“There is no doubt the 3D print of Ivy’s heart that surgeons used to plan and prepare for her procedure contributed to the positive outcome and long-term success of the surgical repair,” said Steve Muyskens, M.D., cardiologist and medical director of cardiac MRI and the 3D Lab at Cook Children’s Medical Center–Fort Worth. “Three-dimensional printing takes away the guesswork for physicians, allowing them to completely visualize a patient’s anatomy and unique anomalies and map their intervention before they get to the operating room.”

Ivy, now 10, was born with congenitally corrected transposition of the great arteries (ccTGA) in which the two lower pumping chambers, or ventricles, of her heart are reversed. In a normal heart, the right atrium sends unoxygenated blood through the right ventricle, which pumps it through the pulmonary artery into the lungs. The blood is then oxygenated and flows back into the left atrium. From there, the left ventricle pumps the blood into the aorta where it is carried throughout the body.

In Ivy’s heart, however, the ventricles were switched. This meant the normally weaker and thinner-walled right ventricle was doing the high-pressure job of pumping blood throughout the body via the aorta—a function best suited for the thicker, stronger left ventricle. This inefficient pumping function put her at high risk for heart failure, irregular heart rhythms and sudden cardiac death. Ivy’s heart was further complicated by having a hole in the bottom chamber and a very small pulmonary valve, causing limited blood flow to her lungs. Ivy’s heart defects were unable to be repaired in infancy, so she had a palliative procedure at 2 weeks old to stabilize her condition while she grew.

Then, at 19 months old, Ivy became a candidate for the Nikaidoh procedure, also known as an aortic translocation. The procedure is named after Hisashi Nikaidoh, M.D., a retired Cook Children’s cardiovascular surgeon and innovator. Dr. Nikaidoh developed the complex open-heart surgery that reverses the translocation and reconstructs a more normal ventricular and arterial layout. He assisted Vincent Tam, M.D., during the surgery. Dr. Tam is a pediatric cardiothoracic surgeon and Cook Children’s medical director of Cardiothoracic Surgery.

To prepare for the procedure and to explain the complex surgery to Ivy’s family, Dr. Tam used a 3D printing of Ivy’s heart that was created in Cook Children’s 3D Lab. The printed heart allowed Dr. Tam to visualize Ivy’s exact anatomy so that he knew what to expect going into surgery.

It also helped Ivy’s family understand her complicated diagnosis and Dr. Tam’s plan to save her life.

“I've noticed over time that having these 3D models helps parents and other care providers, like nurses, better connect with the plan of care,” Dr. Muyskens said. “If you think about it from a parent standpoint, you're trusting this person, a surgeon or even just a cardiologist like myself, to take your child on a journey that's going to make them as happy and healthy as possible, oftentimes with no understanding of this very complex issue. So if you're able to then bridge that gap and have the surgeon show a parent an actual 3D model they can hold in their hands and say, ‘Here's your child's heart. This is why it's so complex. This is why it's different. There’s not a template for this, but this is what we think we can do. Let's walk through this together and figure out how we want to care for your child as a medical team and a family.’ I think that really helps connect those two pieces when you have a really unusual case.”

It certainly did for Ivy’s mom, Elizabeth. She says the physical model of Ivy’s heart helped her understand her baby’s complicated condition and Dr. Tam’s surgical plan. Having that knowledge was empowering and compelled her to hold on to hope during her daughter’s day-long surgery.

Surgical Solutions

In addition to his work in the 3D Lab, Dr. Muyskens is a cardiologist with a full patient load. He often partners with cardiothoracic surgeons to repair congenital heart defects and cardiovascular anomalies in his patients. When 2D imaging was the only resource for visualizing and understanding a patient’s anatomy, it wasn’t uncommon for Dr. Muyskens to hear surgeons say, “We’ll have to see for sure when we get in there,” when discussing a surgical intervention. This element of the unknown sparked a vision to create the 3D Lab to give physicians a deeper, more detailed look into the problems they were working to solve.

“I always felt like we need to be doing more and wondered how I can further the offering we have to help surgeons prepare for complex surgeries,” he said. “Your heart is about the size of your fist. So if you think about the size of a baby’s heart, and then trying to do open-heart surgery and move coronaries and great vessels and create new pathways for blood flow, it's a very complex surgery. By 3D printing the heart, we can have a much better understanding of those relationships prior to surgery. That's how we got started. Now we have three printers, surface scanners and virtual and augmented reality options. We've expanded in a lot of ways.’

While the 3D Lab originated with cardiology, its tools and resources are now available to multiple specialties. In the 10 years since the Lab’s debut with Ivy’s surgery, specialties such as plastic surgery, craniofacial surgery and orthopedics now utilize its capabilities to create precise, unique copies of spines, facial features, bone structures and more. These models can be printed in multiple colors and materials that are engineered to be more tissue-like, whether that’s bone or soft tissues. This creates a more realistic feel for a surgeon who wants to practice drilling into bone or cutting into tissue prior to a surgery. 

Practice Makes Perfect

The 3D Lab has expanded its pre-operative practice options, too.

“We're still doing the printing that we have done, but we've also moved a lot more into virtual surgical and interventional planning,” Dr. Muyskens said. “For complicated cases, we want to have a pre-operative or interventional plan in place so we know, for example in the catheterization lab, the exact camera angles we need and what size stents we should use. Now we can virtually place stents during simulation and review our work. Sometimes we go down the pathway virtually and we realize that it's probably not a great case for the cath lab because it’s actually much trickier than the images would've suggested. So we will then send those patients to surgery. That's kind of been our current frontier.”

