Browsing Category

Cardiology

Code Committee Brings Innovation and Improvements

HC3 is led by Dr. Ahmed Asfari (top row, fourth from left) and Ashley Moellinger (top row, third from left).

Since the inception of the Heart Center Code Committee (HC3) in 2014, Children’s of Alabama’s cardiac arrest rate in the Cardiovascular ICU has fallen nearly 50%. The impact this committee has had on cardiac arrest reduction has come from numerous quality improvement initiatives and safety changes. In 2022, some of the initiatives Children’s implemented include the development of four guidelines, three communication enhancement tools, checklists and numerous safety changes.

Whenever a patient goes into cardiac arrest or a near miss is encountered, HC3 discusses the case. This multidisciplinary committee is composed of physicians, nurse practitioners, bedside nurses, respiratory therapists, cardiovascular operating room staff, chaplains and leadership from the heart center. HC3 meets every other week to evaluate each case, identify what was done well and areas for improvement.

Nurse practitioner Ashley Moellinger, CRNP, MSHQS, and cardiac intensivist Ahmed Asfari, M.D., who participate in quality improvement initiatives within the heart center, began leading the committee in 2021. They’ve focused on three key areas: education, inclusivity and innovation.

“We have unique patients with really complicated conditions that can be challenging to understand,” Moellinger said. “Whenever we review the event, we look for areas where there’s a knowledge gap or a need to enhance skills.” Then the team sends education briefs to the nurses to highlight committee findings.

Every nurse in the department is involved with the committee. “Really, the committee is owned by the nurses,” Moellinger said. An elite team of nurses reviews each case, interviews those involved and develops a presentation to tell the story of the event. Then, the committee decides together what they need to change. “And that’s where the education rollout comes into play,” Moellinger said.

The committee uses artificial intelligence and near-real-time analytic algorithms to analyze cardiac arrest and near-arrest events. “When you’re talking about a cardiac arrest, everyone’s recollection is going to be different,” Asfari said. With the platform, “we can use objective data to show the patient’s course.” It also allows the team to view vital signs in a continuous manner on one screen.

“One of the things we are most proud of is the ‘green’ epinephrine action plan,” Asfari said. When a patient goes into cardiac arrest, epinephrine is one of the first medications administered, but it takes time to prepare the drug. Shorter time to administer it is associated with better outcomes. The action plan calls for prepared epinephrine at the bedside and includes standing orders for the nurse to administer it once the patient’s vitals reach a certain threshold. “It’s made a huge impact on our patient care,” he said. “Cardiac arrest is a real problem for children with cardiac disease because they are so fragile,” Asfari said. “Improving resuscitation and, more importantly, preventing the arrest can improve the outcome.”

August 22, 2023 by childrensal

Cardiology

New Registry Studying Long-Term Outcomes of Heart Procedures

A new national registry is tracking patients’ progress over time following heart procedures as neonates.

Children’s of Alabama has joined a new national registry managed by the Congenital Cardiac Research Collaborative (CCRC), attempting to answer the question: What is the best way to treat ductal-dependent pulmonary blood flow congenital heart conditions?

Conditions such as Tetralogy of Fallot, critical pulmonary valve stenosis and complex single-ventricle arrangements can be treated with various interventions, both percutaneous and surgical. “We’re looking at all neonates (first 30 days of life) who require a procedure, so they get enough pulmonary blood flow to survive,” Children’s pediatric cardiologist Mark Law, M.D., said. “The goal is to take this broad grouping of patients and understand how they do, not just with their first intervention or even their second intervention if they need one, but how do they do as they grow up?” This question of long-term outcomes hasn’t been studied, he said.

“In our field, we tend to be very focused on the short-term, procedural outcome. We have a disease, we have a problem. We do an intervention, and we look at that intervention or outcome. But we haven’t looked at overall survival and longevity; some of the more nuanced outcomes, including quality of life,” Law said.

