Hayes’ Story: Supravalvular Aortic Stenosis
For Dr. Salazar, saving sick kids is a way of life. “Caring for children with congenital heart disease is, for me, personal. It’s my life’s mission. I treat every child, every family, as if it were my own.”
Hayes appeared to be a perfectly healthy newborn. Three hours after birth, however, a nurse who was listening to his heart through a stethoscope heard an abnormal heart sound – a heart murmur – indicating a possible heart problem.
Diagnosed With Supravalvular Aortic Stenosis
A cardiologist ordered an echocardiogram (diagnostic cardiac ultrasound), which showed a severe narrowing (stenosis) of Hayes’ aorta, the large blood vessel that carries oxygen-rich blood from the heart to the rest of the body, and showed that the valve connecting his aorta to his heart (the aortic valve) was small and not opening properly. The condition was so severe that his heart was already showing signs of failure.
These were signs of a rare congenital (present at birth) heart disease known as supravalvular aortic stenosis (SVAS), which is caused by a mutation in the elastin gene (ELN). Supravalvular means “above the valves,” and in SVAS, the section of the aorta that is narrowed is located just above the aortic valve, impeding blood flow to the body. According to the National Institutes of Health, SVAS occurs in 1 in 20,000 newborns worldwide.
Life Flighted to Children’s Memorial Hermann Hospital
Hayes’ mother, Kortney, recalls how stunned and overwhelmed she and her husband, Jeremy, were, upon learning of Hayes’ condition. “We had no idea anything was wrong. Shortly after delivery, a nurse took him upstairs to the neonatal intensive care unit (NICU) and said she would bring him back in a few minutes. We waited and waited, and minutes became hours. The next morning, they asked us to come upstairs. That’s when we found out Hayes was born with heart problems.”
Jeremy adds, “They told us they were Life Flighting him to Children’s Memorial Hermann Hospital in the Texas Medical Center. It all happened so fast. We were terrified. But the cardiologist told us that Hayes would be in the care of an all-star from Boston Children’s Hospital, who had recently joined Children’s Memorial Hermann Hospital’s affiliated team as head of pediatric heart surgery. He assured us Hayes would be getting the absolute best care. That gave us some comfort.”
The diagnosing cardiologist referred Hayes to pediatric heart surgeon Jorge Salazar, MD, Professor and Chief of Pediatric Cardiovascular Surgery at McGovern Medical School at UTHealth and Co-director of the Children’s Heart Institute at Children’s Memorial Hermann Hospital. Dr. Salazar joined McGovern Medical School and the Children’s Heart Institute in September 2017 and specializes in the most complex, advanced pediatric heart procedures.
“A Double Whammy”
Using illustrations of a normal heart and a heart with SVAS, Dr. Salazar explained to Kortney and Jeremy that Hayes’ aorta was severely constricted. Whereas a newborn’s aorta typically measures 7 to 8 millimeters in diameter, Hayes’ aorta measured 1 to 2 ½ millimeters. In addition, there was a blockage above the aortic valve that restricted the blood flow into the coronary artery by about 90 percent.
“Coronary arteries are like the gas lines to the heart,” explains Dr. Salazar. “They allow the heart to have energy to pump. Without gasoline, your car will sputter and stop working. The heart is the same way. In this baby’s case, we were asking the heart to do a tremendous amount of extra work to try to pump through that severe blockage, and with a coronary obstruction. It’s what I call a ‘double whammy.’”
Successful Heart Repair Surgery
When Hayes was just four days old, Dr. Salazar and affiliated pediatric heart surgeon Robert Hanfland, MD, Assistant Professor of Pediatric Cardiovascular Surgery at McGovern Medical School, performed a six-hour procedure during which they opened up the area above the aortic valve, and in such a way as to also relieve the obstruction to the coronary artery. They used patches, made of tissue from Hayes’ pericardium (the sac surrounding the heart), to augment the area around the aortic valve. “We can normally accomplish this with one or two patches,” says Dr. Salazar. “But this patient required four.”
The surgery was a success. Two weeks later, Hayes developed a narrowing in a different part of the aorta, which they had been keeping an eye on from the beginning. Explains Dr. Salazar, “Early on, we told the parents that additional aortic stenosis could occur farther downstream, and we would keep a close eye on Hayes. Two weeks after his initial surgery, we noted a narrowing of his aorta, called a coarctation, toward the back. Catching it early enabled us to repair it through a small incision in the chest wall, called a thoracotomy, rather than having to do another big surgery.”
On January 4, 2018, Hayes went home and is doing well. After his surgery, genetic tests confirmed a diagnosis of Williams syndrome, a genetic condition characterized by medical problems, including cardiovascular conditions, such as SVAS, developmental delays and learning challenges. Approximately 15 percent of congenital heart defects are associated with genetic conditions.
Referral Center for the Most Challenging Pediatric Heart Cases
While Hayes’ case was serious and required complex surgical repair, Dr. Salazar says the Children’s Heart Institute sees the most critical cases that require even more complex procedures. “The [affiliated] team treats kids that many other centers shy away from. For instance, the Center achieves biventricular repairs for many children who traditionally receive a single ventricle repair or arrangement. What this means is that traditionally kids are treated as though they have half a heart; our team provides surgical treatment to give kids back a whole heart. The Children’s Heart Institute at Children’s Memorial Hermann Hospital is one of a very small number of pediatric heart institutes around the world that do this. Because we take on the most challenging cases, Children’s Memorial Hermann Hospital is a national referral site for fetal patients and neonates with congenital heart disease.”
Dr. Salazar credits the multidisciplinary team aspect of the Children’s Heart Institute for the team’s ability to take on the toughest cases. “To be able to have a successful outcome for these highest-risk patients, you have to have a team that's set up to do the most complex repairs in newborns. Newborn surgery is the most demanding of all the surgeries that we do, because the children are so fragile. Our team specializes in performing full repairs up front, even for the most complex babies. For these types of children, many centers perform them in multiple steps. We’ve learned that the babies do better in the long term if we can give them a full repair, up front. The success rate is very high with the one-step complete repair.”
And for that, Kortney and Jeremy are extremely grateful. Says Jeremy, “We are glad we ended up where we did, in Dr. Salazar’s steady hands. And we are thankful for the great support system at Children’s Memorial Hermann Hospital. But I have to say, we’re happy to be on the other side of it.”
Hayes will likely be a patient of the Children’s Heart Institute for life. “Children with congenital heart disease require lifetime care, which the Center provides. These families become a part of our family, and we become a part of theirs,” says Dr. Salazar.
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i The Children’s Heart Institute is a collaboration between Children’s Memorial Hermann Hospital and the affiliated physicians at McGovern Medical School at UTHealth. Typically, patients are seen on an outpatient basis at a UT Physicians Clinic, with all inpatient procedures performed at Children’s Memorial Hermann Hospital.
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