Much like a pilot in a flight simulator, virtual reality allows surgeons to see internal structures, plan their approach and practice their cuts in the Lab rather than saving these critical and time-consuming decisions for the OR when the patient is under anesthesia and the surgical site is open. This tangible experience outside of the OR builds valuable knowledge and spatial awareness of a patient’s unique anatomy, increases surgical accuracy, removes the element of surprise and decreases time under anesthesia—all precursors to improved outcomes for patients. 

Mirror Image Models

The addition of a 4B printer allows the Lab to produce biocompatible materials that can be used temporarily in the sterile surgical field, like custom cutting guides or molds used to repair facial fractures.

Take a cheekbone fracture, for example. Classically, a surgeon would shape a piece of mesh to look similar to the other side, place the mesh inside the cheek area and attach the broken bones to the mesh so that they heal in approximately the same shape as the other side. 

“Now we can take scans of the healthy side, mirror the image and print a 3D model,” Dr. Muyskens said. “Surgeons can then take that into the OR and prebend plates and mesh around the printed model to create the ideal version of the fractured side. Not only does it save time, but it allows the surgeon to create a repair that looks exactly like the healthy side.”

The 3D Lab’s use of surface scanners also contributes to improved aesthetic outcomes. By scanning an already 3D object, like an ear, the Lab can produce exact replicas that a surgeon can use to repair malformations.

Leveraging these tools to plan ahead and remove as much of the guesswork as possible leads to fewer complications and improved outcomes. It also translates to lower health care costs for patients and the health care system as a whole, according to Dr. Muyskens.

“We were pretty early adapters of this technology,” Dr. Muyskens said. “So I think it says a lot about Cook Children’s and our desire to provide innovative care and solve complex problems using state-of-the-art methods.”

10 Years and Counting

Today, Ivy is full of life. Thanks to good surgical planning made possible with the 3D Lab, expert execution by Dr. Tam and Dr. Nikaidoh, and ongoing care from Cook Children’s cardiologist Lisa Roten, M.D., Ivy’s heart is still going strong. She loves to skate, play with her dog and have fun.

“Her personality is so big,” Elizabeth said. “She acts like nothing happened to her. She acts like she's not even sick, and like she doesn't have a pacemaker. She does what she wants to do. She's like a little energizer bunny. Nonstop. She just keeps going.”

Every year, approximately 2,000 seemingly healthy people under the age of 25 die from sudden cardiac arrest (SCA), a tragic statistic that Cook Children's Health Care System is working to change.

In recognition of National Sudden Cardiac Arrest Awareness Month this October, Cook Children's is highlighting its year-round commitment to ensure schools and communities are prepared for such a medical emergency through its Project ADAM program, which provides life-saving training and resources.

Sudden Cardiac Arrest occurs when the heart stops beating, which leads to loss of breath or irregular breathing and prevents blood flow to the brain and other vital organs.

“With the proper training, anyone can do CPR and use an AED, and with enough of us trained and empowered to do so, we have the ability to potentially save someone’s life,” said Sarah Thieroff, Project Adam Program Coordinator.

Project ADAM (Automated Defibrillators in Adam's Memory) is a program named after 17-year-old Adam Lemel. In 1999, while playing high school basketball, Lemel collapsed and went into Sudden Cardiac Arrest. His family learned that he could have been saved with the right preparation and equipment. An automated external defibrillator (AED) was not available. His parents helped start the Project ADAM program at the Children’s Hospital of Wisconsin in his memory. Cook Children’s was the first in Texas and is now one of 52 hospitals and program sites to provide free cardiac resources, including training and AED devices. 

Thieroff recalled the motivating force behind the program's founding: "What we learned now is that Adam could have had a chance. I was faced with a choice. I couldn't save my son, but maybe I can save yours," Adam's father said.

More than 780 schools and 28 school districts are recognized to be Heart Safe. A designated Heart Safe school and district have completed a quality AED training program that includes a CPR/AED trained emergency team, an emergency plan for their AED and required AED drills.

“Knowledge of what to do, and muscle memory from practicing the appropriate steps may correspond to earlier action and in turn lead to a more successful outcome,” said Danielle Moyé, M.D., at Cook Children’s Heart Center.

Beginning the 2026-2027 school year under Senate Bill 865, also known as the Landon Payton Act, public and private schools will be required to implement a cardiac emergency response plan, mandate CPR and AED certification for specific school employees and volunteers, and require drills to test their emergency response.

“With the SB 865 bill, we have legislative support to encourage schools to act now and prepare,” said Dr. Moyé. “And we are ready to help them. We are here and happy to walk through what this looks like and ease some of the burden from the school’s administration, nursing team, and district.”

An AED is supported by the FDA to detect ventricular fibrillation. It has been designed and tested in both children and adults. Cardiac arrest is reversible in most victims if it is treated within a few minutes with an electric shock to the heart to restore a normal heartbeat. This process is called defibrillation. A shock delivered by an AED within 3-5 minutes can save a life and for every minute that passes, survival rates drop by 10%.

Through the Cook Children’s Project ADAM program, anyone can be trained. The program provides schools across Texas with the necessary tools and education to plan and develop their public access defibrillation (PAD) program.

“The expectation is that schools will be prepared for if it happens, not just putting it off or waiting to establish an emergency team, plan and/or drill if/when it happens,” explained Thieroff.

