Physicians performed open-heart surgery on Elenilza Souza in her native Brazil when she was just 18 years old, replacing a faulty valve that had left her gasping for breath after the slightest exertion.
The fix worked for a while, but at age 25, by then living in Mount Laurel, she felt the dreaded symptoms again. One time, when she went to the gym with husband Taylor Tritten, she had to quit after just a few leg lifts.
Specialists at Our Lady of Lourdes Medical Center in Camden, N. J., told her they could replace the faulty valve yet again. But given her young age and recent marriage, they asked a key question: Was she pregnant?
Unbeknownst to Souza, she was.
What followed, according to her physicians, was a medical first.
The valve that needed replacing was Souza’s mitral valve, a vital gateway that directs oxygenated blood into the left ventricle before it is pumped out to the body. Lourdes physicians told her they could replace it with a newer technique that avoided the rigors of surgery, using a catheter to insert the new valve.
But when Souza called two to three weeks later with the news that she was nine weeks along, the question of timing became crucial.
The valve procedure, though not surgery, still would mean exposure to anesthesia and radiation. The team needed to wait until the fetal organs had developed.
Yet the doctors could not wait too long, as Souza’s body would be producing more blood with every month to cope with the growing demands of pregnancy. Without a new valve to handle the increased flow, the lives of the fetus and mother would be at risk, said Arthur Martella, chief of cardiothoracic surgery at Lourdes.
“By the time you get to that third trimester, she would be in heart failure,” he said.
After consulting specialists in maternal and fetal medicine, Martella and colleague Ibrahim Moussa told Souza that the best window for her procedure was the 16th week of pregnancy.
No other pregnant woman had undergone the procedure, as far as they knew after consulting the medical literature and the company that made the valve. But the device, approved less than two years ago for use in the mitral opening, had worked well in most other patients who were ineligible for surgery. It seemed like her best option.
On April 16, the team inserted a catheter into a vein in Souza’s groin, snaking the slender instrument up into her heart. To reach the opening where the new valve would be placed, they first needed to poke a hole through the atrial septum, a wall between two chambers of the heart.
Then came another twist. Because of Souza’s open-heart surgery seven years earlier, her septum was tough and scarred.
Try as they might, they could not get through.
At first, it felt like a bad sore throat.
Souza was 9 years old, growing up on a farm outside São Paulo, and her parents gave her lots of soothing hot tea.
But after a week or two, her illness got worse. She started to feel pain in her joints, and eventually, her hands began to twitch.
Untreated with antibiotics, her strep-like infection had developed into rheumatic fever. The disease is rare in the United States because strep infections tend to be diagnosed and treated promptly, but it remains common in some developing nations. She went to one hospital, then another, finally getting the right diagnosis and treatment with penicillin —but the damage to her heart was done.
Doctors told her she would likely need valve-replacement surgery someday, and, sure enough, that happened when she turned 18.
The surgery helped, and in 2017 Souza moved to the United States to work as an au pair, taking care of a young couple’s children. She started going to the gym.
“I worked out so I could be in good shape, to keep moving with the kids,” she said, often exercising with Tritten.
But by the time the two were married last December, she felt increasingly fatigued. Her replacement mitral valve, which was expected to last at least a decade, had started to fail.
Ordinarily, when the leaflets in the mitral valve fold open to allow the flow of blood with each heartbeat, the opening is the size of a silver dollar, said Moussa, medical director of the structural heart program at Lourdes. In Souza’s case, the leaflets in her replacement valve had become calcified and stuck together. The opening had shrunk to the size of a dime.
The new valve that they planned to insert, made by Edwards Lifesciences of Irvine, Calif., was designed to replace a different structure in the heart called the aortic valve.
Still, the U.S. Food and Drug Administration allows the similar-sized aortic device to be used in the mitral opening so long as two conditions are met. First, the patient must previously have undergone a surgical valve replacement, providing a sort of anchor so that physicians can wedge the new valve easily inside the old one. Second, the patient must be at high risk for undergoing a second valve replacement through traditional surgery.
Souza fit both descriptions. Inserting a new valve with a catheter, her physicians agreed, was the way to go.
There was pushback at first from Souza’s health insurance, which she gets through her husband, who serves in the Air Force. But once Lourdes physicians called to explain that she had no other good options, the insurer gave the go-ahead.
THROUGH THE RIB CAGE
On the day of the procedure, when the physicians were unable to poke through her septum to deliver the new valve, it was not entirely a surprise. Having looked at her heart in advance with a type of ultrasound, doctors could tell that the wall between the chambers might be scarred.
So they were prepared for Option B: cutting through her rib cage to implant the valve in her heart from the other direction. And if for some reason that didn’t work, there was still the option of open-heart surgery, though that would endanger the fetus.
Moussa and Martella met multiple times in advance with colleagues who had expertise in pharmacy, anesthesia, maternal fetal medicine, and cardiology.
“I wanted everybody to think about the worst-case scenario,” Moussa said. “We had her ready for Plan A and Plan B and Plan C in case things didn’t work out.”
The rib cage approach —Plan B —worked.
Souza was in pain from the incision for a few days, but she was up and about by day four.
The size of her mitral opening is not quite normal, but it is much bigger than it had been.
“We can never bring the silver dollar back,” Moussa said. “We took her from a dime to a little bit over a quarter.”
And her baby, due in early October, appears fine.
“It’s incredible what they did,” Souza said, in an interview just nine days after the procedure.
At the Mayo Clinic, which performed many of the procedures that paved the way for FDA clearance to use the aortic device in the mitral opening, physicians say they are unaware of other pregnant patients who have undergone such a procedure.
Transcatheter valves designed specifically for the mitral opening, which is a slightly different shape than the aortic opening, are still in development, said the Mayo’s Mackram Eleid, an interventional cardiologist. But for now, the aortic version is considered a good option.
Physicians do not know how long the devices, made from a combination of animal tissue and synthetic material, will last, though they seem to be good for at least a decade.
Someday, Souza likely will undergo a second open-heart surgery to receive yet another device: a fully mechanical valve that will last the rest of her life. (That kind of implant was not an option this time around, as she would have needed to take blood thinners, endangering the fetus.)
For now, she can enjoy the life of a healthy pregnant woman.
She is supposed to take it easy for four to six weeks. But the day before speaking about her experience, she felt well enough to go shopping with her mother-in-law, Terri Tritten. They made a Target run, and picked out a baby crib.