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hypoplastic left heart syndrome

Cardiology

A bridge to better outcomes

The bridges that connect UAB and Children’s of Alabama play a key role in the Bridging the Fetus collaboration.

By Conan Gasque

Among the crucial features of Children’s of Alabama’s Benjamin Russell building, which was added to the hospital’s campus in 2012, is a set of bridges that connect Children’s to the University of Alabama at Birmingham (UAB) Hospital. The two institutions have worked in partnership for decades, and the building and adjoining bridges further enhanced the relationship. Specifically, they allowed for patients to be transported efficiently from one hospital to the other when necessary for care. A collaboration called Bridging the Fetus takes advantage of this arrangement to help patients with congenital heart disease. It’s helped save many lives, even in some of the most dire situations.

The collaboration involves the Children’s cardiovascular ICU team, cardiologists, cardiac surgeons, UAB’s Maternal-Fetal Medicine team, and neonatologists. And the bridge, of course, which is perhaps the program’s most unique feature.

“The setup we have with the bridge is a big deal for us because it allows us to best take care of moms but also best take care of babies by being so close,” said Laura Brasseale, MSN, CRNP, chair of the Children’s cardiac fetal team.

Laura Brasseale, CRNP

Proper care for both the mom and baby is vital in these cases because many of the congenital heart diseases the team handles are identified prior to birth. Thus, the team must have a detailed plan in place for delivering the baby at UAB, then getting it to Children’s for care while the mom remains at UAB. Thanks to the bridge that connects the UAB Women and Infants Center to the Children’s Heart Center, the transfer can happen in mere minutes. As a result, Children’s and UAB have taken referrals from other parts of the Southeast in these cases where, in another city, the delivering hospital might be 20 minutes or farther away from the Children’s hospital. “It is very unique to our situation that we have all these resources literally connected to each other,” Brasseale said. Few other hospitals in the Southeast offer this type of setup.

The collaborative effort of Bridging the Fetus begins as soon as a patient is diagnosed with a congenital heart disease. Brasseale follows each case and takes information from the mom’s clinic visits with obstetricians and cardiologists. She uses this info to compile a list with data to help the team plan for the delivery. Before the baby is born, the team provides advice and counseling for the parents. They also arrange logistical services, such as transportation assistance, long-term stay options and other resources. Lactation specialists meet with the mom to discuss feeding options and the importance of breast feeding. Palliative care assists with end-of-life decisions, if necessary.

From the time the diagnosis is made, members of the team meet regularly to discuss details of the case and form a plan of care for the child’s delivery. “It just makes us aware so that we save beds for these neonates,” Brasseale said. “We get them transferred over from [the UAB Regional Neonatal Intensive Care Unit] as soon as possible so that we can plan their next steps after birth.”

Typically, the next steps involve taking the child across the bridge to be seen immediately by the heart team and undergo and operation shortly thereafter. The mother, meanwhile, remains at UAB to be cared for at the Women’s and Infants Center.

Cases like these are highly complex, Brasseale said. And the biggest challenge is the simple fact that no one knows when the baby will arrive. But Bridging the Fetus streamlines that for everyone. “We plan according to your due date, according to whether mom has any pre-existing conditions, whether she’s had other children before, where they live. All of that plays a factor in terms of how we plan for delivery on these babies.”  

Because of the planning, the team will know if the baby needs immediate care or if they can stay in the NICU for a few hours until a bed opens up. “It’s definitely a good heads up and provides a multidisciplinary approach for the family,” Brasseale said, “because Maternal-Fetal Medicine can refer them to genetic counseling and the neonatologist so the family can meet a wide variety of people before having the baby. So it can kind of calm nerves as far as that goes.”

The team also prepares the family by giving them hospital tours beforehand so they’ll get “an up-close look of who is going to be taking care of their baby and what their baby’s future looks like,” Brasseale added.

The most notable success stories owed to Bridging the Fetus involve babies with hypoplastic left heart syndrome with intact atrial septum. Babies with this condition lack intracardiac shunting, so oxygenated blood can’t reach the body. Most require immediate extracorporeal membrane oxygenation (ECMO) cannulation after birth to increase the chance of survival to palliative surgery.

Children’s and UAB have seen four such cases since they started Bridging the Fetus in 2016. Three of the babies were cannulated onto ECMO within 15 minutes of birth. Two had successful heart transplants. The results far exceed those at other centers, Brasseale says, and it’s due to the team’s coordinating and multidisciplinary approach. Just getting everyone on the same page has a complexity of its own, with multiple service lines involved and each one featuring 10-20 people with rotating on-call schedules. “There are so many players. I think that’s the biggest part,” Brasseale said. “So it’s just nice for the information to all be in one place considering there’s so many different people who need to be involved.”