Signs and Symptoms of Sudden Cardiac Arrest

• Not breathing or irregular breathing (i.e. agonal gasps)
• Unresponsive and/or sometimes there are “seizure-like” movements.
• If someone witnesses a collapse:
  • Check for responsiveness.
  • Tap/lightly shake the person and ask if they’re okay, then if they’re not responsive, call for help/have someone call 911 immediately
  • Send someone to get the AED. If they’re unsure where the AED is, when they call 911, the 911 dispatcher should tell them the location of the nearest registered AED in the area.

Cook Children’s provides free Hands Only CPR/AED training, cardiac emergency response planning, and AED drill assistance to any school in Texas. Call 682-885-6755 or email ProjectAdamTexas@cookchildrens.org. For more information on free resources on the local Project ADAM program, visit www.projectadamtexas.org or the national Project ADAM page at www.projectadam.com

Courtney Peets was born with a rare and complex heart condition that was treated by pediatric cardiologists from her infancy through her teen years.

When Courtney moved to college, the new cardiologists she saw weren’t familiar with her type of defect. Those cardiologists didn’t have experience with young adults like Courtney, who was born with reversed heart chambers and arteries.  

“I ended up in the emergency room a couple times, but it’s so confusing in an adult world,” she said. “When you hook me up for an EKG (electrocardiogram), it looks like you’ve put the leads on incorrectly.”

Courtney was too old for pediatric cardiology at that point. But she didn’t quite fit in with adult cardiology care either. She didn’t know where to find a specialist for treating adults who have heart problems since birth, called congenital heart disease.

That’s when she joined a support group that introduced her to something she didn’t know existed: cardiology geared for adults born with heart defects. Referrals eventually led Courtney to the Adult Congenital Heart Disease (ACHD) program at Cook Children’s.

Now at age 40, Courtney makes an annual visit to see Scott Pilgrim, M.D., the program’s medical director. Dr. Pilgrim and his ACHD team at the Dodson Specialty Clinics in Fort Worth are able to monitor and help manage her ongoing heart issues. She’s proud to be a patient at Cook Children’s. In fact, every appointment is like a homecoming, because:

• The pediatric cardiologist who first saw newborn Courtney in the neonatal intensive care unit was James Allender, M.D., who helped establish the Cook Children’s Heart Center. Dr. Allender treated her through much of her childhood. 
• Courtney underwent open heart surgery at Cook Children’s at age 17.
• She used to be a registered nurse in the Cook Children’s Hematology and Oncology Center, where she connected with the joy and resiliency of her patients. 

Dr. Pilgrim has helped Courtney manage her blood pressure and other concerns so that she can keep up an active lifestyle of exercise, raising her two sons, and working as Chief Health Service Officer for Burleson Independent School District. She knows she might need heart surgery again someday. The ACHD program gives her confidence she’s in good hands. 

This month, we at Cook Children’s are celebrating the 10-year anniversary of our ACHD program. It was started in February 2014 because leaders at Cook Children’s saw a need. The ACHD services provide comprehensive care and support for hundreds of patients in their 20s, 30s and beyond. Here’s the background.

After Pediatrics

About 1% of all newborns have congenital heart disease, ranging from mild to severe. It used to be considered a pediatric condition because many children with severe defects didn’t survive to adulthood. Thanks to advances in diagnosis and surgery, more children born with heart problems are living longer. An estimated 1.4 million U.S. adults have a congenital heart disease. Their underlying congenital problems can lead to unique health challenges.

“This field of adult congenital heart disease has grown primarily because of the success stories we’ve had in pediatric cardiology,” Dr. Pilgrim said. “The incidence of congenital heart disease hasn’t really changed. Bur the lifespan of individuals growing up with congenital heart disease has tremendously improved.”

The ACHD program looks much like what patients often see in pediatric cardiology, such as EKGs and cardiac magnetic resonance imaging (cMRI), Dr. Pilgrim said. The program offers nutrition, social services, physical therapy, occupational therapy, noncardiac surgery, dental care and more.

Dr. Pilgrim also does pre-pregnancy consultations at the patient’s request. He and the patient discuss the risks for mother and baby. He assesses the structure, function and rhythm of the patient’s heart in those consultations.

“Sometimes we do an exercise stress test to see whether or not they have the aerobic capacity to handle the nine-month marathon of pregnancy,” he said. 

For patients who are already pregnant, the ACHD team works with colleagues in obstetrics and fetal medicine to create a plan based on the patient’s specific heart lesion. That plan includes delivery scenarios and the best options for anesthesia.

The future is bright for this growing subspecialty of cardiology, Dr. Pilgrim said. He pointed out that the adult congenital model at Cook Children’s stems from the Promise to improve the wellbeing of every child in our care and community.  

 “We haven’t neglected the fact that by virtue of us doing surgery as a child, now we have a growing population of adults with congenital heart disease who still need specialized care,” he said.

Repair and Continuing Care

Courtney was born in Fort Worth in 1983 with abdominal organs that were reversed, as well as reversed chambers and main arteries in her heart. Her smaller right ventricle pumps blood throughout her body, while her left ventricle pumps blood to her lungs – the opposite of a normal heart. The right ventricle muscle became progressively thicker, causing obstructed blood flow and low oxygen saturation when Courtney was a girl.

“My lips were always blue,” she recalled. “My parents let me do as much as I could. I played basketball, but I could probably play about a minute before I got tired.”

