Single Ventricle Care at Columbia
Most children with single ventricle defects undergo three surgeries during their first few years of life. Called the Norwood, Glenn, and Fontan procedures, these surgeries ultimately reroute the blood's circulation so that the ventricles have less work to do. Single ventricle defects vary but treatment is similar for all.
Because single ventricle defects are usually diagnosed before birth, your family will already be an integral part of your baby's team of providers as you prepare for surgery. You will participate in planning and help make sure your baby's first days are filled with the contact and care that newborns require.
Our heart surgeons, from the NewYork-Presbyterian Congenital Heart Center, are some of the best in the country. They routinely treat some of the most complex cases, including many patients referred to us from other medical centers, and we have highest survival rate in New York State for pediatric heart surgery and one of the highest in the United States. Children come to us from all over the world for our pioneering heart care.
Surgery for Single Ventricle Defects
You and your family will work closely with the team at the Center for Single Ventricle Care during the first years of your child's life as he or she undergoes a complex series of surgeries.
Your child's first surgery will take place about one week after birth. For most single ventricle defects, this surgery is called the Norwood procedure, an intervention that re-routes your baby's blood flow during the first months of his or her life to help preserve the health of the lungs and the heart. During this procedure, surgeons:
- Build a new aorta using a portion of the pulmonary artery
- Place a shunt to reroute blood from either the aorta or the right ventricle to the pulmonary artery, so that blood can flow to the lungs.
- Close the patent ductus arteriosus, a hole that connects the aorta to the pulmonary artery, and that normally closes after birth but may not in babies with congenital heart defects
- Open the atrial septal defect, a hole in the wall between the two upper chambers of the heart, to ensure that oxygenated blood can return to the right ventricle
Following this procedure, your baby will spend a month in the hospital with round-the-clock monitoring by the single ventricle team. Our Interstage Monitoring Program was developed with a specific focus on this vulnerable stage of treatment. During this month of monitoring, your role as a team member will expand, and we will train you so that you can continue monitoring your baby when he or she is able to go home.
When your baby is ready to go home, you will be well-prepared to take over your baby's care. Our nurses will call you weekly to check in on your baby's weight, oxygen levels, feeding and breathing, and mood. We will also schedule an appointment at two months to check on your baby in person.
At around the 100th day of your baby's life, we will perform non-invasive imaging tests to ensure that your baby is ready for the second stage of surgery.
Your baby's second surgery, the Glenn procedure, will take place sometime between four and six months. The goal of this surgery is to reroute blood flow from the upper body directly to your baby's lungs, so that it does not have to pass through the heart. Following the procedure your baby's ventricle will no longer pump blood to the lungs but will continue to pump blood to the body. During this procedure, your baby's surgeons:
- Disconnect the superior vena cava (which normally brings blood to the heart from the upper body) from the heart and connect it directly to the pulmonary artery and the lungs.
- Remove the shunt that was placed during the Norwood procedure. The shunt is no longer needed because blood is flowing directly to the pulmonary artery—and to the lungs—without first going through the heart.
Following the surgery, your baby will recover for one to two weeks in the hospital, with round-the-clock monitoring. At home, you will continue monitoring and regular check-ups with your baby's cardiologist.
Your child's third and final surgery will take place some time between 18 months and three years. The goal of this surgery is to direct blood flow from the lower body directly to your child's lungs, without passing through the heart. Your child's heart will only receive blood that has received oxygen from the lungs. This gives a single working ventricle one job: pumping oxygenated blood to the body.
During the Fontan procedure your baby's surgeons:
- Disconnect the inferior vena cava (which normally brings blood to the heart from the lower body) from the heart and connect it directly to the pulmonary artery, which brings blood to the lungs.
Following the surgery, your baby will recover for one to two weeks in the hospital, with round-the-clock monitoring. Your child will then continue regular cardiac care with your cardiologist, checking in with our center at ten years and as needed. We'll work closely with your child's cardiologist to transition care seamlessly.
Congenital heart defects require lifetime monitoring and your child will return for visits to our program throughout childhood. Eventually, we help our patients transition to the Schneeweiss Adult Congenital Heart Center, one of the oldest centers in the country for the care and treatment of congenital heart disease in adults.