Heart to heart
UF surgeons and dedicated medical teams give miracle conjoined twins a chance at a full life through ground-breaking procedures.
By Morgan Sherburne
Jacquelyn and her partner, Mark, were anxious. Today was Jackie’s 20-week ultrasound, the day they would find out if they were having a boy or a girl.
Already, Jackie felt bigger than during her first pregnancy. Her baby seemed to be doing cartwheels, with hands poking everywhere inside her.
The ultrasound technician swept a wand over Jacquelyn’s belly, then paused. She looked at Jackie, then at Mark before getting up and leaving the room.
Minutes later, Jackie and Mark were sitting with Jackie’s obstetrician. The couple, the physician said, was going to have twins. Mark was elated, but his joy lasted only a few seconds.
The twins were conjoined, they were told, and likely would not survive the pregnancy. If they did, they would probably die within hours of being born.
Previous sonograms had detected a single heartbeat, but that was because the babies’ hearts were beating in sync. In that moment, Jackie and Mark went from planning for a new member of the family to thinking about funerals.
The two were sent for a second opinion with a physician who specialized in high-risk pregnancies, but the dire outlook didn’t change. A third doctor gave them the same opinion.
“But she also said she would send me for one last opinion with Dr. Co-Vu,” Jackie said, “and it turned our world around.”
‘The odds were really stacked against us’
Jennifer Co-Vu, M.D., director of the Fetal Cardiac Program in the UF Health Congenital Heart Center, specializes in fetal cardiology. She uses noninvasive methods such as echocardiography and MRI scans to examine the hearts of unborn babies, helping diagnose defects and form plans of care for when the babies are born.
Within a week, Jackie was at UF Health, in an appointment with the Maternal and Fetal Medicine Program, where she was seen by Monique Ho, M.D., an assistant professor in the department of obstetrics and gynecology’s division of maternal-fetal medicine. Ho confirmed Jackie’s diagnosis, and referred her to Co-Vu.
At her appointment with the Fetal Cardiac Program, Jackie waited for hours on an examination table while a fetal sonographer moved a wand over her belly, this time in a test called an echocardiogram. Based on Jackie’s previous ultrasound, Co-Vu knew the babies were connected at the belly and chest and, based on the tiny images of the organs, she knew there was a chance their hearts were connected. The echocardiogram could more specifically examine the structures of the babies’ hearts.
All Jackie knew was that three doctors had told her there was little hope for her babies — girls, she now knew.
But Co-Vu was becoming excited. The hearts had connections in the upper atria, in an area that could potentially be separated in cardiac surgery.
The atria are the upper chambers of the heart, where blood enters the organ. Oxygen-poor blood enters the right atrium through two large veins called the inferior and superior vena cava. The atrium pushes this blood into the lower right ventricle, which then pushes blood through the pulmonary artery and into the lungs to become oxygenated. When it leaves the lungs, the oxygenated blood returns to the upper left atria of the heart, moves through the left ventricle, and is distributed throughout the body.
If surgeons could fix heart defects in single babies in the atrium, she thought, was there a chance a surgeon could separate a pair of hearts that shared an atrial connection? She knew that Mark Bleiweis, M.D., chief of pediatric cardiovascular surgery and director of the UF Health Congenital Heart Center, had experience separating conjoined twins at the heart — although in the two previous surgeries, one baby in each pair did not survive. In any case, Co-Vu was confident that Jackie and Mark’s twins would survive after they were born, and whether the parents decided to separate the twins, they needed to start planning for their birth.
Co-Vu called Jackie and Mark into the clinic’s counseling room.
“She told us not only would the babies survive, she said, ‘We think we can separate them,’” Jackie remembered. “We were just blown away.”
Jackie and Mark now faced a challenging decision.
Their babies would survive, but should they risk an operation to separate them? Co-Vu knew there were very few, if any, known cases of conjoined twins who shared a heart connection surviving such a surgery. But raising conjoined twins comes with its own set of difficulties.