Courtney went in for regular checkups with Dr. Allender, who would draw pictures to explain how her heart worked. By the time she was 17, she needed surgery to replace a valve and to patch the leaky holes that were allowing the oxygenated and non-oxygenated blood in her heart to mingle. 

“I was the only teenager on the heart floor. There were little ones all around me,” she said. “I was kind of an anomaly because I'm one of the first generations that survived into adulthood with congenital heart disease.”

Courtney went through a short bout of depression after surgery, unaware of the link between congenital heart defects and mental health.  She couldn’t go to church camp that summer, or drink Dr Pepper, or hang out with her friends as much as she liked.

Medication after surgery helped keep her blood pressure down and her heartbeat more regular. And her oxygen levels improved. She was able to sing in the school show choir and assist as manager of the school sports teams. She went on to earn a master’s degree in nursing.

In her mid-20s Courtney experienced episodes of chest pain where her heart raced to 200 beats per minute. She and her husband didn’t think she could safely go through a pregnancy. That’s when Courtney found a support group for adults with congenital heart disease, which led her to a local cardiologist who specialized in adults with congenital heart disease and an obstetrician who specialized in heart disease in pregnancy.

Reassured that the obstetrician’s experience with other “heart mamas” would help get her body through the stress of pregnancy and delivery, Courtney became pregnant. It was a tough journey; she went into heart failure midway though, and the leaks inside her heart increased. Three weeks early, she delivered a healthy, 5 pound, 2 ounce baby boy. Courtney and her husband grew their family several years later by adopting their younger son. 

Courtney became Dr. Pilgrim’s patient several years after the ACHD program opened at Cook Children’s. She trusts that she’s in the right place with experts knowledgeable about congenital issues. Under Dr. Pilgrim’s guidance at annual appointments, she has come off of medications. She can run and lift weights.

She advises parents to make life as normal as possible for children with congenital heart disease. She feels fortunate her own parents raised her like she was “just Courtney” and not defined by her heart problem.

“I still needed discipline. I still had high expectations at school. My defect wasn't a crutch,” she said. “Sometimes I feel like we don't push our kids because they have something wrong. But a kid is a kid, and they're resilient. So just treat them like a kid. Let them be a kid, because that's going to make them more successful moving on.”

RELATED STORIES:

• Diving Heart First 
• Heart to Heart: Why This Mother and Daughter Share the Same Cardiologist

At the Cook Children's Heart Center, many of our patients grow up with us. We care for the needs of infants with congenital heart disease, and we stay with them all the way into adulthood. Our groundbreaking technologies and expert team of cardiologists, cardiac surgeons, diagnosticians, technicians and health care professionals make up our Adult Congenital Heart Disease program, which provides inpatient and outpatient services. Teenagers or adults with a previously confirmed or newly suspected diagnosis should be referred for a formal evaluation. Because when it comes to patients with congenital heart disease, we're here for life. 

A little over a year ago, Robert and Christina Ryan couldn’t imagine their daughter’s future. They were struggling just to come to terms with her present as their baby lay fighting for her life in the Cardiovascular Intensive Care Unit (CVICU) at Cook Children’s Medical Center - Fort Worth. 

In early January 2023, an illness sent Dorothy “Dottie” Ryan to Cook Children’s Emergency Department where the Ryans learned their daughter’s heart was failing. Dottie was 2 months old at the time. While her illness was brought on by a common cold virus, its severity was exacerbated by a congenital heart defect known as ventricular septal defect (VSD).

A VSD is a hole in the wall between the two lower chambers, or ventricles, of the heart. In the United States, about 1 in every 240 babies are born each year with the defect, making it the most common heart defect in infants, according to the Centers for Disease Control. 

The presence of a VSD allows blood to flow abnormally between the two ventricles, impairing the heart’s ability to pump blood throughout the body and to the lungs. Large VSDs can cause too much blood flow to the lungs, leading to difficulty breathing and fatigue with exertion. 

Because a baby’s greatest form of exertion comes when eating, the presence of a large VSD can lead to poor feedings and inadequate growth. If left untreated, it may also cause irreversible damage to the lungs and to the heart. 

The Ryans knew before Dottie was born that she had a VSD. However, in many cases, the hole closes on its own in-utero or after birth, so they weren’t overly alarmed by the diagnosis. 

But an echocardiogram soon after Dottie’s birth revealed she had not one, but four, VSDs. Dottie would eventually need surgery to close the holes. Over the next few months, Dottie’s cardiologist followed her condition and progress closely, giving her time to grow and develop as much as possible before undergoing a complicated surgical repair.

“Having multiple VSDs is relatively rare, occurring in about 10% of VSD patients,” said Greg Barker, M.D., Dottie’s cardiologist at Cook Children’s Medical Center - Fort Worth. “Often small VSDs will close on their own while large defects need to be repaired. Patients with multiple VSDs often have small defects that ultimately close spontaneously or do not require treatment. So Dottie’s case, with multiple large defects requiring surgical closure, is quite rare, and quite challenging to treat, requiring a very skilled surgeon.”

The Journey to Surgery

The multiple defects meant Dottie’s heart struggled to pump efficiently on a good day. Add to that even a mild cold, like the one that sent her to the Emergency Department in January 2023, and you have a recipe for the heart becoming overwhelmed and unable to pump oxygen-rich blood throughout the body to support healing. Dottie’s heart simply could not cope with the increased workload necessary for fighting infection.  

