-parent support
Since this program launched in 2010, the survival rate between the first and second single-ventricle heart surgeries has increased from 82% to 95%, with fewer children experiencing growth failure and more parents taking advantage of therapies to reduce developmental delays. Our successful program has been replicated at children’s hospitals throughout the country.
Key Points to Remember
Key Points to Remember
- These surgeries affect your child’s circulation system and so your child may appear slightly blue after the first two surgeries, especially when he or she cries. Talk with your doctor if you’re concerned about your child’s complexion.
- Your child will be monitored throughout these three staged surgeries to reduce the chance of risks related to any of the procedures.
- Your doctor will perform a number of diagnostic tests between surgeries and throughout childhood to ensure your child is progressing appropriately.
Support Services & Resources
Support Services & Resources
We offer a broad range of supportive services to make life better for families who choose us for their children's care.