Tetralogy of Fallot Patient and his Family Give Back

What was supposed to be a routine visit with their pediatrician on a seemingly typical Friday morning changed Stephanie Harding and her son Trent’s life forever when their day ended with a diagnosis of Tetralogy of Fallot.

As the pediatrician wrapped up his checkup that morning, he noticed 5-week old Trent’s forehead appeared somewhat purple. He tested his oxygen saturation to find the baby’s oxygen level was low and would need to be seen by a cardiologist immediately for further examination.

A few hours later, the cardiologist determined that Trent had Tetralogy of Fallot, a heart condition made up of four related congenital defects that occur due to abnormal development of the fetal heart. Those three words were as foreign and shocking to Stephanie as the turn of events that day.

“I remember thinking we had only packed enough food and supplies for the baby for a trip to the pediatrician and back. Here we were now, at a hospital, hearing the cardiologist explain what his condition involved and everything else went blank. All I could hear is that Trent had four heart defects that needed to be repaired immediately,” Stephanie says. “We asked for prayer right away; we were in disbelief.”

A few days later, Trent had open heart surgery at Loma Linda University Children’s Hospital to repair the four heart defects.

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Trent recovering after his first surgery, just days after being diagnosed with Tetralogy of Fallot.

“He’s our miracle baby,” Stephanie says. “It’s a miracle that we caught his condition in time. There had been no signs or symptoms before that appointment with his pediatrician.”

In 2017, Trent, then 6 years old, underwent a second surgery, as is typical for a child with Tetralogy of Fallot, to replace the pulmonary valve with Dr. Richard Gates, a pediatric cardiothoracic surgeon and co-medical director of the CHOC Children’s Heart Institute. Stephanie and her husband, Tim, remember having to explain as best they could to their little boy what was about to happen to him once again.

“Trent is quiet and goes with the flow, yet I didn’t know how he would take it. He was so brave through it all,” Stephanie says. “I still remember as he was being wheeled into the operating room and I finally had to let go of his hand so he could go in; he looked up from the gurney and looked at me, with the anesthesia just starting to kick in, and I thought he was surely going to start crying or screaming. Instead, he just gave me this confident look like, ‘I’ve got this Mom, it’s going to be OK,’” an emotional Stephanie recalls.

The surgery was successful, and the Hardings couldn’t be more thankful with the remarkable care and compassion Trent received from CHOC staff, many of whom they keep in touch with today.

Stephanie and Trent occasionally stop by the cardiovascular intensive care unit (CVICU) at CHOC to say hello to the nurses and doctors who took care of them, and drop off gifts for other families going through what they’ve experienced. Stephanie had provided goodies to the hospital, including the CVICU, long before her son was treated at CHOC. Now, giving back to the CVICU means more than ever.

“It feels great to let another parent know they’re not alone, and that there are resources and groups out there that will support them,” Stephanie says.

Trent, now 7, puts it simply. It makes him so happy, he says, to be able to give back to other kids like him.

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The Harding family’s passion to help others doesn’t stop at CHOC. They are very active locally, raising awareness and funds for the congenital heart defect (CHD) community, through groups like Hopeful Hearts Foundation, an organization for families with children affected by CHD.

On Feb.  23, the Hardings are hosting a fundraiser at GritCycle, an indoor cycling gym, with the proceeds benefitting CHOC. Participants can buy a ticket to cycle at the Monarch Beach location.

Trent will need another surgery to replace his pulmonary valve in about five years. For now, he is an active first-grader who enjoys math and jiu-jitsu. He also loves hanging out with his family, including his brothers, Stephan and Dylan, who are very supportive of their ‘miracle baby.’

Learn more about the Heart Institute at CHOC Children's

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Catching a Heart Defect in Utero: Marco’s Story

Meagan and Dante Cipulli quickly settled on a name when they discovered their third baby would be a boy: Marco, which means God of War.

That name would become especially fitting a few weeks later. When Meagan was about six months pregnant, the couple learned their baby had a congenital heart defect called Tetralogy of Fallot and would need open-heart surgery soon after birth.

“Knowing my unborn baby would need open-heart surgery after birth was the scariest experience of my life,” Meagan says. “After receiving his diagnosis, we realized we couldn’t have picked a better name for our little heart warrior.”