“So we got admitted into the CVICU and that's when this journey started,” Christina said. “I remember the doctor telling us she’s in heart failure and we need to schedule surgery, but we had to let her get better from the cold first.”

Dottie did get better and was released from the medical center on January 18 to await surgery in February. But two days later, Dottie began having trouble breathing again and the family returned to the Emergency Department. Within 45 minutes of arriving, the Ryan’s baby girl was on a ventilator. 

The culprit this time? Three bacterial blood infections. 

“It was the hardest night of our lives,” Christina said. “But I felt like the team was amazing. Two cardiologists and the ER physician were there. I was so scared, but I was so thankful for that team that was saving her life in front of my eyes. I hope to never be in that situation again, but it was amazing to see all of these people who spend their whole lives saving children like Dottie. That whole night was miraculous.”

Over the next few weeks, Dottie’s condition improved again but she needed to remain in the hospital to continue the intravenous medications that helped fight infection and stabilize her heart. As the days went by, her heart needed more and more support, prompting doctors to schedule her surgery sooner rather than later.

The Best Gift

On the evening of February 22, 2023, which happened to be Christina’s birthday, Dottie’s physicians convened to discuss a surgical plan. That night, Christina took a rare break from the hospital to have dinner with her older children while her husband stayed with Dottie. While at dinner, she received an unexpected call with surprising news that moved her to tears. Dottie’s surgery was scheduled for the very next morning. 

“It was the best birthday gift ever,” Christina said. “To see this whole team of people mobilize 12 hours before surgery was amazing to me.”

On surgery day, many of the cardiac intensivists who cared for Dottie in the weeks prior stopped by the waiting area to visit with the family, with one taking a moment to pray with them. 

“Before they took her away to surgery it just felt like Dottie was so important,” Christina said. “Even though they see hundreds, if not thousands, of kids every year, in that moment they saw so much value in her little body and showed she was worth fighting for. We already knew that, but it was amazing to see these doctors and nurses value and love her, too.”

During the 8-hour surgery, Vincent Tam, M.D., medical director of Cardiothoracic Surgery at Cook Children’s, successfully patched three holes in Dottie’s heart. The fourth, which could not be patched, closed on it own a few months after surgery. 

Dottie spent the next few weeks at Cook Children’s recovering from surgery. Because she stopped taking a bottle or nursing while on the ventilator before surgery, she needed a feeding tube for adequate nutrition. Shortly after an additional procedure to place the feeding tube, Dottie was able to go home in March 2023. 

Little Girl, Big Smile

Although a tiny infant at the time, Dottie made a big impact on the staff at Cook Children’s. She’s known for her smile, even attempting to grin while on the ventilator. 

“As Dottie’s condition improved, we learned her sweet smile and endearing demeanor which made caring for her such a joy,” said Brynli Clayton, BSN, RN, CCRN, CVICU nurse supervisor at Cook Children’s. “Working in a high acuity environment our staff knows all too well the reality of patients who do not get better, which makes the ones that do that much more special. Watching Dottie continue to grow, when she comes by for visits, and hearing updates about her development seals in our minds that the work we are doing matters.”

To celebrate Dottie’s first birthday, the Ryan family collected pacifiers and donated them to the CVICU in recognition of the kindness the family says they experienced at Cook Children’s.

Now 16 months old, Dottie is in full toddler mode and doing her best to keep up with her siblings, 5-year-old Eddie and 3-year-old Lucy. She still requires nutrition support but is learning to eat on her own more and more each day. 

And the future the Ryans couldn’t imagine for their daughter a year ago is now full of hope and as bright as her signature smile.

“Thankfully, Dottie has had an excellent repair,” Dr. Barker said. “While there are still things that we are watching, I expect her to do very well and be able to have a normal, healthy life.”

Through it all, the Ryans learned to cherish the triumphs, no matter how small. 

“I’d encourage other families facing a challenge to find joy in and celebrate the tiny victories because those can give you tremendous hope,” Christina said. “They did for us.”

Phillip Burch, M.D., a cardiothoracic surgeon at Cook Children’s, performed three surgeries on Sydni before her first birthday. He says Cook Children’s Heart Center sees about 500 surgical cases a year, with around 300 of them being open-heart surgeries. Most cases aren’t as severe as Sydni’s.

“She has a canal defect where the central portion of her heart didn’t form appropriately,” Dr. Burch explained. “The veins that drain her lungs did not return to their normal position.”

Sydni is the Peña’s third child. They say her pregnancy was normal, until they attended a doctor’s appointment at the 20-week mark.

“After the scan, the doctor came into the room, and I remember her face was different,” Ashlea said. “The doctor said she saw an issue with Sydni’s heart on the anatomy scan and was referring us to a maternal-fetal medicine doctor to get it checked out.”

The referral would be the beginning of a very long journey and a new normal for the Peña family. At their appointment, the maternal-fetal medicine doctor explained the challenges their daughter would face after birth.

“The first thing I remember him saying is, ‘Your daughter has a condition called Heterotaxy syndrome,’” Ashlea explained. “This meant her stomach was on the right side of her body instead of the left, and this condition caused her heart defects.”

The family visited Kristal Woldu, M.D., a fetal cardiologist at Cook Children’s, and learned Sydni would require open-heart surgery within 24 hours of birth.

“My husband broke down and cried,” Ashlea remembered. “That was the first time he realized this wasn’t a minor inconvenience, but this was going to require immediate intervention.”