Onesies come with two arms and two legs. Bottles are designed for babies who aren’t face-to-face with their twins. Car seats are engineered for just one baby, not two.
“Psychologically, with babies facing each other, it’s just hard for them to achieve a good realistic activity of daily living,” Co-Vu said. “We were very scared at first that this may have not been the right decision. But, yet again, we wanted to give these kids the best chance in gaining the most normal life they could have.”
Over the next 16 weeks, fetal sonographers in the Fetal Cardiac Program took endless scans of the twins in utero.
Just like other babies, but uniquely
On the twins’ birth day — two days short of full term at 36 weeks — a phalanx of nurses, obstetricians and pediatric cardiologists packed the delivery room. Jackie said the birth went quickly.
“When they came, it was beautiful,” Mark said. “The whole team parted like the Red Sea.”
The twins were whisked away to the neonatal intensive care unit, where a room had been prepared for them. Jackie was only thinking of the babies. Before meeting Co-Vu, she had been preparing for funerals. Now, she needed to see them, alive and healthy.
“Forty-five minutes after my C-section was finished, I was in the NICU,” Jackie said. “I was just happy to see them.”
In the NICU, the babies were assigned two nurses, Sara Garnto, R.N., and Natalia Hart, R.N., who were to provide around-the-clock care.
Garnto and Hart’s jobs were two-fold. They performed jobs typical of nursing — feeding the twins, giving them medication — and a less tangible job: gaining the trust of Jackie and Mark, who had to leave their twins at the end of the day in order to return home and care for their daughter, Lilli, 4.
Garnto began picking up shifts to care for the twins. Her husband wondered if they were saving for a trip he didn’t know about; in order to protect the privacy of Mark, Jackie and the twins, Garnto couldn’t explain the uptick in her work hours.
“I’d just say, ‘Well, we have some patients at work that I’m really concerned about,’” Garnto said.
During those hours, Garnto cuddled them as best she could. She told them their parents loved them, when Jackie and Mark had to leave the hospital for the night. She monitored the medications the babies were given and kept an eye on their blood pressure. She knew which baby tended to urinate more, and when the babies wanted to be moved into a more comfortable position. She could tell when they were tolerating their formula and when they were not. She watched them develop individual personalities — Scarlett is a firecracker, and Savannah is more laid back.
The babies would put their tiny fists in each other’s faces, working themselves up in anger, then they would pat and soothe each other.
“They would stick out their fists and their arms, but because they were face-to-face, it was like they were doing it in each other’s faces,” Garnto said. “But they would work it out just as if they were teenagers, fighting over makeup or clothes. They would battle it out, and then say, ‘OK, you can borrow the sweater.’”
During the day, Jackie was by their bedside. When she wanted to hold the babies, a team of nurses carefully lifted them into her arms. NICU nurse Natalia Hart sewed tiny outfits together, belly to belly, for the babies. The nurses made them headbands and decorations for their room. A pharmacist made the sisters headbands and onsies emblazoned with the words “wombmates.”
“They were always either hitting each other or hugging,” Jackie said. “Otherwise, they did everything other babies do. They smiled, they ate, they peed, they pooped. We were just waiting.”
The surgeons, physicians and anesthesiologists were waiting, too. Though the girls were healthy when they were born — a collective 10 pounds, 5 ounces — any chance for the babies’ tiny organs to grow was welcome.
The early days of the babies’ lives were filled with MRIs, X-rays and echocardiograms to confirm what the doctors saw in utero.
The scans gave Co-Vu valuable information about how the hearts had grown, but the cardiologist wanted to take it one step further. She wanted to be able to hold the hearts and examine not only their connection, but what the hearts looked like on the inside.
Co-Vu used the scans and, in consultation with a Colorado company called 3D Systems, created a 3-D model of the twins’ connected hearts. The physicians could pull it apart to see how the interior of the hearts grew. The model is color-coded to delineate different structures in the heart.
Co-Vu discovered separation was critical to keep Savannah and Scarlett healthy. Scarlett was born with a heart defect called “transposition of the great arteries with pulmonary stenosis,” a common condition for twins who share a heart connection, said Bleiweis.