The idea came about as the result of the Comparison of Methods of Pulmonary Blood Flow Augmentation in Neonates: Shunt Versus Stent (COMPASS) trial, which is comparing overall survival and post-surgical complications, hospital length of stay and quality of life between a surgically inserted shunt and one threaded into the ductus arteriosus, the artery that connects the aorta and pulmonary artery in newborns. That trial, in which Children’s is also participating, will follow patients for two years. Part of the funding enabled the development of the registry. Participation requirements for the registry are also broader than for the clinical trial, opening it up to a larger population. It will also be part of Cardiac Networks United, which manages numerous other registries.

The CCRC registry, which currently has 14 participating institutions, started in 2022 and has been collecting data for less than a year. Children’s was among the first institutions to join. The registry will track how patients fare over time and provide valuable information about which treatment pathway is best for which condition and which child. Children’s has been enrolling patients since October 2022 and currently has about 11. The goal is 15 to 20 a year. Registry information on each participant will be updated annually, but Law said that’s one of the challenging parts of the study—for instance, if families move out of the area and are seen at other institutions. That challenge, Law said, could be overcome through the robust electronic health records children’s hospitals use to share information.

August 22, 2023 by childrensal

Cardiology

Society of Thoracic Surgeons National Database Reinforces Cardiothoracic Program’s Success

The Children’s of Alabama cardiothoracic surgery program is among the nation’s best in expected-to-observed mortality rate.

A pivotal way to measure a program’s success is by comparing it to others. And according to outcomes recorded in the Society of Thoracic Surgeons (STS) National Database, the Children’s of Alabama cardiothoracic surgery program continues to surpass national trends.

“In 2023, quality is the big catchphrase, but the only way we know we’re providing the best care is to look at results,” Robert Dabal, M.D., chief of pediatric cardiothoracic surgery at Children’s said. “The database allows us to analyze data over lots of different time frames—months or years. It gives us the ability to look at results over time to make sure we’re always improving.”

Established in 1989, the database has become the gold standard for clinical registries, containing data on more than 8.8 million patients and 4,300 surgeons. An important subset is the STS Congenital Heart Surgery Database, which has captured records from more than 600,000 congenital heart surgeries in North America with more than 1,000 physicians. It monitors metrics such as patient complications, reoperations and deaths.

Cardiothoracic surgeons at Children’s perform about 440 congenital heart surgeries each year. The database shows that patients who might not survive in other hospitals are surviving at Children’s, Dabal explained. “Our observed mortality is less than the expected mortality, which is right where we want to be.”

Additionally, the most recent analysis of all pediatric cardiothoracic surgery programs in the Southeast shows Children’s has the second-lowest rates in the region—and one of the lowest rates in the country—in that same expected-to-observed mortality category. Combining the STS data with information culled from several other databases helps provide both a big-picture and granular assessment of Children’s progress and where it may still fall short.

“Perfect is not a realistic goal in medicine, but continually improving your results is an attainable goal,” Dabal said. “That’s why we’re always looking at these results.”

Even the best databases, however, can’t capture all factors related to a program’s success or goals. Along those lines, Dabal hopes future iterations can track young congenital heart patients’ long-term outcomes, not just perioperative data points.

“Most of our patients survive their operation, so the larger pediatric cardiothoracic community is focused on what happens to them one year, five years or 10 years out,” he said. “We want patients to survive surgery, of course, but also to lead normal lives—to go to school, get married and have children of their own. Our bigger focus is looking at the long-term quality of the outcomes we’re providing.”

August 21, 2023 by childrensal

Cardiology

Communication, Metrics Drive Quality Improvement in Cardiothoracic Surgery

Ashley Moellinger (left) leads QI projects for the Children’s of Alabama cardiothoracic team.

Two years after launching a quality improvement (QI) project to reduce re-interventions for one of the most complex heart surgeries performed in newborns, the cardiothoracic team at Children’s of Alabama is ready to call it a success.

The project, which is part of the National Pediatric Cardiac Quality Improvement Collaborative, was designed to understand why re-interventions occurred after the Norwood procedure, which involves constructing a new, larger aorta for babies born with hypoplastic left heart syndrome. Patients who don’t require an intervention during the hospitalization after their initial surgery have a mortality rate of about 6% while those who require another surgery or catheterization procedure have a 26% mortality rate.