After learning the devastating news, Ashlea began reading stories from other families whose children were cared for by Cook Children’s Heart Center. Still, the news was hard to process.

“It became real when she showed us the diagram where normal body parts were supposed to be, compared to what Sydni’s looked like in the early stages. We knew we had a big hurdle ahead,” Tony said.

After Sydni’s birth, the family was told to plan for a six-week hospital stay. This meant figuring out how they were going to care for Sydni, as well as their daughters, ages 2 and 5.

“We moved into my brother and sister-in-law’s apartment, and they moved into our home to take care of our older girls,” Tony said.

With this arrangement, the Peña’s were never more than three to five minutes away from the medical center and someone was always able to be by Sydni’s side.

Sydni’s projected six-week stay nearly doubled into 82 days at Cook Children’s. The first half was spent in the Cardiac Intensive Care Unit (CICU). Sydni was then moved into the step-down unit when she no longer required intensive care. Ashlea remembers how exhausting this time was, and how long the days and weeks seemed to drag on.

“I would run home and shower and come right back. The in and out was draining,” Ashlea remembered.

During Sydni’s first surgery, she had a shunt placed in her heart to help with her pulmonary blood flow. The surgery was successful, but when she came off of the ventilator, she had a hard time maintaining her oxygen levels. Nine days after her first surgery, the doctors informed the family Sydni would need a second surgery to revise the shunt. To make sure the procedure was successful, the medical team left her chest open for a several days while they monitored her progress.

With back-to-back surgeries and Sydni being on a bypass machine, she began having feeding issues.

”She had terrible reflux. She vomited everything up constantly, and wasn’t gaining any weight,” Ashlea recalled.

The Peña’s remember being optimistic, but every time they got their hopes up about going home, Sydni gave them a sign she wasn’t ready. That stress became obvious to cardiac intensivist Lane Lanier, M.D.

“One morning, I was sitting next to her bed and Dr. Lanier squatted down in front of me and took my hand and said, ‘Ashlea, you have something called ICU fatigue. I can tell you’re at the point where most people want to scream and throw something out the window,’” Ashlea remembered. “He reassured me we were going to get our baby out of the hospital. He couldn’t promise it would be the next day or week but assured me we would follow Sydni’s timeline and go home when she was ready.”

Ashlea says those words gave her the strength to go on and continue fighting for her daughter’s full recovery.

“When something like this happens, you aren’t given a choice, you go into survival mode,” Ashlea explained.

During their time at Cook Children’s, the Peña’s family members brought their older daughters to the medical center to visit their sister, have lunch and play outside.

“Cook Children’s does such a good job helping siblings and families during difficult times,” Tony remembered. “The music room, arts and crafts, the game room… all of those things were good for us to explore with our older girls.”

When Sydni was released, she was sent home on more than 10 medications, as well as a G-tube for feeding. The Peña’s had to get used to being home alone and not having Cook Children’s staff there to help around the clock. They were also full-time parents to three children for the first time. Ashlea said having Sydni enrolled with the Cook Children’s Health Plan (CCHP) played a major role in transitioning home.

“The most helpful part of CCHP has been our patient advocate Jamie,” Ashlea said. “She helped us get therapies set up for Sydni, as well as made sure we had the right supplies for her tube feedings. Knowing there is a nurse advocate to call when we encounter an issue has been a stress reliever for my family.”

Today, Sydni has graduated from most of her therapies and even learned to do a somersault with the help of her big sisters. While Sydni has come a long way, she still has another major open-heart surgery later this year. This surgery, a biventricular repair, will give her two ventricles, like other human hearts.

“We hope that she has good longevity and a good quality of life,” Dr. Burch said. “Based on the imaging we have, it’s reasonable to presume we can provide a good technical outcome for her.”

Sydni’s parents say they are grateful for Cook Children’s, as well as the valuable lessons they’ve learned along the way.

“I have learned to appreciate every moment of life because you never know when it’s going to be turned upside down for you,” Ashlea said.

The couple also offers advice for other parents who find themselves in their shoes.

“Do your research, speak up for your child, and ask questions,” Tony said. “Seek out trauma therapy once you can, it makes a huge difference once you realize everything you’ve been through.”

Before Waylon could receive the heart surgery he so badly needed, his parents learned their surgeon was leaving the area. The Guidrys were suddenly faced with uprooting from their home in Fate, Texas (Rockwall County) and taking their very sick 6-month-old son out of town for surgery, most likely to either Houston or Chicago. Waylon was born at 27 weeks and 3 days with a rare heart condition called Tetralogy of Fallot, which creates obstruction to blood flow to the lung and is associated with a hole between the pumping chambers of the heart .

As they considered their options and prepared to pick up their lives, the phone rang one afternoon. It was Waylon’s cardiologist to tell them a new heart surgeon, Vinod Sebastian, M.D., would take on the case at Cook Children’s Medical Center in Fort Worth.

“Jordan and I didn’t know what to do,” Katie said. “We researched our options and we just couldn’t make up our minds. When we got the phone call, we were so relieved. Dr. Sebastian received all of Waylon’s history and was confident he could repair the Tetralogy of Fallot with one surgery and also spare his pulmonary valve, which traditionally has to be cut open and resected during this repair.”