The team also is participating in research related to its work with Bridging the Fetus. One study is taking a look at how delayed cord clamping among neonates with congenital heart disease can effect neurodevelopmental outcomes. Another is a collaboration with the Pediatric Cardiac Critical Care Consortium (PC4) and aims to advance understanding of the maternal-fetal environment and its influence on postoperative outcomes in children with congenital heart disease. “The role of maternal characteristics in postnatal outcomes is a major knowledge gap in the treatment of congenital heart disease,” Brasseale said. “Identifying these prenatal risk factors is the first step toward developing therapeutics to modify them.”

The team hopes these studies will pave the way to a better understanding of these highly complex neonates, which can only help further streamline their care and produce more positive outcomes.

“It’s really cool to see the team come together to provide the best care for the patient, because it’s a lot of different people from a lot of different disciplines and even hospitals,” Brasseale said. “But it’s very exciting to see a baby thrive that was really not destined to have a good outcome. So it’s very exciting to be a part of such a big team.”

Cardiology

Children’s of Alabama’s interstage home monitoring program growing

Brittany Abercrombie, NP, and Alan Brock, M.D., discuss the progress of a patient in the Hearts at Home program.

As the reputation of the Children’s of Alabama Pediatric and Congenital Heart Center of Alabama has grown, so has the success of its programs. Case in point—Hearts at Home, an interstage home monitoring program for any patient with single ventricle physiology who has undergone their first palliation procedure. In the last five years, the program has seen steady growth in the number of these patients, and leaders say the center’s reputation is among the reasons why.

“I think as a heart center in general, we’ve just had an influx of patients,” said Brittney Abercrombie, a nurse practitioner and the coordinator of Hearts at Home. “And so by default, that means that we are having more interstage patients.”

When Abercrombie moved into her role five years ago, Hearts at Home was caring for six to eight patients at a time. Now, she says they typically have about 13. Yearly, the program follows as many as 30, compared with 23-25 when she began. In the last couple of years, they’ve attracted more patients from outside Alabama, including children from Georgia, Tennessee and the Pensacola, Florida, area. Some of the program’s patients chose Children’s over other options in the region.

“I think they recognize that our outcomes here are some of the best in the Southeast,” said Alan Brock, M.D., the program’s medical coordinator. “And when they have the opportunity to look around and pick which program they want, I think patients are choosing us.”

As a result of the program’s success, hypoplastic left heart syndrome—a condition that brings many patients to the program—has become one of the most common forms of single ventricle congenital heart disease the hospital treats, Brock added. “I think it’s because we’re getting better at what we do and we’re saving a lot more lives now,” he said. “That is part of the reason that there are more patients coming into our program.”

What is Hearts at Home?

Through the Hearts at Home program, the families of patients with hypoplastic left heart syndrome and other forms of single ventricle congenital heart disease have access to education and technology that helps them to monitor and track their child’s heart health at home during the period between their first and second stages of palliation—procedures designed to repair their congenital heart defect. This time is tenuous for the child and often stressful for the parents, requiring a great deal of medical management, including monitoring, medications, adhering to strict feeding regimens, checking vital signs and having emergency access to equipment. “I think especially for these first-time parents, they don’t know what’s normal and what’s not,” Abercrombie said. “They’re not only learning to parent, but they’re learning how to parent a medically fragile child, so I think that’s a big challenge for them.”

There’s also the threat of morbidity, which is what led to the creation of interstage monitoring programs. The effort began in 2008 with the formation of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). Since then, interstage monitoring programs across the country have succeeded tremendously, dropping the interstage mortality rate by more than 40%, Brock said.

The programs are effective because of their focus on education, data and communication. The work begins before a family even leaves the hospital. While there, they go through extensive training to help them understand their child’s condition, how to manage it and the warning signs that might arise. Once they’re home, they track all of their child’s vitals—specifically heart rate, oxygen and saturations—through an app called Locus Health. This data is accessible by the patient’s care team, giving them a look at the patient’s trends and helping them to quickly identify any problems. “It helps us see the whole picture while they’re at home,” Abercrombie said. If any issues do arise, the family can connect with the care team via messages through the app, and providers can even use the app for telehealth appointments, if necessary.

In one case at Children’s, monitoring may have saved a child’s life. Abercrombie says the team detected a change in heart rate and some feeding intolerance, which, combined with the patient’s trends, indicated they needed medical attention. The team called the mom, got the patient in for a visit and prevented a medication overdose. “If we didn’t have [the monitoring], there’s a good chance that could have ended up in a mortality,” Abercrombie said.