Children’s slashed its Norwood re-intervention rate and lengths of stay by:

Improving communication among team members
Identifying and targeting metrics
Focusing not on “finger-pointing,” but on how to improve the process

Overall, the unit has seen a 30% reduction in re-intervention in the first phase of the surgery and an 18% drop in the average length of stay, as well as significant improvements in other quality markers, including days to extubation and the use of certain medications like opioids and vasopressors. In addition, interventions for post-operative bleeding fell from 18.5% to 4.2%.

“The two main functional components of what we’re doing are situational awareness and communication,” cardiovascular intensivist Hayden Zaccagni, M.D., said. Together, they allow for more scripted and pinpointed conversations about potential complications, he said. “The communication factor is the most important thing—making sure that all the different disciplines that care for these children have the same kind of knowledge umbrella and communicate about it.”

Also important is having clear expectations about the post-operative period. A high-level map at the bedside clearly shows those metrics on a day-by-day basis for cardiovascular, neurological, respiratory and feeding specialists.

The third piece, according to Ashley Moellinger, RN, CRNP, who co-leads QI initiatives in the department, is holding small group-focused meetings to dissect re-interventions. “We get together those involved and say, ‘How can we prevent this from happening again?’” she said.

As with any QI project, data rules. For instance, one of the most common complications the team saw was post-surgical bleeding, so they developed guidelines to quantify the amount of bleeding in the OR not just in volume, or millimeters, but by measuring the blood coagulopathy, or impaired clotting. That led to the discovery that the lab instrument used for the measurement was outdated. And that, in turn, provided hospital administrators with reason to update the machine because they could see the potential impact on patient outcomes.

While the project is a success based on the numbers, it’s also a success in a less tangible way, Zaccagni said. “The morale of the unit, something we haven’t been able to objectively measure, is also improved.” He thinks it’s due to having a better understanding of where patients have come from medically and where they are now. “There’s this huge sense of collaboration.”

The team hopes to apply the lessons learned and new systems to other cardiothoracic surgeries.

January 16, 2023 by childrensal

Cardiology, Inside Pediatrics

Xenotransplantation Takes Steps Toward Clinical Trials

_{^{Children’s of Alabama cardiothoracic surgeon David Cleveland, MD, MBA, leader of the xenotransplantation program at Children’s of Alabama and the University of Alabama at Birmingham (UAB).}}

News that surgeons at the University of Maryland Medical Center had implanted a genetically modified pig’s heart into a human rocked the medical world earlier this year. But it didn’t surprise Children’s of Alabama cardiothoracic surgeon David Cleveland, MD, MBA, who is leading a similar xenotransplantation program at Children’s and the University of Alabama at Birmingham (UAB).

The program focuses on developing genetically modified solid organs from pig models for transplantation. To date, Cleveland’s team has successfully transplanted a genetically modified pig kidney into a brain-dead patient. The kidney produced urine.

Three years ago, Cleveland presented preliminary results from a study showing little reactivity in an infant’s blood to cells from a triple-knockout (TKO) pig. The pig had been genetically modified to delete the three major antigens that react with natural human anti-pig antibodies. Even those human cells that did react demonstrated a very mild reaction.

Back then, Cleveland said the next step was a transplant in a non-human primate, something required before the FDA would approve human trials.

Now he’s done it. So far, Cleveland and his team have implanted four infant baboons with the genetically modified pig hearts, with one of the animals living as long as eight months. In humans, the goal isn’t to have the heart last a lifetime but, rather, just long enough for a human heart to become available for transplant.

“I think we have to consistently demonstrate a four-to-six-month survival in non-human primates before the FDA approves a clinical trial,” Cleveland said. The team plans to implant more animals with the hearts this fall and is working on several grants to continue funding the study. Cleveland hopes to be able to submit the design for a clinical trial to the FDA sometime in 2024. In the meantime, he and his team published the results of the first baboon study in The Annals of Thoracic Surgery.

It’s quite possible, however, that the first transplant might occur outside a clinical trial with a humanitarian device exemption from the FDA. The authorization allows a device—in this case, the pig heart—to be used without showing effectiveness in formal clinical trials. That’s how the patient at the University of Maryland Medical Center was able to receive his heart.