In this defect, the pulmonary artery, normally connected to the lower right ventricle of the heart, is connected to the left ventricle, and the aorta, normally connected to the left ventricle, is connected to the right ventricle. This causes oxygen-poor blood to flow back into the body without passing through the lungs, and oxygen-rich blood to flow back into the lungs, explained Co-Vu.
Their shared connection meant that the babies were also sharing a blood supply. Without correction, one twin — likely Scarlett — would go into heart failure.
“There was no way to predict how the babies’ blood would flow, and which baby would get more blood than the other,” Co-Vu said. “They would have eventually needed medication to help them, and if we didn’t separate them, the babies may not have survived long term.”
But when Bleiweis examined Co-Vu’s 3-D model, he confirmed the hearts could be separated.
“With the 3-D reconstruction of the hearts, we could really evaluate the connection and its proximity to vital structures,” Bleiweis said.
Bleiweis and Co-Vu saw that the connection was close to vital veins, including the pulmonary veins and the superior vena cava of both twins, which would typically carry oxygen-rich blood into Scarlett’s upper right atrium. But because of her heart defect, the veins were carrying oxygen-poor blood into the heart. The 3-D model allowed Bleiweis to carefully plan the separation around these crticial veins.
“The odds were really stacked against us,” Bleiweis said. “Many other big centers asked us why we would do this because once there’s a cardiac connection, the outcomes have been so poor that we didn’t want to give anybody any false hope. We were realistic, and I think the family understood that.”
This complication wasn’t the only one. The doctors also closely monitored the development of the twins’ liver.
“All the imaging we obtained before the babies’ birth and after indicated that they had what looked like one giant liver without any true plane of separation,” said Saleem Islam, M.D., M.P.H., chief of the division of pediatric surgery and a professor in the department of surgery.
The liver itself requires two blood supplies, Islam said. One is the hepatic artery, and another is called the portal vein. The portal vein carries blood from the gastrointestinal tract to the liver, and is the primary way the liver can metabolize nutrients.
“For the first month, because the babies were so small, all of the imaging sets we obtained were unable to determine whether the babies had two portal veins or just one,” Islam said. “Without a portal vein, the liver would not survive.”
And without a liver, one of the babies would not survive.
Jackie and Mark prayed, Jackie wrote in a journal she kept about her experience.
“Back to being heartbroken again,” she wrote. “How could we lose one after all they have been through? I prayed. We prayed. We asked everyone to pray.”
Once more, the babies defied the odds. As the babies grew, the doctors were able to find both portal veins — though one was a little smaller than the other.
“We were in heaven,” Jackie wrote. “The doctors continue to tell us they are one-of-a-kind babies, and this is all very unknown and risky, but they are hesitantly hopeful, cautiously optimistic.
Me,” she wrote, “I KNOW GOD’S GOT IT.”
A long short time
The surgical teams also needed time to prepare for the surgery, scheduled for June, when the babies would be 10 weeks old. Every aspect, down to how anesthesia would be delivered to the twins, was complicated.
The girls’ shared blood supply meant Andrew Pitkin, MBBS, MRCP, FRCA, an associate professor of anesthesiology and medicine, had to map how blood moved through the twins’ bodies so he could deliver anesthesia for the surgery as well as during MRI scans.
“I had been up to visit the babies, and was watching the two breathe,” Pitkin said. “One was breathing out while the other was breathing in. It was very inefficient, because each one was undoing what the other was doing.”
In the scanner, the girls had to hold completely still, and to breathe in sync in order for anesthetic to be delivered to them. This meant they had to be sedated, and that a ventilator had to control their breathing for them — but the hospital had only one ventilator that can be used in the MRI room, which uses an incredibly strong magnet to obtain imaging. Because of this, other equipment in the room when the scanner is in use has to be free of magnetic components.
