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.
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:
Staged reconstruction surgery to repair HLHS begins at birth and occurs in three staged operations:
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.
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.
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 catherization. Your child may return home a week or two after surgery.
Your child receives high-quality care throughout these staged reconstruction surgeries at Riley at IU health. We are one of only a few children’s hospitals nationwide to achieve a three-star rating from the Society of Thoracic Surgeons (STS) for our outcomes on these procedures. Our outcomes are better than the national average of children’s hospitals in the STS database – highlighting our commitment to providing the best care for your child. Learn more about our volumes & outcomes.
Our Interstage Home Monitoring Program is a convenient and accessible way for you 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:
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.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
A home-monitoring program for the littlest Riley Hospital heart patients is saving lives.Continue reading