“But our goal, ultimately, is to participate in an NIH-funded clinical trial,” Cleveland said, adding that those trials are conducted in more patients with strict safety monitoring and comprehensive data collection.

September 2, 2022 by childrensal

Cardiology, Inside Pediatrics

Pea-Sized Device Proves Lifesaving for the Tiniest Babies

^{Mark Law, MD, and William McMahon, MD, are pediatric cardiologists at Children’s of Alabama.}

Imagine a baby who weighs less than two pounds. Now imagine that baby has a life-threatening, congenital heart condition called patent ductus arteriosus (PDA), in which the opening between the two blood vessels leading from the heart hasn’t closed. Now imagine performing open-chest surgery on that tiny neonate.

“Patent ductus arteriosus is a significant problem for many premature babies,” pediatric cardiologist William S. McMahon, MD, said. Usually, however, it closes on its own. That’s not the case in micropreemies, defined as babies born before the 26th week of pregnancy or weighing less than 28 ounces (700-800 grams).

PDA affects up to 80% of micropreemies, keeping them on ventilators longer and increasing the risk of lung disease. It can also lead to gastrointestinal problems and affect neurological development. That’s why closing the opening is critical.

Yet the risks of operating on such a tiny preemie are nearly as great as the risks of PDA, said pediatric cardiologist Mark Law, MD, so few surgeons perform the surgery.

“It’s a hard decision to make because many babies will survive with the PDA, or it may get smaller,” he said. While there is medication available to treat PDA, it’s often unsuccessful and has its own risk of kidney complications. Still, given the risk of open-chest surgery in such a small baby, “many surgeons would choose to continue to manage the baby medically,” Law said.

That changed in 2019 with the approval of the Amplatzer Piccolo Occluder, a pea-sized device that a cardiologist can snake through the femoral vein and into the heart via a catheter. “With this device, we now have the opportunity to intervene in a minimally invasive fashion with much less insult to the baby and a much lower risk of serious complications and still get the PDA closed,” McMahon said.

The occluder also allows them to avoid the artery, which they use to close PDAs in full-term or larger premature babies. In such tiny babies, however, putting a catheter through the artery could cause significant damage. “It’s part of the miracle of this device that we don’t have to access the artery,” McMahon said. “It’s much safer going in through the vein.”

Unlike most catheterization procedures, implanting the occlusion device requires a team of specialists, including neonatology, the cardiac catheterization team and anesthesiology, among others. “They’re fragile babies at risk just for the exposure and movement,” Law said. To date, the team has implanted the device in more than 20 babies.

“Not only will the device keep more of these tiny babies alive,” he said, “but, hopefully, it will result in more micropreemies who get to the end of the first year of life with fewer difficulties like chronic lung disease, GI tract disease and neurodevelopmental problems.”

“Any little thing that you can help these babies do better in the first year of life, so they are less dependent on medical technology and even less damaged from the care required to keep them alive,” McMahon said, “ends up in a better baby in the future.”

August 18, 2022 by childrensal

Cardiology, Inside Pediatrics

Which Device is Most Effective at Treating Cyanotic Congenital Heart Defects?

_{^{Mark Law, MD, is a pediatric cardiologist at Children’s of Alabama.}}

Doctors at Children’s of Alabama are working to answer a question that could help save the lives of infants with cyanotic congenital heart defects. In a new study, they’re asking which device is better: the Blalock-Taussig-Thomas (BTT) shunt or the ductus arteriosus (DA) stent.

The study is called the COMPASS Trial, short for Comparison of Methods of Pulmonary Blood Flow Augmentation in Neonates: Shunt Versus Stent. Children’s pediatric cardiologist Mark Law, MD, is the leading institutional investigator for Children’s.

“This is a very interesting study for our field,” Law said. “We have very few randomized controlled trials to show us which treatment strategy is best. There’s some thinking that DA stents may require additional, non-emergent interventions after they are implanted, and some data suggests the shunts may require a higher number of emergent interventions early on. We just don’t know.”

