* Carmel is believed to be the smallest baby in Northern California — and perhaps the world — to successfully undergo the high-risk Norwood procedure for hypoplastic left heart syndrome
* Initial surgery over the Thanksgiving holiday on her marble-sized heart was so high-risk that the statistical chance of survival could not even be predicted
For Release: May 4, 2016
STANFORD, Calif. — Five-month-old Carmel Smith of Hayward, California, headed home last month from Lucile Packard Children's Hospital Stanford with parents Uyen and Chauncey and big sister Chloé. But first, she made heart surgery history.
Born eight weeks premature on Nov. 23 at a weight of only 1.3 kg (less than 3 pounds), Carmel is the smallest baby ever in Northern California — and probably anywhere — to successfully undergo a risky open-heart surgery called the Norwood procedure. Cardiothoracic surgeon Frank Hanley, MD, performed the operation the day after Thanksgiving, four days after she was born. The Norwood is the first step in correcting circulation issues caused by Carmel’s serious heart defect, called hypoplastic left heart syndrome.
“To our knowledge, Carmel is one of the smallest babies — if not the smallest baby ever — to survive the Norwood,” said Hanley, executive director of the Children’s Heart Center at Stanford Children’s Health.
Hanley said Carmel has “defied all expectations” by also doing extremely well during the high-risk post-operative period that followed, and by undergoing a successful follow-up surgery on March 11 called the bi-directional Glenn. Her recovery from the Glenn went well, and she was released from the hospital less than a month later, on April 8.
“Carmel is our miracle baby,” Chauncey said. “It’s been a long road with a difficult pregnancy and delivery, the HLHS diagnosis and many ups and downs. But now we know there’s light at the end of the tunnel, and we couldn’t be more grateful to Dr. Hanley for saving Carmel’s life.”
Babies born with hypoplastic left heart syndrome are missing the left ventricle, which is the major pumping chamber of the heart and normally pumps oxygen-rich blood to the body. Surgical treatment includes three open-heart surgeries (Carmel will undergo the third one, the Fontan procedure, when she is 3 to 4 years old). These staged operations redirect the blood flow so that the right ventricle, which normally pumps deoxygenated blood to the lungs, can be used as the body’s main pumping chamber.
Chauncey and Uyen were shocked by the unexpected HLHS diagnosis during a 20-week prenatal checkup at the hospital’s Perinatal Diagnostic Center. But the Smiths quickly learned that the Heart Center’s extraordinary outcomes meant that they couldn’t be in a better place for what lay ahead — the surgeries to reconfigure the circulatory system, as well as lifelong care from congenital heart specialists to optimize the function of the heart’s single pumping chamber.
The Norwood procedure, which Carmel underwent in November, was far riskier than the two subsequent operations. The Norwood has only a 50 percent chance of success when performed on any infant in the “low-birth-weight” (under 2.5 kg) category. “The smaller a baby is under this threshold, the riskier the surgery becomes,” explained Hanley, who is the Lawrence Crowley, MD, Endowed Professor in Child Health at the Stanford University School of Medicine.
With Carmel’s weight at only 1.3 kg and her heart the size of a marble at the time of the surgery, Hanley could not even provide her parents with her statistical chances of survival. He only knew that, like Carmel, they were very small.
While Hanley and his team are known for taking on surgically complex cases that others can’t or won’t, the confluence of risk factors in Carmel’s case was extraordinary. “That’s why there have been very few successful Norwood operations for any low-birth-weight babies, let alone for one as small as Carmel,” he explained.
But once again, Hanley beat the odds and performed a successful Norwood, balancing the blood flow between Carmel’s lungs and the rest of her body.
Before Carmel could go home for the first time Feb. 5, Chauncey and Uyen underwent a home care “boot camp” of sorts, learning how to monitor her during the risky interim period between her discharge after undergoing the Norwood procedure and her admission for the Glenn surgery several weeks later.
Historically, the nationwide survival rate during this interstage period was 85 percent. Over the past decade, an innovative Home Monitoring Program developed and led by pediatric cardiologist Gail Wright, MD, has brought the hospital’s interstage survival rate up to 98 percent.
The HMP tool kit includes two low-tech but lifesaving pieces of equipment: a scale and a pulse oximeter, a small device that clips on the baby’s finger to measure oxygen levels and heart rate. The Smiths recorded Carmel’s numbers once per day and reported them to a nurse practitioner at Packard Children’s. The program also supported close communication between the Smiths, the hospital’s medical team and Carmel’s community pediatrician.
The HMP did its job by detecting issues with Carmel’s oxygen levels that required her to be admitted to the hospital for treatment in late February. “Without this close surveillance, this red flag could have gone undetected and potentially could have led to death during this risky interim period,” said Wright, who is a clinical associate professor of pediatric cardiology at the School of Medicine. “Instead, Carmel was promptly admitted and successfully treated.”
Once Carmel’s health stabilized, she underwent the Glenn procedure — in early March, sooner than expected — and it went smoothly. The Glenn surgery brought the blue (deoxygenated) blood from half of her body directly into the lungs. The Fontan surgery that Carmel will undergo in a few years will bring the blue blood from the other half of her body into her lungs so that her blood can be fully oxygenated.
“We are definitely blessed that both the Norwood and Glenn surgeries went so well,” Uyen said.
This experience has brought the Smiths even closer together as a family. “Even after everything Carmel has been through, her tiny body is still so strong and resilient,” Uyen said. “It puts things into perspective.”
With Carmel home, the Smiths say their family is whole again. They can enjoy the small moments of everyday life together that others may take for granted. Moments like reading Peter Rabbit. “When we start to read a book, she pauses for a moment, and then she gets this look that shows how much she’s enjoying it,” Chauncey said. And when Carmel wants to go for a wagon ride, 2.5-year-old Chloé insists on pulling it.
Now, the Smiths want Carmel’s story to serve as a source of inspiration and support for other parents whose children are born with serious and seemingly impossible congenital heart conditions.*
“We have hope now,” Uyen said. “We want other parents to have hope, too.”
* Parents who have children born with single ventricle defects can obtain support through the National Pediatric Cardiology Quality Improvement Collaborative, including its Parent & Family Resources page.
* Discover more about the Heart Center at Lucile Packard Children's Hospital and Stanford Medicine Children’s Health.
Stanford Medicine Children’s Health, with Lucile Packard Children’s Hospital Stanford at its center, is the Bay Area’s largest health care system exclusively dedicated to children and expectant mothers. Our network of care includes more than 65 locations across Northern California and more than 85 locations in the U.S. Western region. Along with Stanford Health Care and the Stanford School of Medicine, we are part of Stanford Medicine, an ecosystem harnessing the potential of biomedicine through collaborative research, education, and clinical care to improve health outcomes around the world. We are a nonprofit organization committed to supporting the community through meaningful outreach programs and services and providing necessary medical care to families, regardless of their ability to pay. Discover more at stanfordchildrens.org.