The family arrived at Cook Children’s on Nov. 28, 2016 and Waylon underwent heart surgery on Dec. 14. Tetralogy of Fallot is a rare heart defect that occurs in about 5 out of every 100,000 babies.The surgery is a complicated one because the congenital heart disease results in four main congenital heart defects:

• Ventricular septal defect (VSD)
• Override of the aorta over the VSD
• Right ventricular outflow tract obstruction
• Right ventricular hypertrophy

ifelong monitoring is required due to the increased incidence of arrhythmia, exercise intolerance and reduced right ventricular function.

Often times when the surgery is performed, surgeons cut open and resect the pulmonary valve in the baby’s heart. If that happens, it usually means a heart surgery later in life to replace the valve. That was not the case for Waylon.

“As far as Waylon’s heart, his long-term prognosis is excellent,” Dr. Sebastian said. “Waylon is unlikely to need any further cardiac surgical intervention. In the past, Waylon’s condition was incurable. Even 10 years ago, the surgical repair routinely involved cutting open and resecting the pulmonary valve. His heart surgery is very gratifying because his heart problems are no longer an issue.”

Waylon and his family will face other non-cardiac health issues in the future, but for now the family feels very fortunate to have found Cook Children’s and Dr. Sebastian.

“He’s doing great now,” Katie said. “We are so grateful and blessed for having this opportunity to come to Cook Children’s and for our son to receive all the help he needs."

Waylon has been at  home now for more than two months and Katie is busy planning his 1 year old birthday party on May 30.

About Dr. Sebastian

Vinod Sebastian was born and raised in India and has pursued specialty training in the US in surgery, cardiothoracic surgery and pediatric cardiac surgery. During training he realized his passion of becoming a pediatric cardiac surgeon and the unique ability to provide life altering treatments to neonates, infants, children and adults with congenital heart disease.

He trained at Stanford University with Frank Hanley and VM Reddy in the field of pediatric cardiac surgery. During this time he trained in techniques of “single stage unifocalisation” and “extremely low birth weight cardiac surgery” at one of the largest practices in the world.

Dr. Sebastian is happy to be back in Texas at Cook Children's and providing pediatric cardiac surgery services in the Dallas/Fort Worth area.

In his spare time, he enjoys being outdoors, reading, watching cricket, tennis and swimming.

About Cook Children's Cardiothoracic Surgery Program

When it comes to your child, any kind of surgery is concerning. When that surgery is related to the heart, it can be a very frightening time. The cardiothoracic surgeons in the Cook Children's Heart Center are recognized for their skill and expertise. And, because they perform an average of 400 surgeries each year, they know how challenging it is for you and your child, and they will work closely with you to ensure you understand all your child's surgery will entail and the risks involved in order to provide the best plan of treatment. Click to learn more about the program.

Kenda Hooker was 3 years old when doctors discovered a hole in the top chamber of her heart. Like many congenital heart defect (CHD) patients, she went on with her life assuming her heart had been ‘fixed.’ As memories faded, her medical records were lost and her parents, who knew the most about her condition, passed away. So when she ended up with health problems nearly three decades later, doctors didn’t know where to begin.

“I started feeling dizzy and fatigued. I even passed out a few times,” said Kenda. “Several doctors told me the same thing, ‘You’re a new mom, you’re probably depressed. I even had my husband go with me to tell them that there was something wrong.”

Kenda wasn’t depressed. Instead, she was having issues with arrhythmia and would need a catheter ablation to remove the faulty electrical pathway inside her heart. That was just the first of several unfortunate diagnosis’s Kenda would hear over the coming years.

Next, it was the severe heart defect found in her first daughter, Koralyn.

“Koralyn Marie was born April 9, 2012. We knew prenatally that she had hypoplastic left heart syndrome,” said Kenda. “She spent her whole life at Cook Children’s, we were never able to take her home.”

Koralyn’s cardiologist, Lisa Roten, M.D., said her little heart was severely underdeveloped. As hard as they tried, there was little the doctors and nurses at Cook Children’s could do to save her. She passed away at 4 months old.

With the arrival of Kenda’s second daughter, Karis, in October of 2015 came more unwelcomed news. Another heart defect was diagnosed, again prenatally, but this one was less severe.

“Karis has an atrial septal defect,” said Kenda. “She had another hole in her heart but it has already closed on its own.”

Dr. Roten says there’s no way to know right now if there’s a genetic reason Kenda’s daughters both experienced heart problems. After all, she also has three heart-healthy boys.

“Kenda is part of the first generation of complex CHD patients who are truly thriving,’ said Dr. Roten. “A lot of the surgeries we perform now were not an option before the 1980’s so we’re seeing a new group of patients who have undergone surgery and are able to lead pretty normal lives.”

Dr. Roten says there are so many of these patients that a new subspecialty has even been created. At Cook Children’s, The Adult Congenital Heart Disease program treats patients who have outgrown pediatric care, including women with CHD who are considering becoming pregnant.

For Karis, her future is full of hope. Dr. Roten isn’t sure she will even need surgery. Even if she does, it likely won’t be an open heart operation like the one her mother had. Instead, her heart could be mended in the cardiac catheterization lab at Cook Children’s, with just an overnight stay. But like her mother, she will need to see a cardiologist for lifelong care.

“The fact that people with CHDs are going on to have kids shows just how far we have come,” said Dr. Roten. “At the end of the day, it may not be the life you would have chosen for yourself or your child, but it’s not a bad life. People with CHDs are going on to live good quality lives. The most important thing is to keep going.”