Finding heart defects before birth

When a second ultrasound by a perinatologist confirmed that baby Marco’s aorta was enlarged, Meagan was referred to CHOC Children’s pediatric cardiologist Dr. Nita Doshi.

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Dr. Doshi performed a fetal echocardiogram, which uses sound waves to create a picture of an unborn baby’s heart.

The evaluation confirmed that Marco had Tetralogy of Fallot, a heart condition comprised of four related defects that cause inadequate amounts of blood to reach the lungs for oxygen, then sending oxygen-deficient blood throughout the body.

“I was in complete shock,” Meagan says. “As a nurse, I knew exactly what Tetralogy of Fallot was and that he would need open-heart surgery.”

Planning began immediately. With the help of Dr. Doshi, the Cipullis began researching hospitals, cardiologists and surgeons who could care for Marco when the time came.

CHOC emerged as the clear choice, and the Cipullis opted for Dr. Doshi to continue as Marco’s cardiologist and Dr. Richard Gates, director of cardiothoracic surgery as well as CHOC’s surgeon-in-chief, to perform the corrective surgery.

Organizing pre-, post-birth care

Meagan moved her obstetric care to a physician aligned with St. Joseph Hospital so that when Marco was born, he could be immediately transferred next door to CHOC’s neonatal intensive care unit (NICU).

During a perinatal conference, the Cipullis met with the obstetrical team at St. Joseph and CHOC’s neonatal team to discuss Marco’s birth and care.

“That conference allowed me to have all my questions answered. It gave me peace of mind that all those related to our care were on the same page,” Meagan said. “I knew that I had made the right choice after meeting with the care team.”

The remaining weeks of Meagan’s pregnancy were an emotional roller coaster. The couple prepared their older sons as best they could for what was to come with their younger brother.

Not understanding the full extent of their baby’s medical needs, the Cipullis’ were terrified. However, they felt at ease knowing a plan was in place.

“Each day of my pregnancy after diagnosis was filled with worry and fear. But they were also filled with gratitude and hope knowing we were fortunate enough to have Marco’s diagnosis in utero and were able to plan for his care after birth,” Meagan says.

The Cipullis didn’t have to wait long for Marco. On May 16, 2017, Marco was born five weeks ahead of schedule. After a brief rest on his mother’s chest, Marco was moved to CHOC’s NICU, where he stayed for the next five days before going home.

Surgery day

Marco was back at CHOC about three months later for surgery with Dr. Gates to repair his heart defects.

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“At first it all seemed so surreal and somehow I was able to keep it all together until the moment they wheeled Marco into the operating room,” Meagan says. “While he was lying in the crib, he looked over his shoulder and gave me and his dad this smile and look like, ‘I got this, guys, don’t worry.’ I don’t think I have ever cried harder in my life.”

The surgery went well, and Marco spent five days recovering in CHOC’s cardiovascular intensive care unit.

Today, Marco is a happy and healthy 9-month-old who loves to smile and laugh. He sees Dr. Doshi every four months for follow-up appointments, but otherwise requires no additional medication or therapy.

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Many babies with Tetralogy of Fallot will require additional surgeries as they age. The Cipullis however are hopeful that Marco’s early interventional measures will last for many years.

Feeling positive

Meantime, the Cipullis are enjoying every minute with their three boys. They are grateful for the care they received at CHOC after catching Marco’s condition early.

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Meagan recommends that other families who find themselves in similar situations be vocal about their fears, but also stay positive about their baby’s future.

“My husband and I each night would talk about what we were feeling that day,” she recalls. “At first, it was more about our fears and worries, but eventually each day we would talk more about our excitement and joy to meet our little warrior.”

Learn more about the Heart Institute at CHOC Children's

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Innovation Comes Home

Five-year-old Emma has hypoplastic left heart syndrome (HLHS) and has undergone three open-heart surgeries since first coming to CHOC Children’s― the only Orange County hospital providing pediatric open-heart surgery― at just 2 days old. After the last surgery, she went home with an advanced, wireless monitoring device provided by the CHOC Cardiopulmonary Health Intelligence Program (CHiP). This lightweight tablet monitors Emma’s blood pressure and other vital signs, instantly transmitting the data to her care team at CHOC who can incorporate it into her electronic medical record as needed. The tablet also offers her parents extra peace of mind and direct access to Emma’s team of specialists at CHOC.