The team

The Hearts at Home team includes, in addition to Abercrombie and Brock, cardiologists who see most of the interstage patients, a nutritionist who specializes in cardiovascular disease, a social worker and speech therapists. Nurse practitioners or intensivists are available to answer parents’ questions 24 hours a day, which can be reassuring. “It is just a very small group of people that are caring for these patients day in and day out, along with the family,” Abercrombie said. “And so I think that they feel a lot of comfort and confidence in knowing that there’s someone there to talk with them and help them throughout the day.”

This frequent communication can lead to close relationships between the parents and the care team—so much that when the child eventually “graduates” from the program (after having their second palliation procedure) and no longer has the same level of access to the team, the achievement is often bittersweet.

“It’s a good thing,” Abercrombie explains to the parents. “It means your baby has a much more stable heart. You shouldn’t need us as much. They can do a lot more normal baby things.”

“But [the parents] do have a little bit of sadness about losing kind of that access,” she added.

When a new patient enters the program, the team contacts their pediatrician to share information about the patient’s condition and explain how the program works and what to expect. They also reach to local EMS in the patient’s community to inform them that a congenital heart disease patient lives nearby so they’ll be prepared in case there’s ever an emergency.

Going forward, the program may expand to older patients. Brock hopes to focus future efforts on neurodevelopmental outcomes and “how these kids develop throughout the course of their single ventricle life,” he said. Nationally, the NPC-QIC recently merged with the Fontan Outcomes Network to form Single Ventricle One (SV-ONE) in an effort to follow these patients beyond their palliation procedures into their teens and beyond.

Cardiology

Flow restrictors offer hope for high-risk newborns

Flow restrictors, like the one pictured, are showing promise for helping patients with hypoplastic left heart syndrome.

Babies born with hypoplastic left heart syndrome, in which the left side of the heart doesn’t fully develop and thus can’t pump blood very well, typically require open-heart surgery soon after birth or a hybrid surgical approach combining stenting and open surgery to establish systemic blood flow and control pulmonary blood flow. Given the already fragile state of the infant, however, neither approach is optimal, and both have high mortality rates.

Doctors at Children’s of Alabama are helping these children by using an internal pulmonary flow restrictor created by modifying a microvascular plug. They form the device by cutting a tiny hole in the Gore-Tex covering of the microvascular plug originally designed to close blood vessels in children and adults. With this use of this device, they are able to postpone surgery for months until the infant is bigger and stronger while still controlling pulmonary blood flow in a completely non-invasive manner.

“The problem we’re addressing is over-circulation of blood to the lungs,” explained pediatric cardiologist Mark Law, M.D. “Traditionally, it requires open-heart surgery to place a band around the pulmonary artery to reduce blood flow. However, this surgery is invasive and can be very stressful for the baby.”

Now, Law and his team are able to treat the infant in the cath lab rather than an operating room. There, they thread the plug through the femoral or internal jugular vein into the pulmonary artery to partially restrict excessive blood flow to the lungs. “It’s a far less invasive procedure and the recovery is faster,” he said. It also allows the surgeons to operate without scar tissue from a previous surgical procedure.

This procedure enables babies who are too fragile for surgery to safely grow stronger before undergoing their first operation. In one study, 10 out of 13 infants treated with this technique survived to their next surgery, typically performed within two to six months of the catheter procedure. When compared to historical data from similar high-risk patients who underwent traditional procedures, the new technique was associated with significantly better six-month survival rates after adjusting for other risk factors.[1]

Importantly, the baby’s pulmonary arteries grow over that time, which is crucial for their long-term outcomes. In addition, the devices can be easily removed during subsequent surgical procedures.

Law and his team have presented a series of five cases in which they used the technique on babies who were too medically fragile for surgery. All survived and all were later able to undergo the needed open surgery. “We’re confident some of these babies wouldn’t have made it otherwise,” he said. “But because they’re older, bigger, stronger, and breathing on their own,” the procedure was much less risky.

Still, challenges remain. The device must be customized for each patient, and its small size limits its use to very young infants.

Nonetheless, pediatric cardiologists across the country are adopting the procedure, Law said. “We’re all learning from each other and sharing data to refine the technique.”

“This technique represents a shift in how we think about treating congenital heart defects,” Law said. “The ability to delay surgery and avoid scar tissue is a game changer.”


[1] Sperotto F, et al. Circ Cardiovasc Interv. 2023;16:e013383. DOI: 10.1161/CIRCINTERVENTIONS.123.013383