Of the two devices, the BTT shunt has been around longer. Developed in the 1950s, the shunt was the subject of a made-for-TV movie called “Something the Lord Made.” At the time, the shunt was lifesaving for babies with Tetralogy of Fallot. Today, it is used to manage babies with various heart defects that result in cyanosis, which causes insufficient blood flow from the heart to the lungs, resulting in low oxygen levels. These infants are often referred to as “blue babies.”

“It was field-changing when it was developed,” Law said of the shunt. However, it also requires open-chest surgery, which can lead to more complications and a higher risk of death.

DA stents, developed more recently, a much less invasive. A stent can be threaded into the ductus arteriosus, the artery that connects the aorta and pulmonary artery in newborns. Once in place, the stent is expanded to prevent the DA from closing.

Both the BTT shunt and the DA stent provide a stable source of blood flow to the lungs until the baby is large enough for an operation to repair the heart defect. Both options are relatively safe and effective, but both also present the risk of complications and death.

No one knows which is better because, until now, there’s never been a large study directly comparing the two procedures. The COMPASS Trial is a multi-institutional, NIH-funded study with the Pediatric Heart Network to see if one is safer and more efficacious than the other or if they are equally effective.

Investigators hope to enroll 300 newborns over the next two years and follow each through the first year of life. Most centers, including Children’s, should start enrolling patients this fall.

Overall survival and post-surgical complications are key endpoints for the study, as well as hospital length of stay and quality of life. Investigators also hope to learn which approach is best based on the child’s anatomy.

“We hope to come out the other end smarter and knowing which is the best therapy,” pediatric cardiologist William McMahon, MD, said. “Because right now, we just don’t know.”

August 18, 2022 by childrensal

Cardiology, Inside Pediatrics

Harmony Device Saves Children from Open-Heart Surgery

In July 2021, interventional cardiologist William McMahon, M.D., Mark Law, M.D., and the Pediatric Cardiac Catheterization Lab team at Children’s of Alabama, snaked a catheter device through a vein in a 16-year-old girl’s leg up to her heart and replaced the valve. She went home the next day with just a small scar on her leg that will eventually become invisible. The U.S. Food and Drug Administration approved the device, called the Harmony transcatheter pulmonary valve, in March. Drs. McMahon and Law were the first cardiology specialists in a 10-state region to use it.

Fewer than 20,000 children are born each year with Tetralogy of Fallot, a condition marked by four major heart defects: ventricular septal defect, or a hole in the two lower chambers of the heart; a narrowing of the pulmonary valve and main pulmonary artery; malaligned aortic valve; and ventricular hypertrophy, or thickening, of the right ventricle. These children often need surgery soon after birth and a pulmonary valve replacement by the time they’re adolescents or young adults.

In the past, that meant another open-heart surgery and time spent on cardiopulmonary bypass, which carries significant risks of complications; a week or more in the hospital; scarring; weeks of recovery at home; and a low but real risk of death. In addition, because the children have already had major heart surgery, scar tissue from the previous procedure makes the valve replacement even more difficult.

In July 2021, however, interventional cardiologist William McMahon, M.D., and his colleague Mark Law, M.D., together with the Pediatric Cardiac Catheterization Lab team at Children’s of Alabama, snaked a catheter device through a vein in a 16-year-old girl’s leg up to her heart and replaced the valve. She went home the next day with just a small scar on her leg that will eventually become invisible.

The U.S. Food and Drug Administration had just approved the device, called the Harmony transcatheter pulmonary valve, in March. Drs. McMahon and Law were the first cardiology specialists in a 10-state region to use it. While similar devices have been available for pulmonary valve replacement, few children with Tetralogy of Fallot qualified because of their previous surgeries. Now, Dr. McMahon estimates that four out of five children with the condition will qualify.

The new procedure is a game-changer, he said. “We have many patients who live in fear of that surgery because they’ve been told they need another open-heart surgery since they were 8 or 10. We certainly have some patients who reasonably don’t want to do it and some who put it off. That becomes a problem because it means their heart is working harder.”

Dr. McMahon says the advantages to the Harmony device are obvious: “There’s a quicker recovery; lower risk of major complications and death; and they’re able to get on with their lives sooner.” The team has completed 10 procedures so far with no complications other than some arrhythmia that resolved with treatment.

