Noah's Hearty Now

By Ridgely Ingersoll 

A human heart is a marvel of engineering elegance, but not every baby is fortunate enough to be born with a healthy one.

In fact, nine out of every 1,000 newborns have a heart defect.

Pediatric urologist solves the puzzle of her on-again-off again tummy pains.
CHKD heart specialists team up to fix his heart defect in the knick of time.

Three years ago, one of them was Noah Hoffmann, the first child of David and Laura Hoffmann of Gloucester.

Noah’s pediatrician, Barbara Allison-Bryan, detected a heart murmur at one of Noah’s first checkups. While many heart murmurs are “innocent” and don’t signify problems, others are more worrisome. Noah’s was the latter, so his doctor referred him to the pediatric cardiology specialists at CHKD.

Noah made his first trip to CHKD’s Health and Surgery Center at Oyster Point when he was just three weeks old. There, cardiologist Charles Bullaboy did several tests, including an echocardiogram to evaluate Noah’s heart. The test revealed that his aorta, the artery that carries oxygenated blood from the heart to the rest of the body, was too narrow, a condition called aortic stenosis.

Most cases of aortic stenosis occur because the valve between the left ventricle of the heart and the aorta is malformed and doesn’t open completely. Noah’s condition was unusual because the narrowing was in the aorta itself, just above the valve.

“We were so surprised to learn that he had a heart defect that we couldn’t really take in anything they told us after that,” Laura remembers. “At home we looked up ‘aortic stenosis’ online and found a lot of very scary information,” she continued. A few days later, they had a heart-to-heart with Dr. Bullaboy. “He made us feel so much better,” Laura said. “He said, ‘I am here for you, and we are just going to take it one step at a time.’ He told us Noah might need surgery someday, but in the meantime we shouldn’t hold him back from anything.”

Cardiologists rate the severity of aortic stenosis from trivial-to-mild to moderate-to-severe. “At birth, Noah’s case was trivial-to-mild,” Dr. Bullaboy said, “but we watch children with this condition very closely during the first two years. When there is an abnormality in that artery, it may not grow properly with the child. Even the tiniest increase in severity of the stenosis can make a big difference since the artery is small to begin with.”

Noah flourished and grew normally – at one point hitting the 97th percentile on his pediatrician’s growth chart. “Noah’s personality is very outgoing, active and helpful,” his mom says. “We couldn’t have slowed him down, even if Dr. Bullaboy had told us to!”

For two years, Noah saw Dr. Bullaboy at CHKD’s outpatient center in Newport News every three to four months. Though his condition worsened slightly, it wasn’t enough to warrant a change in treatment – until last summer.

“In August, we went in thinking it would be like every other checkup,” Laura said. “But after Noah had his echocardiogram, Dr. Bullaboy told us he was concerned. He wanted Noah to see one of his partners who could do a cardiac catheterization to give them a better look at his heart.”

Riley and her mom, Cheryl.
Noah doesn’t miss a beat now.

The Hoffmanns met with CHKD cardiologist Michael Vance to discuss the catheterization, which they learned would be in CHKD’s Heart Catheterization Lab while Noah was under general anesthesia. “We wanted to know everything we could beforehand,” Laura says. “Dr. Vance was a great teacher. He said that CHKD’s anesthesiologists work with thousands of children every year, and Noah would be in very good hands. He even drew diagrams of everything he was going to do.”

The cardiac cath gave the physicians a clear picture of what was coming next for Noah and his worried parents. “Noah’s stenosis had progressed to a critical level, significantly blocking the flow of oxygenated blood from his heart to the rest of his body,” Dr. Vance said. “In some cases, we can open narrowed valves and vessels during catheterization, so children don’t need open-heart surgery,” he explained. “But the location of Noah’s defect – in the wall of the aorta itself – made that too risky. A balloon procedure might damage the wall of his aorta and cause an aneurysm [a sac-like ballooning of the wall that could burst].”

Dr. Vance gave Laura and David the news. Their son, he said, would need open-heart surgery to ensure a wide enough artery for proper blood flow.

“I was so relieved that he’d come through the first procedure that I couldn’t think beyond that,” Laura said. “The fact that he would need open-heart surgery didn’t really sink in until the next day.”

That’s when the Hoffmanns met the doctor who would become the next member of Noah’s CHKD team: pediatric cardiac surgeon Muhammad “Ali” Mumtaz, who leads CHKD’s cardiac surgery team.

Dr. Mumtaz met the family the very next morning. “We talked in the activity room of the PICU while Noah played,” Laura said. “He was very reassuring and spent a lot of time with us, explaining what would need to be done. We really liked him and felt confident that we were still in good hands with CHKD.”

Surgery was scheduled for the next week.

“I didn’t sleep the whole week,” Laura said. “I had to inform all my friends. Gloucester is a pretty tight-knit place, and soon, Noah was on a hundred different prayer lists. Everyone was so great and so supportive. I can’t imagine how hard it would have been on us if we had to leave CHKD and our incredible support system to get the care that Noah needed.”

Riley and her mom, Cheryl.
Noah and baby sister, Payton

On the morning of the surgery, Laura found that this time they were better able to handle the moment when Noah would go off to surgery. “They gave him medicine that made him sleepy, so neither one of us cried. Brandy told us that she’d come out about every hour and a half to let us know how things were going,” she said, referring to cardiovascular nurse practitioner Brandy Kramer. “And she did just what she said she’d do.”

In the operating room, a heart-lung machine was used to add oxygen to Noah’s blood and pump it to other parts of his body while his heart was stopped during surgery. Dr. Mumtaz dissected the abnormal aorta, removed the defective tissue and built a tissue graft to create a wider artery that could accommodate normal blood flow.

The surgeon says that Noah was the first patient at CHKD to receive a graft of a new substance called CorMatrix. “It is a bioengineered material that essentially provides a scaffold for Noah’s own cells to grow on and bond with, in the hope that the repair will grow with him,” he said. “The hope is that the new material will eliminate the need for future surgeries.”

After many anxious hours, and reassuring updates from Brandy, Dr. Mumtaz told the Hoffmanns that Noah’s stenosis had been even more significant than was expected.

“But Noah did great throughout the surgery,” he announced. Noah spent the next several days in the pediatric intensive care unit. “Brandy told us what to expect,” Laura recalls. “Noah would be pale and there would be tubes. There were doctors and nurses all around him, but they let us be there too.”

Only one day after his surgery, Laura was surprised to find her son sitting up in bed. “I couldn’t believe it,” she said.

When it was time for him to venture from his bed, a nurse provided a wagon for his first trip to the playroom. “The minute he saw that wagon, his face just lit up,” Laura said. “I hadn’t seen that smile in four days. He was still my Noah.”

Noah hasn’t slowed down since then. He went home a week after surgery. Today he plays with his baby sister, Payton, who was born with no heart problems whatsoever. And he returns to his special team at CHKD to monitor his progress.

“So far, he hasn’t missed a beat,” Laura reports.

Drs. Bullaboy and Vance practice with Children’s Specialty Group PLLC at CHKD. Dr. Mumtaz is the director of CHKD Surgical Specialty Group’s cardiac surgery practice.

This story was featured in the second quarter 2010 issue of KidStuff, a publication of Children's Hospital of The King's Daughters.