Emma and her family have participated in CHOC Walk in the Park to give back to the hospital that has cared for her since birth.

Videoconferencing for “virtual visits” mean CHOC doctors, nurses and other members of her care team are always close by. It’s part of a larger pilot program that integrates several emerging health technologies to provide even more highly personalized care.

HLHS is a type of heart defect that occurs when the left side of the heart does not form properly during pregnancy. In patients with hypoplastic left heart syndrome, most of the structures on the left side of the heart are small and underdeveloped. The degree of underdevelopment differs from child to child. Perhaps the most critical defect in HLHS is the small, underdeveloped left ventricle. This chamber is normally very strong and muscular so it can pump blood to the body. When the chamber is small and poorly developed, it will not function effectively and can’t provide enough blood flow to meet the body’s needs. For this reason, an infant with HLHS will not live long without surgical intervention.

Mid-way through taking a walk in the CVICU, Emma takes a break to dance with surprise visitor Mickey Mouse.

Babies born with this condition must begin the first of three open-heart surgical procedures within days of birth. Emma underwent her first surgery when she was 6 days old and had her third surgery almost four years later. After each surgery, her parents Patrice and Kevin saw noticeable improvement in her energy, mobility and quality of life.

During one stay in CHOC’s cardiovascular intensive care unit (CVICU), Emma and her family dressed up as superheroes and paraded throughout the unit.

But children like Emma need frequent office visits to monitor heart function, medication and symptoms. Over the past five years, her parents have made countless trips to CHOC, sometimes as often as every day. At home, they’ve meticulously tracked their daughter’s medications, blood pressure and overall health with pen and paper.

That changed last year thanks to a five-year CHOC pilot program designed to provide more personalized treatment for kids with heart failure. Emma’s parents now check her vital signs instantly at home with the same clinical accuracy as an office visit. With the Integrated Medical Tablet provided by the CHOC Cardiopulmonary Health Intelligence Program (CHiP), Emma’s doctors, nurses and care team are only a touch screen away.

home monitoring for choc childrens patient
Thanks to a five-year CHOC pilot program designed to provide more personalized treatment for kids with heart failure, Emma’s parents now check her vital signs instantly at home with the same clinical accuracy as an office visit.

“After her last open-heart surgery, Emma was still healing at home when we began testing out this machine,” Patrice said. “It gave us such peace of mind as we were still closely looking at her oxygen saturation and blood pressure. We didn’t have to go into the office every day to get this information—it could be recorded and sent to her specialists from the comfort of our own home.”

Instant Communication 

Surgery may be behind her, but Emma continues taking medications that require constant monitoring and adjustment. The tablet has built-in alerts to notify her doctor if the results are outside the range of the personal limits that have been set for her.

And if something doesn’t look quite right, Patrice and Kevin don’t have to drive Emma over to CHOC. They can take photos and videos with the tablet and send them directly to Emma’s care team.

Although Emma and her family love visiting with her care team, they are happy this at-home monitoring has resulted in less frequent trips to CHOC.

“Emma is on a diuretic for her heart condition, so being able to show her doctors if she is swollen or puffy is helpful,” Patrice said. “We are also able to type up our notes and questions for her doctors.”

Videoconferencing capabilities allow “virtual visits,” including wellness checks and parent education, at any time with the CHOC cardiopulmonary team. Beginning in 2018, CHiP will expand services to patients with other types of heart conditions.

“Patients and families feel much more comfortable outside of the clinical setting, but when they’re not in the hospital, they feel very nervous about the health of their child. They have much more peace of mind knowing that their child has quick access to CHOC from home,” explains Dr. Anthony Chang, CHOC’s Chief Intelligence and Innovation Officer, “We’re proud to offer a clinical environment to test and evaluate new technologies such as artificial intelligence and remote monitoring solutions.”

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Dr. Anthony Chang, Emma’s pediatric cardiologist, and CHOC’s chief intelligence and innovation officer

Far More Than a “Fancy Gadget”

Remote monitoring, video conferencing and wireless access to a patient’s electronic medical record are exciting advancements in care for children with heart failure. In this pilot study, however, CHOC is exploring the additional benefits that may come from leveraging these capabilities with other emerging health technology.