About Cook Children's Heart Center
The cardiology team at Cook Children's has extensive experience in the diagnosis and treatment of pediatric heart care. They know the unique requirements of treating the growing hearts of children, including those with extremely rare and difficult conditions. Our areas of expertise include cardiac surgery,interventional cardiology, adult congenital cardiology, electrophysiology, cardiac testing and imaging,echocardiography, fetal echocardiography and cardiac anesthesiology.

Liam lived from March 25 to Nov. 1, 2013, and spent much of his brief life at Cook Children’s with critical issues ranging from an operation to repair his intestines, to heart problems, to feeding concerns.

He seemed to be constantly fighting until it was too much for his little body to overcome.

Now a little over a year later, Vickie Phathaphone, Liam’s mother, looks back on her time at Cook Children’s with an emotion that may be surprising to some – gratitude.

Recently, Phathaphone reached out to Cook Children’s Facebook page. She wanted to thank the staff who treated her little boy.

“My family and I, including Liam, loved the medical staff at Cook Children’s and you guys gave us the most amazing experience and educated us well,” Phathaphone said. “The staff at Cook Children’s helped my family and me by teaching us how to not be afraid to hold our little Liam.”

Phathaphone said doctors and nurses updated her family every day and Cook Children’s updated her by cellphone as needed. She said the NICU nurses taught her so much, ranging from the importance of breast milk and how to pump it, to how to carefully bathe, feed and hold her fragile baby.

“Although being a parent is natural, with Liam’s condition it was quite frightening,” Phathaphone said. “We did not want to hurt him, but we were reassured by the nurses what not to do and what to do. I loved how the staff treated Liam, as he was a part of their family, and I am thankful that Liam was under such amazing care.”

After letting a group of the physicians know about the letter, they were appreciative of Phathaphone’s kind gesture and began to reflect on the loss of not only Liam, but other children who have died too soon.

Surgeons Vincent Tam, M.D., James Miller, M.D., and Lisa Roten, M.D., a cardiologist, share with us what their life is like after the loss of a child.

Dr.Tam

He tries to stay in control as he fights back the tears.

But on this day, as hard as he tries, Vincent Tam, M.D., a pediatric heart surgeon at Cook Children’s, struggles to keep his composure. He’s thinking about Liam.

Dr. Tam performed two heart operations on Liam. All the procedures were successful, but other underlying problems were simply too much for the baby, who was born premature, to overcome.

In practice for 23 years, Dr. Tam copes with the death of a patient by compartmentalizing the loss. He tries his best not to dwell on it, so it won’t impact his performance in the operating room or his role as husband and father. Then, every once in a while, he receives a note or hears from a parent whose child has died, like the one from Phathaphone. The parents don’t want to express grief or even anger, but simply to say thanks.

“It’s hard because if somebody dies or somebody has a bad complication, I think, ‘Oh gosh, could I have done something differently?” Dr. Tam trails off and after a long pause continues to speak. “There’s all kinds of questions that you can ask as a Monday morning quarterback, but personally I’ve dealt with it in a way that I feel like I’ve … um …”

Again, a long pause as he composes himself. “I do the best I can with every surgery and that’s all I can do.”

Dr. Miller

Shortly after Liam’s birth, James P. Miller, M.D., a pediatric surgeon at Cook Children’s, operated on him to repair gastroschisis, a condition where the baby is born with the intestines on the outside of the body.

Even after 17,000 surgeries and 27 years as a surgeon, it only takes a quick glance at a child’s chart for the memories to come flooding back. He remembers Liam and his mom. He says he knew from being around her for a short period of time and the love and compassion she always showed to her child, Phathaphone would be a great mom.

Dr. Miller has gotten to know some patient families well over the years. Throughout his career, he’s been to many of their weddings, but he’s also seen death.

“It can devastate you for weeks,” Dr. Miller said. “It can be like reopening a wound when you realize it’s the first anniversary of a child’s death. You hope you have somebody in the hospital that you have helped or made a difference in their lives, just to get out of your rut. I don’t think people realize how much doctors do feel. When you do what we do with these kids, you do get attached to them.”

Dr. Roten

For the patient families who are cared for by Lisa Roten, M.D., a cardiologist who specializes in fetal echocardiography at Cook Children’s, they see someone who cares deeply for her patient families. But they also may be surprised to know that she understands Dr. Tam’s need for compartmentalization.

“The truth is, you have to do that,” she said. “You wouldn’t be a physician if you couldn’t do that. We have a work life and we have a personal life. I can’t carry that home with me. I do talk about the loss of my kids, but with the people who can empathize with me. I talk to my colleagues and staff, the other doctors and nurses, who cared for the child.”

Still, Dr. Roten admits to developing a special bond with most of her families and says a gesture like the one from Phathaphone and other parents means a lot to her. As much as the families sometimes need closure by writing a note or speaking to the doctors, Dr. Roten also needs to find a way to say thank you to the parents for entrusting their child’s care to her and goodbye to the children.

Dr. Roten makes every effort to reach out to families who have had a child pass and to go to the funerals whenever possible. It allows her to complete the circle of care.

“At the end of the day, we’re all humans. We are people who have families and we are touched by these families we see,” Dr. Roten said. “Even after the child dies, they never really leave our lives. There are ways that we continue on together. We take our cues from the family. If they ever want to reach out or touch base with us, that’s comforting to us. It is the end of a relationship that sometimes you’ve had for years and years with families and sometimes like in Liam’s case, it’s been shorter.

“But they are all so special to us.”