Some patients returned to work or school three days after the procedure. And while the valve won’t last forever, Dr. McMahon expects a new valve could be inserted within the old one in the same manner. “That’s the overall goal of these valves,” he said. “To reduce the total number of heart surgeries that our patients need during their lifetime.”

William McMahon, M.D., is an interventional cardiologist at Children’s of Alabama and a professor in the Division of Pediatric Cardiology, University of Alabama at Birmingham Department of Pediatrics.

February 15, 2022 by childrensal

Cardiology, Inside Pediatrics

Improving Quality and Outcomes in Cardiology

Ashley Moellinger, RN, CRNP, Cardiovascular Services, Children’s of Alabama

Children’s of Alabama is deeply committed to continual improvement in every part of the care pathway. Two quality-improvement projects in cardiology are already showing the results.

Handoff of Care

Medical errors are the third-leading cause of death in the United States.[1] The Joint Commission reports that two-thirds of serious medical errors, or “sentinel events,” are tied to poor communication, and half involve communication during care handoff, such as when a patient is transferred from the intensive care unit (ICU) to surgery or back.[2]

The handoff is an important faultline for miscommunication that can lead to patient harm, said Children’s of Alabama cardiovascular intensivist Hayden Zaccagni, M.D. It’s not just communication between the intensivist and the surgeon; it involves the pediatric anesthesiologist, bedside and surgical nurses, advanced practice practitioners, and respiratory therapists.

“It’s a big team that cares for these patients,” Dr. Zaccagni said. Research shows that standardizing the handoff from the ICU to the operating room increases communication without delaying surgery and increased provider satisfaction and patient readiness for surgery while reducing errors.[3]^,[4]

The cardiology service didn’t have standardized protocol for handoffs, so Dr. Zaccagni, together with Ashley Moellinger, RN, CRNP, leda quality-improvement (QI) project to develop a process that prioritized clear, concise, and consistent communication from the cardiac ICU to the operating room or catheterization lab.

They started with a survey of 82 staff members, which found that 69 percent had experienced a safety event related to inadequate handoff. The survey also showed that communication was the primary barrier to transition followed by organizational barriers.

The team developed a tool and process for handoffs that involved all clinicians who interacted with the patient. “This multidisciplinary approach is so important,” said Moellinger.

Now, the night prior to surgery, the nurse practitioner, bedside nurse, and respiratory therapist complete a data form on the patient. The next day, the entire team meets at the bedside to review the form and bring up any concerns. “A big part of this is around situational awareness, or concerns we have about the patient that might not be obvious from reading through the chart or notes,” said Moellinger.When the patient is transferred, the team verbally goes through the tool again to ensure there are no outstanding questions or changes in condition.

The team is also tracking what it calls “moments of clarity”—when the process unveiled a potentially problematic issue such as a difficult airway, unavailability of vasoactive drip, patient cardiac arrest the prior night, or airway management for a patient with worsening oxygen levels.

The goal, or “smart aim,” was to demonstrate a standardized handoff in 80 percent of transition interactions, with 80 percent completion of patient data points by December 2021, and 95 percent compliance by July 2022.

Reintervention Reduction

This reintervention reduction QI project focuses on the most complex cardiothoracic surgery performed in newborns. Called the Norwood procedure, the surgery involves constructing a new, larger aorta for babies born with hypoplastic left heart syndrome. Nationally, patients who don’t require an intervention after their surgery have a mortality rate of about 6 percent compared to the 26 percent mortality rate in those who require another surgery or catheterization procedure.

The project, which is part of the National Pediatric Cardiac Quality Improvement Collaborative, was designed to understand why reinterventions occurred and identify opportunities to recognize the warning signs early in the post-operative period.

The Children’s team first performed a root-cause analysis of the 69 patients who required additional interventions between January 2015 and June 2020. That involved identifying what triggered the complication and how it could have been prevented. Of the 69 patients, 23 (34 percent) required an unplanned cardiac surgery or catheterization while hospitalized after the first-stage operation. Half of the surgical interventions were to explore unexplained bleeding, and half of the catheterization interventions were for conduit stenting to improve pulmonary blood flow. Fewer than five patients (12.5 percent) who required a reintervention died compared to none in the other group.