One of those technologies is precision medicine, a treatment approach that incorporates the patient’s genetics, environment and lifestyle. Another is pharmacogenomics, which evaluates how genetics may impact a patient’s individual response to a particular medication.

As a pediatric cardiologist within the CHOC Children’s Heart Institute, Dr. Chang is interested in how these technologies, combined with data analytics and artificial intelligence, may further personalize treatment and improve outcomes.

“Our goal is to use all available technologies and provide the most advanced, innovative care that is best suited for the individual patient,” Dr. Chang said. “CHOC is taking a national role among children’s hospitals to develop a model that may ultimately be used by other pediatric specialties for better management of complex patients.”

Learn more about the Heart Institute at CHOC Children's

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CHOC Patient Benefits from World’s Smallest Pacemaker

Sofia Rodriguez and CHOC Children’s cardiologist Dr. Anthony McCanta are making medical history. The petite 12-year-old recently became the smallest and youngest patient to receive the Micra® Transcatheter Pacing System (TPS). The innovative physician, who specializes in pediatric electrophysiology, joins an elite group of doctors in the world who have implanted the device, and is likely the first to do so in a child.

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The Micra® Transcatheter Pacing System is about the size of a vitamin.

About the size of a vitamin, Micra TPS, which was approved by the Food and Drug Administration last year, provides the most advanced pacing technology at one-tenth the size of a traditional pacemaker. And unlike traditional pacemakers, the device does not have cardiac leads, which are the wires that carry electricity from the pacemaker to the heart and the other way around. This activity occurs between 100,000 to 200,000 times in most children, eventually putting a strain on the leads. The leads can then break or stop working properly, requiring a new device to be implanted.

In addition to being leadless, the Micra TPS is small enough to be delivered through a catheter and implanted directly into the heart instead of a surgical “pocket” under the skin. This offers patients a safe alternative to conventional pacemakers without the complications associated with leads — all while being cosmetically invisible.

Sofia’s story

Diagnosed shortly after birth with a particularly complex form of tetralogy of fallot, a rare and serious heart condition, Sofia had her first open-heart surgery before turning 1 month old and a second, to implant a traditional pacemaker, when she was 6 months old. Abdominal surgery followed, as did a diagnosis of DiGeorge syndrome, a chromosomal disorder that affects the development of several body systems.

Sofia tackled each health challenge with a fierce determination to survive and, more than that, to enjoy her childhood. Her parents and the team at the CHOC Children’s Heart Institute supported her every step of the way, equally committed to her quality of life.

When the pre-teen needed a new pacing system (the cardiac leads from the first device had broken), Dr. McCanta advocated for the Micra TPS. He knew the benefits it would bring to Sofia, and other CHOC patients.

“The immediate benefit to Sofia is that she does not have another scar on her upper chest. The long-term benefit is she has no cardiac lead in her vein that could break, become infected or cause a blood clot,” explains Dr. McCanta. “When you consider the course of her entire life, the benefit of reducing complications of pacing is almost immeasurable.”

Sofia’s parents are proud of the part she played in the medical milestone. “She’s always been special, and here’s just one more thing that demonstrates how special she truly is,” says Sofia’s dad, Edgar. “We are thankful for people like Dr. McCanta and so impressed with the new technology. We feel truly blessed.”

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Sofia and her dog, Yoda

When she’s not on her computer, Sofia loves watching videos and listening to Latin music. She’s outgoing with lots of friends and a bright future ahead of her.

Learn more about the electrophysiology program at CHOC

Related posts:

  • Tetralogy of Fallot Patient and his Family Give Back
    What was supposed to be a routine visit with their pediatrician on a seemingly typical Friday morning changed Stephanie Harding and her son Trent’s life forever.
  • Catching a Heart Defect in Utero: Marco’s Story
    Meagan and Dante Cipulli quickly settled on a name when they discovered their third baby would be a boy: Marco, which means God of War.That name would become especially fitting ...
  • Innovation Comes Home
    With the Integrated Medical Tablet provided by the CHOC Cardiopulmonary Health Intelligence Program (CHiP), Emma’s doctors, nurses and care team are only a touch screen away.

