In less than 24 hours, Abigail Franklin’s life would change forever.
“I’m going to be able to do everything I couldn’t do before,” she said standing in the Hermann Atrium, two floors below the Memorial Hermann-Texas Medical Center operating room where she would soon undergo major surgery.
Eve and John Franklin stood nearby, listening to their 15-year-old daughter’s words, which defied Abigail’s past and a future her family once assumed she would face.
Exercise—maybe even tennis lessons? Taking the stairs instead of the elevator, for sure. Abigail would be healthy enough to start a family of her own one day if she wanted—years from now, of course.
For the high school sophomore and aspiring cardiologist, it was the idea of running that excited her the most.
“I don’t think I’ve ever really run far,” Abigail said. “I can run for a few seconds, but I always get really tired.”
A phone call brings unexpected hope
Abigail’s legs work fine. Her heart was another story—a complicated tale that began before birth. As she got older, her condition worsened and her physical limitations increased, as Eve and John knew they would, inevitably. Activities as simple as walking to her pre-AP chemistry class at her high school in College Station, Texas left Abigail gasping for air and her grade 4 heart murmur vibrating forcefully.
At best, Abigail’s heart would last until she was 30, the family was told. She would then be placed on a heart transplant recipient waiting list. It could be days or years before she found a match.
Then came the phone call in June from Becky Sam, BSN, RN, the Pediatric and Congenital Heart Surgery Nurse Manager at Children’s Heart Center at Children’s Memorial Hermann Hospital, which left Eve in disbelief and full of hope for her daughter.
“I kept pulling the phone away from my ear and looking at it,” Eve said. “She was telling me an experienced surgeon, Dr. Salazar, had reviewed Abigail’s medical history and that he could fix her heart.”
“One in a million of one in a million”
Abigail was born with congenitally corrected transposition of the great arteries (CCTGA), a rare condition in which the connections of her heart developed backwards. Her right ventricle, the pumping chamber that is supposed to supply blood to her lungs, is connected to her body. The left ventricle, conversely, was connected to Abigail’s lungs.
The condition occurs in fewer than 1 percent of all people with congenital heart defects and is unique to each individual. But Abigail’s heart was exceptionally different.
“The physician who first treated Abigail told us that she was one in a million of one in a million,” Eve said. “CCTGA presents differently in each patient with one or more aspects. But Abigail had all five aspects of the defect.”
Abigail had an arterial obstruction (severe pulmonary stenosis), a leaky valve (Ebstenoid valve), transposed ventricles (ventricular inversion), atria and ventricles displaced from one another (atrial ventricular discordance) and holes (atrial and ventricular septal defects) in her heart.
Abigail’s complications worked together in an improbable harmony for nearly 15 years, aided by a pacemaker for heart block that was implanted when she was only 3 days old. Until recently, Abigail lived a relatively normal life outside of the physical limitations that kept her from even casual activities, like riding a bike.
Finding a solution for Abigail’s heart
The functioning imperfections of Abigail’s heart began unraveling in January of 2017. She tired easily and struggled to get enough oxygen to her lungs.
The Franklins sought several opinions throughout the state. Pediatric cardiovascular surgeon Jorge Salazar, M.D., the co-director of Pediatric and Congenital Heart Surgery at Children’s Heart Center at Children’s Memorial Hermann Hospital, thought he could help. Dr. Salazar thought a double-switch procedure through open heart surgery would address Abigail’s heart problems, avoid the pending heart failure and eliminate the need for an eventual heart transplant.
Dr. Salazar planned to surgically re-position Abigail’s major heart vessels to match the configuration and blood flow of a normal heart, but first he had to determine whether her obstructed transposed left ventricle would be strong enough to pump blood to the body when the heart was correctly connected.
“After looking at the images of Abigail’s heart, I spoke to Eve and said, ‘I have good news,’” said Dr. Salazar, who serves as professor and chief, Division of Pediatric and Congenital Heart Surgery at McGovern Medical School at UTHealth.
“The amazing thing about Abigail’s heart was that the obstruction in her transposed left ventricle that had caused it to work harder than it needed to, in a sense, had been training itself for its intended job her entire life,” Dr. Salazar said. “It was kind of miraculous.”
A successful surgery
On Sept. 12, Abigail underwent a successful 12-hour open-heart surgery that would alter the trajectory of her future. Dr. Salazar and the surgical team closed the holes in Abigail’s heart and rewired her arteries and ventricles.
Abigail expected to still need her pacemaker even after surgery. However, after surgery it was discovered that Abigail’s heart was able to regulate its rhythm on its own. She would no longer need her pacemaker, or the quarterly appointments required to check its battery.
Approaching a new frontier
Two weeks later, Abigail returned home. Once she heals from surgery, she will be able to test new boundaries and explore activities she never before thought possible.
For the first time, the Franklins have been freed from the worry that their daughter would someday need a heart transplant.
“Abigail is smiling more and she’s getting used to the new way her heart circulates blood through her body,” Eve said. “She feels different and it’s all good.”
One month following the surgery, Eve waited for Abigail in their church’s sanctuary after her daughter’s Sunday school class. Abigail ran up to her mother, her face pink and flush, laughing with friends.
Her hand was on her chest, over the spot where a damaged heart had once vibrated with such force it took Abigail’s breath away.
This time was different.
“‘Mom, I can’t feel it! That’s never happened before!’” Eve recalled her daughter saying. “Before, even at rest, her heart felt like a cat was purring on her chest. With simple exertion, it would pound like a gong, heralding the dizziness and difficulty breathing to follow.
“But she can’t feel that anymore. That is a miracle.”
To find a pediatrician or cardiologist nearest you, visit http://www.memorialhermann.org/schedulenow/ or call 713-222-CARE (2273). For more information about Children’s Heart Center at Children’s Memorial Hermann Hospital, click here.