Reviewing the entire care pathway from the cardiovascular ICU to the operating room and back, including rates of post-operative bleeding and the timing for administering blood products, “we were essentially able to come up with a solution that we should communicate more effectively between team members in the operating room,” said Dr. Zaccagni. One way to improve communication is to wait at least 30 minutes in the operating room after closing the sternum to estimate chest tube output. Another is to standardize blood work when a patient is bleeding in case it’s due to a rebound effect of blood thinners given during the surgery. In addition, the team developed a standardized tool for the post-operative debriefing with the entire team.

The efforts are already paying off, said Moellinger, with fewer reinterventions since they began in 2020. “Standardization and, thus, reducing variation in everything we do is an important component for the best outcomes,” she said.

[1] Makary MA, Daniel M. Medical error—the third leading cause of death in the US. BMJ. 2016;353:i2139.

[2] The Joint Commission. Inadequate hand-off communication. Sentinel Event Alert. September 12, 2017. Issue 58.

[3] Caruso TJ, Marquez S, ,Luis J, et al. Standardized ICU to OR handoff increases communication without delaying surgery. Int J Health Care Qual. 2017;30(4):304-311.

[4] Joy BF, Elliott E, Hardy C, Sullivan C, Backer CL, Kane JM. Standardized multidisciplinary protocol improves handover of cardiac surgery patients to the intensive care unit. Pediatr Crit Care Med. 2011 May;12(3):304-8.

February 15, 2022 by childrensal

Cardiology, Inside Pediatrics, Pulmonology

Saving Children with Pulmonary Hypertension – One Patient at a Time

Children’s of Alabama cardiologist Frank Bennett Pearce, M.D.

When the cardiology team at Children’s of Alabama heard the family history of a 6-year-old boy who presented with an episode of syncope, they knew immediately what was wrong. His father had undergone a double lung transplant at the University of Alabama at Birmingham to cure his pulmonary hypertension (PH). Now his son had been diagnosed with the same thing.

But that wasn’t the only problem. The boy had also developed a supraventricular tachycardia requiring radiofrequency ablation, which was successful.

“So we cured that,” said Children’s cardiologist Frank Bennett Pearce, M.D., the boy’s cardiologist. But then the patient continued having episodes of syncope, particularly during exertion. “When that happens in patients with PH, it’s because the blood can’t get through the lungs to the left side of the heart, limiting cardiac output,” said Dr. Pearce. To address that problem, Dr. Pearce and his team performed an atrial septostomy, creating a tiny hole between the atria in the atrial septum. Second problem fixed.

Discharged on oral medications, the child did well with close follow up for several years, said Dr. Pearce, although he was vulnerable to pneumonia and other infections.

Then in 2020, at age 13, he took a turn for the worse. “There are three principal metabolic pathways involved in treatment of PH,” said Dr. Pearce. Two—endothelin and phosphodiesterase—have effective oral drugs for treatment. The third, the prostaglandin pathway, is more difficult to address, he said. In the past, it required a central line for IV infusions of treprostinil, a prostaglandin pathway medication. “Most families are very reluctant to go to the central line because it creates major problems in their lifestyle and is a quantum leap in terms of the negative effects on these children,” he said.

However, treprostinil can also be administered subcutaneously through a small catheter and external pump, much like an insulin pump. Unfortunately, the day the teen was scheduled for cardiac cath and initiation of subcutaneous treprostinil, he became very cyanotic. “We didn’t think it was safe,” Dr. Pearce said. Instead, the boy was admitted to the CVICU on inhaled and oral prostacyclin inhibitors. Despite increasing the dosage, his disease progressed. Finally, the team put him on the intravenous form of treprostinil, and he improved. Eventually, they were able to transition him to the subcutaneous form of the drug via the pump, and he became the first patient at Children’s to be initiated onto subcutaneous treprostinil.

He’s now home and undergoing evaluation for a lung transplant. “He’s a typical teenage kid but able to deal with all these challenges and keep a pretty good attitude, thanks to support from his family,” said Dr. Pearce. “He just hangs in there.”

August 27, 2021 by childrensal

Older Posts