Pitkin was out for a run one day, and the solution came to him: It would be much more efficient to ventilate the babies completely in sync, forcing the the babies to breathe together. Pitkin realized he could ventilate the babies the same way he ventilated an adult’s lungs separately: by using a special adapter used when anesthesiologists want to vent a single adult’s lungs separately.
For the surgery, the team decided to ventilate the babies separately.
“Once we knew we were going to vent the babies completely independently, we required two separation ventilation systems, two anesthesiology teams, which included two attending anesthesiologists and two residents — and two complete sets of monitoring equipment,” Pitkin said.
Those two teams of anesthesiologists, a team of surgeons led by Bleiweis, Islam and his support staff, and a plethora of nurses and residents spent hundreds of hours down to planning the surgery. Two days before the operation, the team practiced a dryrun of the surgery, planning which baby needed to be situated closest to the exit. The team decided where to situate the various equipment that would be used to keep the babies alive. They labeled all the machines and tubing for each baby blue or orange. The team knew where every cord would lie, where every scalpel would be placed. In the center of it all, two dolls, sewn chest-to-chest, were stand-ins for the twins.
On the day of the separation surgery, the operating room had been booked for 48 hours. Mark and Jackie prepared themselves for a long wait. “It was very intense and emotional, as you can imagine,” Jackie said.
The two had breakfast, arriving in the surgical waiting room at 10:30 a.m.
In the operating room, Islam first separated the chest and abdominal wall to access the liver. In previous scans, Islam was not able to get a clear picture of the internal anatomy of the twins’ large, shared liver, which was full of vital blood vessels.
Instead, Islam and his team had to separate the liver millimeter by millimeter, using an ultrasound during the operation to guide the division.
“We knew that blood vessels from Baby A were communicating with the blood vessels of Baby B such that blood was actually flowing through the liver of one baby and exiting the liver of the other through multiple connections,” Islam said. “So as we divided the liver tissue, we identified the vessels on the intraoperative ultrasound, and clamped them individually as we went across.”
Islam was able to separate the liver with minimal blood loss and each baby was able to get enough liver with adequate blood supply to be able to survive.
It was time for Bleiweis’ team.
They clamped the blood flow between the hearts. For the first time, each baby’s heart was pumping blood through a single body. Then, Bleiweis set to work separating those hearts.
Going into the surgery, Bleiweis knew that the hearts were connected very closely to two major blood vessels: the superior vena cava and the coronary sinus veins, which all deliver oxygen-poor blood to the right atrium. But even with this complexity, the surgery went smoothly.
“It was astronomical, how fast the surgery went,” Bleiweis said.
Miracle babies go home
Back in the waiting room, Jackie and Mark were given hourly updates. By 1:30 p.m., the twins were out of surgery. By 3:30 p.m., the twins were in their own, separate rooms in the pediatric cardiac intensive care unit.
Both Mark and Jackie marveled that their two babies were in separate rooms. But the separation was not without its difficulties. Scarlett needed to be isolated and ventilated because of her heart condition. Savannah was healthier, but both babies lost the ability to nurse because they had been intubated for their recovery from the surgery.
“In the NICU, it was easy. I only had to be in one place,” Jackie said. “But up here — we spend an hour here, an hour there, an hour here. I always felt guilty about wherever I’m not.”
But Jackie can now lay that guilt to rest. Savannah and Scarlett have both been released from the hospital and are home — Savannah in mid-September, and Scarlett a week later. They continue to recover and gain strength. An occupational therapist is helping both babies relearn how to nurse. Scarlett is building strength in preparation for a surgery to fix her heart defect, which Bleiweis wants to address after a year.
“In the world, there have not been many successful separations with a cardiac connection,” Bleiweis said. “It’s an exciting time for us as a team, for us as UF Health, and most importantly, it’s wonderful for this family and the babies.”
Savannah and Scarlett’s success confirmed something Jackie already knew — had known from the day she learned she was carrying conjoined twins. Her girls are strong.
“They’re survivors. From day one, they have just been miracle babies,” Jackie said. “They’re miracle babies, every step of the way.”