A Reunion of Heroes: Katie’s Story

After recently being discharged, Katie Flathom stopped by the CHOC Children’s cardiovascular intensive care unit (CVICU) again to make some introductions.

Suddenly, the 16-year-old has a group of heroes in her life: the coach who resuscitated her on school campus and the CHOC team that treated Katie for three weeks and will continue her care as she navigates life with a newly diagnosed heart condition.

During a recent athletic conditioning class in school, Katie collapsed and went into sudden cardiac arrest.

Her trained and quick-thinking conditioning coach snapped into action and resuscitated Katie with CPR until paramedics could arrive and transport her to CHOC.

“It was the longest 10 minutes of my life,” said Greg Vandermade, Katie’s coach at Mater Dei High School who also credits other students for alerting him to Katie’s condition and calling 911, as well as fellow staff who assisted by obtaining an automated external defibrillator (AED) to shock Katie’s heart into a normal rhythm.

At CHOC, Katie continued to have irregular heartbeats that required further defibrillation and cardioversion, procedures that help restore the heart’s natural heart rhythm, said Dr. Anthony McCanta, a CHOC cardiologist.

Katie also went on extracorporeal life support, a treatment that takes over the heart’s pumping function and the lungs’ oxygen exchange until a patient can recover from injury. This allowed the CHOC Children’s Heart Institute team to continue to treat her life-threatening arrhythmias with medication, Dr. McCanta said.

Dr. McCanta performed an electrophysiology study procedure and implanted beneath Katie’s skin a subcutaneous implantable cardioverter defibrillator, a device that helps prevent sudden cardiac arrest in patients.

After Katie’s discharge and further testing, she was diagnosed with Arrhythmogenic Right Ventricular Dysplasia, or ARVD.  A rare type of cardiomyopathy  where the muscle tissue in the heart’s right ventricle is infiltrated and replaced by fatty tissue and scar tissue, ARVD weakens the heart’s ability to pump blood and makes the heart susceptible to life-threatening arrhythmias.

The diagnosis also means Katie, a cross country and track athlete, will need to give up running for good.

“It was hard at first,” she said.

But instead of sitting on the sidelines, Katie’s decided to pick up golf, a sport that’s compatible with ARVD.

Katie has even begun incorporating a golf swing into her physical therapy sessions at CHOC, and she had two clubs in tow as she, her family and coach Greg visited the CVICU recently.

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When Katie came back to visit the CVICU team and reunite her heroes, her CHOC care team presented her with a heart-shaped pillow, which they all signed with well wishes.

There, Dr. McCanta and the CHOC team presented Greg with a plaque recognizing him for his swift response and efforts that surely saved Katie’s life.

“Coach Greg responded to Katie with CPR on the spot and saved her life that day,” Dr. McCanta said. “His heroic actions, and those of Katie’s schoolmates and staff, including obtaining and appropriately using the AED, are the reason that Katie is alive today.”

Katie’s story underscores the importance of being trained in CPR and in the use of AEDs, Dr. McCanta said.

“Having AEDs in schools and training staff and students in CPR with an AED are some of the most important interventions that we have in saving lives of young people experiencing sudden cardiac arrest,” he said.

Getting AEDs installed in schools is among the goals of CHOC’s Life-Threatening Events Associated with Pediatric Sports – or LEAPS – program.

Coincidentally, Katie’s own grandmother, a nurse and health services coordinator in the Irvine Unified School District, has collaborated with LEAPS and helped get AEDs installed on her district’s campuses.

“Never did I think though that this would happen to one of my own family members,” said Marcia, Katie’s grandmother.

Learn more about pacemakers and cardioverter defibriilators

Related posts:

  • Tetralogy of Fallot Patient and his Family Give Back
    What was supposed to be a routine visit with their pediatrician on a seemingly typical Friday morning changed Stephanie Harding and her son Trent’s life forever.
  • Catching a Heart Defect in Utero: Marco’s Story
    Meagan and Dante Cipulli quickly settled on a name when they discovered their third baby would be a boy: Marco, which means God of War.That name would become especially fitting ...
  • Innovation Comes Home
    With the Integrated Medical Tablet provided by the CHOC Cardiopulmonary Health Intelligence Program (CHiP), Emma’s doctors, nurses and care team are only a touch screen away.