A Lucky Heart

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Ridgely Ingersoll

CodySmith1Expecting a baby is kind of like waiting to open a present. You’re allowed a few really good hints about what’s inside: grainy ultrasound images and fluttery kicks that get stronger as the months pass. And all along, you yearn to hold that gift in your arms.

But even in this era of 4-D ultrasounds and prenatal testing, babies can surprise us medically. When that happens, the pediatric experts at Children’s Hospital of The King’s Daughters are ready to respond.

That’s just what happened when Cody Matthew Smith was born on July 9, 2007. His parents, Jessica and Matt Smith of Hampton, had no idea their baby would be born with a rare, life-threatening heart defect.

Matt is in the Navy, and Jessica received regular prenatal care, including high-resolution ultrasounds. When she developed dangerously high blood pressure in her ninth month and her doctors at the Langley Air Force Base Hospital performed an emergency c-section, they were probably more worried about her health than her baby’s.

Matt and Jessica have two other children, so they knew what to expect in the delivery room. But from the moment of Cody’s birth, nothing played out as the young parents anticipated.

“He looked really gray at birth,” Matt remembers. “And he never pinked up like babies usually do.” Cody didn’t spend any time in the delivery room after he was born. Nurses whisked him right to the nursery. When Matt tried to follow, he was asked to wait outside so they could run some tests. “About an hour later, they said he might have pneumonia from breathing in fluid while he was being born,” Matt said. “They told us the CHKD transport team was coming to take Cody and Jessica to Portsmouth Naval Hospital.”

Cody spent just enough time at the Naval Medical Center to receive an accurate diagnosis from Navy cardiologist Mike Carr. Instead of pneumonia, Dr. Carr said Cody had total anomalous pulmonary venous return – TAPVR – which is a rare and very complex heart defect.

Dr. Carr told the devastated parents that Cody would need immediate open-heart surgery to survive, so he was transferring the baby to CHKD.

“That’s when I fell apart,” Jessica said. “I didn’t know if I would ever see my baby again. It was awful. I’ve never felt so helpless in my life.”

It’s hard to think of a baby with a heart defect as being lucky. But in one important way, Cody was a very lucky little boy. Just a few months before he was born, CHKD’s heart surgery program established an affiliation with Columbia University in New York, one of the top 10 heart programs in the country. This association brought Columbia faculty member and pediatric cardiothoracic surgeon Christian Gilbert to CHKD’s full-time professional staff – just in time to save Cody.

Dr. Gilbert described Cody’s condition: “In TAPVR, the four pulmonary veins, which normally carry oxygen-rich blood from the lungs to the left side of the heart, drain instead into the right side of the heart. Of the three types of TAPVR, Cody’s was the most complex.

CodySmith2“Instead of connecting to the left side of the heart, Cody’s pulmonary veins were abnormally joined into one large vein that drained his oxygenated blood down through his diaphragm into his liver. His blood then traveled through the tiny capillaries of the liver and through the inferior vena cava back to the right side of his heart.”

Cody also had a hole between the right and left sides of his heart, called atrial septal defect. This defect actually allowed him to live, because it provided a route for his blood to move from the right to the left side of his heart and then out to his tissues. But his heart was pumping a mixture of oxygenated and non-oxygenated blood. That’s why Cody’s skin was so gray.

But the biggest problem for children with Cody’s form of TAPVR, says Dr. Gilbert, is that the diaphragm and the vascular system of the liver obstruct circulation like a sponge under a spigot. “The blood from his lungs had nowhere to go, creating dangerous levels of pressure and fluid in his lungs. This condition, called pulmonary edema, is what kills most babies with Cody’s condition,” he said. “Fortunately, Dr. Carr made the right diagnosis at Portsmouth Naval. The TAPVR diagnosis is one that’s frequently missed, and time is of the essence with this condition. He knew that we could not delay this child’s surgery.”

Dr. Gilbert’s pager went off at 3 a.m. alerting him to Cody’s imminent arrival at CHKD. Before he left home, Dr. Gilbert mobilized his surgical team, including fellow cardiothoracic surgeon John Artrip and cardiac anesthesiologist Jimmy Windsor.

Just three hours later, Cody was in the operating room at CHKD connected to the heart-lung machine, a device that adds oxygen to the blood and pumps it to other parts of the body so the heart can be stopped during surgery.

For the next four hours, Dr. Gilbert and his team worked within the newborn’s tiny chest cavity, first closing the connection that allowed the blood to drain into the liver.

“Next, we lifted the heart up out of the chest cavity to create an opening in the left atrium for the pulmonary veins,” Dr. Gilbert explained. “We then connected the pulmonary veins to the left atrium and closed the atrial septal defect.” The entire procedure, he recalls, was complete by 10 a.m., just hours after Cody’s dramatic birth miles away in Hampton.

After surgery, Cody spent two weeks in the PICU where he was monitored closely. “He was surrounded by tubes,” Matt recalls. There was a tube in his airway to help him breathe and IVs to give him fluids and medications. Another line measured his blood pressure. Tubes in his chest drained air, blood and fluid from the chest cavity. He was fed through a tube that went through his nose to his stomach. And his urine was drained and measured through a catheter.

But his parents were relieved that their baby was alive and thriving. A week and a day after he was born, Cody was strong enough to breathe without the ventilator, and his mother had her first real opportunity to hold him in her arms. “That was a great feeling,” she said with a smile.

After 14 days, Cody was stable enough (and almost tube free!) to be moved from intensive care to a monitored nursing unit where his biggest challenge was to learn how to eat – a normal developmental process that was pre-empted by more serious concerns in Cody’s first days. With a little help from occupational therapists, he quickly became an eager eater. And 22 days after his dramatic entrance into the world, Cody went home to Hampton to meet his big sisters, Alyssa, 6, and Gabrielle, 5.

Today, there is little evidence of the drama that surrounded Cody’s birth except for the healing scar on his chest. In the months and years to come, he will be monitored by a pediatric cardiologist, but by all indications today, his blood is circulating normally. Thanks to CHKD, Dr. Gilbert’s skills and his expert team, Cody’s cheeks – as well as his future – look rosy.

And that is a gift worth celebrating, his parents say. “We know we’re lucky to have him,” Jessica says. “If it weren’t for CHKD and Dr. Gilbert, he wouldn’t be here right now.”

Drs. Gilbert, Artrip and Windsor comprise CHKD Health System’s cardiac surgery division. They are employed through the Bon Secours Heart Institute. Drs. Gilbert and Artrip are members of the faculty of Columbia University.

This story was featured in the fourth quarter 2007 issue of KidStuff, a publication of Children's Hospital of The King's Daughters.

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